MANUAL OF

MANUAL OF CARDIOLOGY

V Jacob Jose MD DM card, MS Univ of Penn, FCCP, FACC, FIAE Professor of Cardiology Department of Cardiology Christian Medical College Hospital Vellore, Chennai

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Manual of Cardiology

© 2007, V Jacob Jose All rights reserved. No part of this publication should be reproduced, stored in a retrieval system, or transmitted in any form or by any means: electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the author and the publisher. This book has been published in good faith that the material provided by author is original. Every effort is made to ensure accuracy of material, but the publisher, printer and author will not be held responsible for any inadvertent error(s). In case of any dispute, all legal matters are to be settled under Delhi jurisdiction only.

First Edition: 2007 ISBN 81-8061-924-9 Typeset at JPBMP typesetting unit Printed at Gopsons Papers Ltd, Sector 60, Noida To My daughters Riya Jose and Nisha Jose

PREFACE

There are excellent monographs in cardiology for cardiologists, but there exists none for the undergraduates in Medicine. I felt this problem when I was taking classes for our undergraduates. Hence I decided that some of the notes that I distribute to medical students during their classes be made into a book. The idea was to keep it as simple as possible but at the same time to give them a full understanding of the subject. Since this is a small book, over simplification was unavoidable. I am sure students will find this as a basic text and will use the regular reference books to expand their knowledge. I am deeply indebted to our Director, Dr. George Chandy for giving me the permission to publish this book. I would also like to express my due thanks to my colleagues in the department who have critically looked at the text of this book. I wish to express my thanks to Dr. Noah Chelliah, who was kind enough to give some of the teaching slides he has been using in USA. These slides have been incorporated in the section of arrhythmias. I also would like to express my sincere thanks to my secretary Ms. Deepa Dayalan for typing and proof reading. I would welcome your critical comments or suggestions regarding this book, so that we will incorporate the same in our next edition.

V Jacob Jose

CONTENTS

SECTION I: CLINICAL EXAMINATION 1. History ...... 3 2. General Examination ...... 9 3. Arterial ...... 14 4. Jugular Venous Pulse ...... 21 5. Precardial Palpation ...... 25 6. Sounds...... 33

SECTION II: 7. Acute ...... 43 8. Mitral Stenosis ...... 54 9. Mitral Regurgitation ...... 64 10. Aortic Stenosis ...... 70 11. Aortic Regurgitation ...... 76 12. Infective ...... 84

SECTION III: ISCHEMIC HEART DISEASE 13. ...... 96 14. Acute Myocardial Infarction ...... 110

SECTION IV: HEART FAILURE 15. Heart Failure ...... 127

SECTION V: CARDIOMYOPATHY 16. Dilated Cardiomyopathy ...... 139 17. Hypertrophic Cardiomyopathy ...... 141 18. Restrictive Cardiomyopathy ...... 148 x Manual of Cardiology

SECTION VI: AND AORTA 19. Acute Pericarditis ...... 153 20. ...... 156 21. Constrictive Pericarditis ...... 160 22. Dissection of Aorta ...... 162

SECTION VII: CONGENITAL HEART DISEASE 23. Atrial Septal Defect...... 170 24. Ventricular Septal Defect ...... 179 25. Patent Ductus Arteriosus ...... 186 26. Tetralogy of Fallot ...... 191 27. Coarctation of Aorta...... 203

SECTION VIII: ARRHYTHMIAS 28. Atrial Fibrillation ...... 207 29. Atrial Flutter ...... 214 30. Narrow QRS ...... 216 31. Ventricular Tachycardia ...... 220 32. Heart Block...... 223 33. Cardiac Arrest and Sudden Death ...... 226

SECTION IX: HYPERTENSION 34. Hypertension ...... 235

SECTION X: PULMONARY VASCULAR DISORDERS 35. Pulmonary Arterial Hypertension ...... 243 36. ...... 247

SECTION XI: INVESTIGATIONS 37. Chest X-ray ...... 253 38. ECG ...... 259 Index ...... 265 Section I CLINICAL EXAMINATION

HISTORY 1

DYSPNEA Abnormally uncomfortable awareness of breathing.

New York Heart Association (NYHA)—Functional Classification All patients with dyspnea should be assessed in relation to their functional ability and this is usually graded as per the above classification. This classification is also used for fatigue, palpitation and

TABLE 1.1: NYHA classification Class Functional classification I Ordinary physical activity does not cause dyspnea II Ordinary physical activity results in dyspnea III Less than ordinary physical activity causes dyspnea IV Inability to carry on any physical activity without discomfort

Limitations 1. Subjective in nature 2. Not easily reproducible 3. Class 0 is not there. Hence many people are now using American Thoracic Society scale of dyspnea which has grade 0 as well and also has quantification in terms of length of distance walked. 4 Manual of Cardiology

Etiology of Dyspnea Cardiac Causes 1. Left ventricular failure of any cause 2. Mitral stenosis.

Lung Causes 1. Asthma 2. COPD 3. Interstitial lung disease 4. Large pleural effusion.

Chest Wall Causes 1. Kyphoscoliosis.

Metabolic Causes 1. Ketoacidiosis 2. Aspirin poisoning.

Other Causes 1. Anemia 2. Obesity.

Acute Dyspnea 1. Acute asthma 2. Acute pulmonary edema 3. Pulmonary thromboembolism 4. Pneumothorax 5. Foreign body in the airway. Paroxysmal nocturnal dyspnea (PND): In this form of dyspnea, the patient develops breathing difficulty 2-5 hours after going to sleep and the patient is forced to get up and seek fresh air. History 5

Mechanism 1. Decreased sympathetic drive of the heart during night 2. Nocturnal depression of respiratory center.

Orthopnea This can be defined as dyspnea in the recumbent position which is relieved by elevation of the head. Many patients use several pillows at night to elevate the head to avoid orthopnea. In severe heart failure patient may spend the entire night in sitting position.

Mechanism 1. Redistribution of fluid from abdomen and legs into the chest during recumbency increases the pulmonary capillary pressure 2. Elevation of diaphragm in the lying down position.

Trepopnea Dyspnea in left or right lateral decubitus position.

Platypnea Dyspnea at upright position 1. Left atrial thrombus 2. Left atrial tumor 3. Pulmonary .

CHEST PAIN Chest pain is one of the most important manifestations of heart disease. It is important to identify whether the chest pain is due to a cardiac reason or not. In the history, the following points need to be taken into account. One way of remembering is PQRST P—Precipitating factors: In angina the chest pain is worsened with exercise, cold weather, emotional stress. 6 Manual of Cardiology

Q—Quality: This is a very important point because since the pain is visceral in nature the pain is felt as pressure, heaviness or squeezing. R—Relief: The pain is typically relieved by rest or nitroglycerin. S—Site: Usually it is retrosternal in nature, radiating to the neck, jaw, shoulder or arms. T—Timing: Usual duration of pain is around 2 to 10 minutes in stable angina. In unstable angina the pain may be as long as 10 to 20 minutes. In patients with myocardial infarction the pain usually last more than 20 minutes.

Causes The following are the cardiovascular causes of chest pain: 1. Stable angina 2. Rest/unstable angina 3. Myocardial infarction 4. Pericarditis 5. Dissection of aorta 6. Pulmonary embolism 7. Pulmonary hypertension.

Angina Equivalent Some patients with angina do not have chest pain; instead they may have symptoms such as dyspnea on exertion. Other angina equivalents are discomfort seen in areas of secondary radiation without chest pain. For example, patient may have discomfort in the lower jaw or ulnar aspect of left arm or neck. Features of noncardiac pain: 1. Stabbing or shooting pain 2. Pain lasting less than 30 seconds 3. Well localized, left submammary pain 4. Pain that is constantly varying in its location. History 7

SYNCOPE Syncope is defined as transient loss of consciousness and postural tone due to decreased cerebral flow with spontaneous recovery. A good history with simple tests such as ECG can give a clue to the diagnosis for syncope. In the history, it is important to differentiate between the following: 1. Is the sensation described is a “sensation of movement without actual loss of consciousness?” If so, then this is due to vestibular or labyrinthine dysfunction and suggests vertigo. 2. Is it a sensation of loss of balance with actual loss of consciousness? This occurs secondary to loss of joint sense, visual disturbances, etc. 3. In syncope there is reduced cerebral blood flow leading onto loss of consciousness or a sensation of lightheadedness which may precede the same.

History Taking The following five points need to be taken in history: 1. Preceding events: From the history, you must ask whether syncope happened after prolonged standing or seeing some unusual sight such as an accident, or turning the head, etc. 2. Type of onset: The onset is sudden in arrhythmia and in seizures. It is gradual in vasovagal syndrome. 3. Position at onset: This history is important because arrhythmia can happen in any position. However, vasovagal syncope occurs on standing for a long time. 4. Postsyncopal clearing: Clearing of consciousness is gradual and takes a long time in seizures where as it is very brief in cardiac reasons. 5. Associated events: Tongue biting, urinary incontinence are common with seizures. 8 Manual of Cardiology

TABLE 1.2: Differentiating features of causes of syncope Vasovagal or Neurally Mediated Cardiac Neurogenic Syncope Preceding events Standing, seeing None None some accident or blood Type of onset Gradual Sudden Sudden Position at onset Standing Any position Any position During the episode Pallor Pallor Face congested Tongue biting Urinary incontinence Motor activity Recovery Gradual Slow Prolonged about 5 minutes

PALPITATION Unpleasant awareness of the beating of the heart.

History Taking If you can ask the patient to tap out the rate and regularity of the , it may give you an idea to the etiology. The following items need to be covered in history: 1. Is it isolated, occurring as skipped beats? Suggests ventricular ectopic beats 2. Is the attacks sudden in onset and sudden in offset? Suggests supraventricular tachycardia 3. Is the palpitations regular or irregular? Irregular suggests atrial fibrillation 4. Is it related to exercise? Common in atrial fibrillation, thyrotoxicosis 5. Is it related to taking any drugs? Such as thyroid tablets, coffee, tea 6. Associated flushing and sweating in a middle age women suggests menopausal syndrome. General Examination 9

GENERAL EXAMINATION 2

While taking the history and before inspecting the cardiovascular system you must assess the general condition of the patient as a routine. The following points need to be examined from the cardiovascular point of view.

DYSMORPHIC FEATURES 1. Look for periorbital puffiness, loss of lateral eyebrows— suggests hypothyroidism. 2. Depressed bridge of the nose—can occur with Down’s syndrome or Elfin’s face of supravalvar stenosis. 3. Hypertelorisim—the distance between the two inner canthi is increased, seen in pulmonary stenosis. 4. Blue sclerae—osteogenesis imperfecta. 5. Webbing of the neck—coarctation of aorta. 6. Polydactyly—atrial septal defect. 7. Short stature—coarctation of aorta.

CYANOSIS Bluish discoloration of skin and mucus membrane due to reduced hemoglobin is called as cyanosis.

Types 1. Central cyanosis: Cyanosis is seen in the skin and warm areas such as oral cavity. This is observed in right to left shunts in the heart, e.g. TOF. 10 Manual of Cardiology

Central cyanosis manifests at a mean capillary concentration of 4 gm/dl of reduced hemoglobin. In general when the systemic arterial saturation is less than 85%, cyanosis is manifest. 2. Peripheral cyanosis: Decreased capillary blood flow allows more time for extraction of oxygen. Seen in cardiac failure, , venous or arterial obstruction. 3. Differential cyanosis: Cyanosis more marked in the legs than in the upper limb; in patients with patent ductus arteriosus with pulmonary hypertension the blood gets shunted from the pulmonary to aorta. The blood going from the pulmonary artery to the descending aorta through the ductus is less saturated with the result the lower limbs are cyanosed. On the other hand, oxygenated blood coming the pulmonary is ejected through the ascending aorta with the result that the upper limbs are not cyanosed.

CLUBBING Selective bulbous enlargement of distal segment of fingers and toes due to proliferation of connected tissue.

Mechanism Due to right to left shunt, platelet derived growth factors are not inactivated in the lungs. This results in development of AV shunts in the distal phalanges, which results in the swelling.

Causes Cardiac Causes • Cyanotic congenital heart disease • Endocarditis • Atrial myxoma. General Examination 11

Lung Causes • Bronchial carcinoma (usually not small cell) • Chronic lung suppuration—empyema, abscess, bronchiec- tasis, cystic fibrosis • Fibrosing alveolitis • Mesothelioma.

Gastrointestinal Causes • Inflammatory bowel disease (esp. Crohn’s disease) • Cirrhosis of liver • GI lymphoma • Malabsorption, e.g. celiac disease.

Rare • Familial • Thyroid acropathy.

Testing 1. Keep the dorsal aspects of two fingers side by side with the nail touching normally; you should see a kite shaped gap. If not clubbing is present- Schamorth sign. 2. Lateral Profile: View the side of the flexed distal finger look for nail bed angle. If there is no dip there is clubbing. The hyponychial nail—fold angle can also be measured, if this is more than 180 degree then clubbing is present. 3. Fluctuation: Place the finger of the patient between the examiners two thumbs. Next palpate the nail bed of the patient with the examiner’s two index fingers and elicit fluctuation. 4. Phlalangeal Depth Ratio: The vertical distance measured at the junction of the nail bed is more than the inter phalangeal depth.

Grades Grade I—Increased glossiness of the nail bed. 12 Manual of Cardiology

Grade II—Fluctuation of the nail bed or obliteration of the angle of nail bed. Grade III—Excessive curvature or parrot beak appearance. Grade IV—Hypertrophic pulmonary osteoarthopathy— perios- titis of wrist and ankles.

Unilateral Clubbing • AV fistula • interfering with arterial supply to one arm.

EDEMA Firm pressure is applied on the pretibial region for 10 to 20 seconds. Note whether there is pitting or non-pitting. Non- pitting edema suggests that it is due to poor lymphatic drainage such as Filariasis. Pitting edema can further be classified as slow or fast. Slow Edema—The pitting remains for more than one minute; this is seen in conditions such as cardiac failure. Fast Edema—The pitting disappears in less than 40 seconds. This is seen in conditions such as hypoproteinemia. In patients who are lying down in bed, edema may be more marked in the sacral region. In children, facial edema may be more marked than peripheral edema.

FIGURE 2.1: Algorithm to identify the cause of edema General Examination 13

Causes a. Increased venous pressure—right heart failure, constrictive pericarditis b. Lower oncotic pressure—cirrhosis, nephrotic syndrome c. Lymph edema—filariasis, Milroy’s syndrome. 14 Manual of Cardiology

ARTERIAL PULSE 3

Pulse is a pressure wave originating in the aorta due to ejection of blood during systole from LV, and this wave travels along the arterial wall at a rate much faster than the actual column of blood (Pulse wave velocity is 5 meters per second; velocity of intra-luminal blood is 40 to 50 cm per second).

EXAMINATION OF THE PULSE Press with the examining fingers until maximum pulse is sensed. Pulse is felt as changing displacement superimposed on the baseline displacement produced by compressing the artery. The index finger is the best finger to feel the pulse. Scale for comparing the pulse 0 - Absent pulse 1+ - Present but diminished pulse 2+ - Average normal pulse 3+ - Moderately increased 4+ - Markedly increased pulse

FEATURES OF ARTERIAL PULSE TO BE EXAMINED 1. Rate 2. Rhythm 3. Waveform or character 4. Radio femoral delay 5. Vessel wall thickness 6. Peripheral . Arterial Pulse 15

Rate The resting heart rate shows considerable variation ranging between 50 to 120 beats/min. Rate should be counted for atleast 30 seconds. When rhythm is irregular, count for full one minute. Pulse deficit: In atrial fibrillation the rate of palpable arterial pulse is slower than the rate of ventricular contraction. Pulse deficit is identified by comparing the rate of arterial pulse (palpable) with the heart rate judged by simultaneous precordial . In order to observe the pulse deficit it is better to have two persons simultaneously examine the patients; one person will count the heart rate using the stethoscope; the second person will count the pulse rate from the radial artery. The deficit is said to be significant if the difference between heart rate and the pulse rate is more than 10. If a second observer is not available then you may first count the heart rate by auscultation and then subsequently count the pulse rate using the radial artery.

Rhythm The normal rhythm is initiated from the sinus node and this follows a regular cycle within physiological variations. The rhythm can be intermittently irregular or can be continuously irregular. The common causes for intermittently irregular pulse is either ventricular premature beats or supraventricular premature beats. In the table below shows the causes for irregularly irregular pulse are given.

TABLE 3.1: Causes of irregular rhythm IRREGULARLY IRREGULAR 1. Sinus arrhythmia 2. Atrial fibrillation 16 Manual of Cardiology

CHARACTER Hyperkinetic Pulse (Large or Bounding pulse) Indicates the rapid ejection of an increased volume of blood from the left . This is more prominent in brachial, radial and femoral than in the carotid artery. Water Hammer pulse: Refers to an extremely rapid forceful ascending limb of the arterial pulse wave. Collapsing pulse: Refers to a sharp decrease in the pulse wave after its peak (descending limb).

Causes 1. Aortic regurgitation 2. Patent ductus arteriosus 3. Thyrotoxicosis 4. Anemia 5. Pregnancy.

Hypokinetic Pulse This is a small amplitude pulse resulting from decreased LV stroke volume with decreased LV ejection time, as there is less blood to eject. Causes include congestive cardiac failure, hypo- tensive states like , left ventricular outflow tract (LVOT) obstruction. The rate of rise of the hypokinetic pulse is important. If it is prolonged it suggests LVOT obstruction.

Pulsus Parvus et Tardus It is a small amplitude pulse with delayed systolic peak seen in aortic stensois. Occasionally, there may be a detectable shoulder on the upstroke of the carotid pulse, (the anacrotic shoulder).

Bisferiens Pulse This is a pulse with two positive waves in systole. It is best felt in the carotid; sometimes more easily felt in the brachial or radial artery. Arterial Pulse 17

FIGURE 3.1: The figure above shows graphically the character of various pulses

Causes 1. Severe aortic regurgitation 2. Moderate aortic stenosis with severe aortic regurgitation 3. Hypertrophic cardiomyopathy.

Dicrotic Pulse The dicrotic (from Greek, dickrotos means double beating) pulse is a twice-peaked pulse with one peak in systole and second peak in diastole, the later, due to an accentuated and palpable dicrotic wave that follows the second heart sound. It is best felt in carotids.

Causes 1. Cardiomyopathy or severe LV dysfunction 2. Following valve replacement for AR or MR (indicates poor LV function) 3. Febrile states in young subjects (controversial) 4. During inspiration in pericardial tamponade. 18 Manual of Cardiology

Pulsus Paradoxus During inspiration the pulse becomes weak or disappears completely. This can be correlated by taking blood pressure during which there is a fall of 10 mmHg or more during inspiration.

Causes 1. 2. Pulmonary Embolism 3. Asthma Severe

FIGURE 3.2: The figure above shows graphically the character of various pulses

Pulsus Alternans In this form of abnormal pulse wave, the height of the pulse wave is alternatively tall and small (Figure 3.2). Method of determination: Cuff pressure is elevated above the systolic BP, then gradually reduced 1 to 2 mmHg/sec. Initially are heard with stronger beats. On further lowering of mercury pressure there is sudden doubling of Korotkoff sounds. Arterial Pulse 19

Causes • Severe LV dysfunction

Pulses Paradoxus With inspiration there is a decrease in the pulse amplitude and the systolic fall during inspiration is more than 10 mmHg. Methods of determination: The cuff pressure is raised above systolic BP then gradually decreased with 1 to 2 mmHg per sec. Initially Korotkoff sounds are heard during expiration, on further lowering of cuff pressure, Korotkoff sounds are heard in both phases of respiration. The difference of pressure from first audible Korotkoff sound to sounds heard in both phases if more than 10 mmHg is abnormal.

Causes • Pericardial tamponade • Constrictive pericarditis • Restrictive cardiomyopathy • Severe COPD.

Hill’s Sign Normally lower limb systolic BP is not more than 20 mmHg higher than upper limb systolic BP whereas diastolic BP is identical. If the lower limb systolic BP is more than 20 mmHg compared to the upper limb, the Hill’s sign is positive.

Severity of aortic regurgitation: (based on Hill’s sign) Mild aortic regurgitation: 20 to 40 mmHg Moderate aortic regurgitation: 40 to 60 mmHg Severe aortic regurgitation: >60 mmHg

RADIO FEMORAL DELAY Method of Examination • Make the patient lie down supine 20 Manual of Cardiology

• Place the patients’ right arm straight, parallel and close to the body. • Place the left hand of the examiner at the radial artery • Place the right index finger of the examiner at the right femoral artery of the patient. The idea is that you must examine the femoral artery and the radial artery as close as possible, in one line. If you keep the patients hand far away from the patients’ femoral artery then you will not appreciate the delay. In coarctation of aorta the peak of the femoral artery pulse is delayed. This is something similar to the aortic stenosis, in which there is a delayed peak. In fact we are actually comparing the delayed peak of the femoral pulse with the radial pulse. Kindly note that it is not a delayed arrival, but delayed peak.

VESSEL WALL THICKNESS Three-finger method: The index, middle and fourth finger of the left hand is used for palpating the radial pulse. With the fourth finger compress the artery distally so has to prevent flow from ulnar collateral. Compress the radial artery with the index finger thus occluding the flow into the artery. Now use the middle finger to roll the artery and palpate its thickness. The only condition that pathologically makes the radial artery thick is Monkeberg’s sclerosis. Jugular Venous Pulse 21

JUGULAR VENOUS PULSE 4

Jugular neck veins are inspected for the following: 1. To detect elevated venous pressure 2. To detect abnormal waveforms.

TECHNIQUE 1. It is better to examine the right side of the neck, because these veins are directly connected to the heart in a straight line. 2. It is better to position the patient at 45º angle to look for venous pressure elevation. 3. Internal jugular veins: Are used to assess the waveforms. It is traditionally taught not to use external jugular because, the valves in the external jugular vein will interfere with the measurement of pressure. However, this teaching is erroneous. 4. Hepatojugular test: Apply firm pressure in the patients’ mid abdomen for 10 to 20 seconds. At this time, observe the . If the pressure raises more than 3 cm and remains elevated for 15 seconds or more this test is said to be positive. A positive hepatojugular reflux suggests that the patient has got right ventricular dysfunction. This test is useful in patients with mild right sided heart failure in whom the jugular venous pressure may be normal at rest. 5. Differentiation Jugular Venous Pulse From Carotids: At the bedside, it is important to differentiate between jugular venous wave from carotid artery pulsations and the differences between them are given in Table 4.1. 22 Manual of Cardiology

TABLE 4.1: Differences between Jugular venous pulse and carotids Variable JVP Carotids 1. Site Lateral Medial 2. Visible Better seen than felt Better felt than seen 3. Waves 2 Waves Single 4. Respiration Varies with respiration No change 5. Compression Obliterated Not obliterated with usual light pressure

JUGULAR VENOUS PRESSURE (JVP) MEASUREMENT Method 1. Measure from the sternal angle as shown below, using 2 scales. 2. Keep scale A vertically up from the sternal angle. 3. Keep the scale B horizontally at the top column of blood. 4. Measure the distance in scale A and this value is expressed in centimeters of water (Figure 4.1).

Measurement of JVP

FIGURE 4.1: The figure shows the placement of two scales to measure Jugular venous pressure. Scale A is placed vertically from the sternal angle. Scale B is placed horizontally from the upper level jugular venous pulsation.

Causes of Elevated JVP a. Cardiac failure b. Hyperkinetic state—anemia c. Bernheim effect: A bulge of interventricular septum towards the right ventricle restricts the filling capacity of the right ventricle and, hence, elevates the venous pressure on the right side. Jugular Venous Pulse 23 d. Raised intrapleural and intraabdominal pressure— pregnancy, large pleural effusion. Kussmaul’s sign: An increase in the JVP with inspiration, which is seen in constrictive pericarditis or in cardiac tamponade.

WAVEFORMS MACKENZIE has labeled the waveforms as A, C and V waves and this nomenclature is still used (Figure 4.2). A wave = atrial contraction. C wave = carotid artifact, not seen clinically. V wave = venous filling. X descent = systolic contraction, leading onto descent of tricuspid valve. Y descent = opening of tricuspid valve, leads on to a decent in the wave form.

FIGURE 4.2: The figure shows Jugular Venous waveform in relation to ECG. “a” wave follows the “p” wave in ECG and “y” descent follows the “T” wave. 24 Manual of Cardiology

TABLE 4.2: Jugular venous wave forms and its significance Wave Abnormality Significance A Wave Absent Atrial fibrillation A prominent Pulmonary stenosis Pulmonary hypertension Cannon wave Complete heart block (Giant A waves) Ventricular ectopics Junctional rhythm V Wave Fused CV wave (or) Tricuspid regurgitation Systolic wave X Wave Absent Tricuspid regurgitation Prominent Constrictive pericarditis Restrictive cardiomyopathy Y Wave Slow Tricuspid stenosis Prominent Constrictive pericarditis Absent Cardiac tamponade

FIGURE 4.3: The above figure shows a prominent CV wave which is characteristic of tricuspid regurgitation. In this X descent is obliterated and there is a systolic wave due to back flow from the right ventricle to right which produces a systolic fusion wave called as CV wave.

FIGURE 4.4: This Jugular venous wave form shows prominent descents in the form of ‘x’ and ‘y’. Y is actually prominent than the ‘x’ and this kind of pattern is seen in constrictive pericarditis. Precardial Palpation 25

PRECARDIAL PALPATION 5

APEX BEAT The , otherwise called as cardiac impulse or apical thrust, is normally produced by left ventricular contraction. It is the lowermost and outermost point of definite cardiac impulse, which imparts a perpendicular thrust to the palpating finger.

Mechanism The palpable apical impulse in a normal subject is produced by an anterior movement of the LV during early systole. During the isovolumic contraction, the LV rotates in a counter clockwise direction along its long axis, and the juxta- apical region lifts and makes contact with the anterior chest wall.

Patient Position Apex beat is usually located in the supine position. However, this does not correspond to the cardiac size as seen in the chest X-ray. Accordingly, some authors suggest that apex beat be visualized/palpated in the sitting position. This is not done routinely.

Location 1. Normally the left ventricular impulse is medial and superior to the intersection of the left mid-clavicular line and the fifth 26 Manual of Cardiology

intercostal space. Hence if the apex beat is beyond the mid-clavicular line (MCL), can be taken as a sign of cardiac enlargement. 2. Displacement of the apex beat lateral to the mid-clavicular line or more than 10 cm lateral to the mid-sternal line is a sensitive, but not specific indicator of left ventricular enlargement. 3. In tall, thin persons, the apex beat can be distal (6th interspace) and more medial than usual. 4. In infants and in adults with short, stocky chests, the impulse is often in the fourth left interspace. 5. The apical impulse may displace upward and to the left by a high left diaphragm or during pregnancy. 6. Scoliosis to the right rotates the heart leftward. 7. Pectus excavatum shifts the heart to the left.

TABLE 5.1: Site of apex to detect enlarged cardiac size Sensitivity Specificity Position beyond MCL 40-60% 70-90% Apex greater than 10 cm 60-80% 28-97%

Size In the supine position, the apex usually measures less than 2.5 cm and occupies only one interspace. In the left lateral decubitus position diameter greater than 3 cm is an accurate sign of LV enlargement.

Duration The impulse is usually brief—maximum of 0.08 secs.

Amplitude In normal individuals, the apex beat is felt as a gentle outward motion. There may be respiratory alterations in the amplitude of the apex beat. Pay attention to the end of expiration. Precardial Palpation 27

CHARACTER Normal Apical Impulse • A gentle non-sustained tap • Early systolic anterior motion that ends before the last third of systole • Located within 10 cm of the mid-sternal line in the 4th or 5th intercostals space • A palpable area less than 2 to 2.5 sq cm and detectable in only one intercostals space • Diastolic events are normally not palpable • May be completely absent in older persons.

Sustained Apical Impulse The left ventricular heave or lift, which is more prominent in concentric hypertrophy, is characterized by: 1. Area that is larger than the normal apex, i.e. more than 2 to 3 cm in diameter. 2. Occupies more than one intercostal space. 3. It is characterized by prolonged duration of the outward movement. When this duration is compared with the carotid pulse wave, it extends till the down stroke of the carotid pulse. On simultaneous auscultation with palpation of the apex beat, the bottom of the fall of the apex beat will be closer to the second heart sound.

FIGURE 5.1: Schematic representa- tion of sustained apical impulse 28 Manual of Cardiology

Hyperdynamic Apical Impulse There is increase in the amplitude of the LV impulse without a change in contour. The sequence between the onset of the carotid and the outward movement remains normal. It is appreciated as thrust of large amplitude that immediately disappears from the palpating fingers. When the duration of the ejection phase is estimated from the carotid pulse upstroke and down-stroke or from the interval between first and second heart sound, the hyperdynamic apical impulse does not extend throughout the systole.

FIGURE 5.2: Schematic representation of normal and hyperdynamic apical cardiac impulse

Hypokinetic Impulse In this type of apical impulse, the rate of rise and the amplitude are reduced. In addition, the apical impulse may be displaced laterally. This is observed in patients with dilated cardio- myopathy.

Dyskinetic Impulse LV aneurysm may produce a sustained systolic bulge several centimeters superior to the left ventricular impulse, sometimes termed an ectopic impulse. It can be associated with a systolic impulse in unusual location such as over the mid precordium. Precardial Palpation 29

The most common site of an ectopic impulse is above and medial to the expected location of the LV impulse. The ectopic segment, often as readily seen as palpated, is usually caused by the anterior wall dyskinesia of coronary artery disease (CAD). LV aneurysm produces a larger than normal area of pulsation of the LV apex. An anterolateral or apical aneurysm may be felt best at the apex but an anterior aneurysm may be felt several centimeters away from the apex. Some patients with dyskinetic left ventricles may have two distinct areas of rocking movement separated by several centimeters. Patients with LV aneurysm will have palpable ‘a’ wave, and thus a double apical impulse.

Double Apical Impulse In hypertrophic obstructive cardiomyopathy, a double impulse is felt in systole itself.

PARASTERNAL LIFT Except in the first few months of life, right ventricle is not palpable. In children and in occasional adults with thin chest walls, a brief, gentle impulse may be palpable over the left third and fourth interspaces. This impulse is usually in diastole. In normal individuals during systole a gentle systolic retraction may be felt.

Examination Technique 1. The patient is examined in supine position at 30 degrees. 2. It is desirable to use held end expiration for RV examination. 3. Two methods of RV palpation are recommended. a. The heel of the hand can be applied firmly to the left sternal edge during full expiration, keeping the fingers elevated while sensing the systolic impulse and observing the motion imparted by the RV contraction. This techni- que is relatively insensitive in detecting subtle systolic 30 Manual of Cardiology

movement and does not localize the impulse so derived (Figure 5.3, Plate 1). b. A more refined method employs several fingertips applied simultaneously and in parallel in the third, fourth and fifth intercostal spaces during held expiration. The free left hand is used for timing with the right carotid pulse as reference or by applying the stethoscope to the chest for the identification of the first and second . An impulse felt in the 4th and 5th intercostal spaces represent the body of the right ventricle. Impulse felt in the 3rd left intercostal space represent the right ventricle outflow tract (Figure 5.4, Plate 1). 4. Subxiphoid Impulse: The subxiphoid region, which allows palpation of the right ventricle, should be examined with the tip of the index finger during held inspiration. With the hand flat, the index finger should be pressed just under the rib cage and up toward the left shoulder. The examiner should be careful to differentiate the downward- directed motion of the enlarged RV from the anterior pulsations of the abdominal aorta, which can often be felt in the epigastrium. Palpable RV diastolic events are often best detected with this approach (Figure 5.5, Plate 2).

CAUSES OF PARASTERNAL LIFT 1. RV volume overload: The parasternal lift has a quick rise and quick fall; the duration is brief, e.g. atrial septal defect (ASD). 2. RV pressure overload: A sustained left parasternal outward thrust reflect RV hypertrophy due to pressure overload, as in pulmonary arterial hypertension or pulmonary stenosis. Right ventricular failure with reduced RV ejection can also produce a prolonged left parasternal impulse. Precardial Palpation 31

3. Left atrial impulse: Significant mitral regurgitation (MR) may cause left atrial expansion during LV systole producing a left parasternal impulse. The systolic bulging of the left atrium (LA), which is transmitted through the RV, commences and terminates after the LV thrust. Hence it is late systolic in timing. LA impulse is best appreciated by placing the index finger of one hand at the LV apex and the index finger of the other in the left parasternal region in the third intercostal space; the movement of the latter finger begins and ends slightly later than that of the former.

Dressler’s Grading of Left Parasternal Lift Grade 1: Faintly felt. Grade 2: Felt, seen, but can be obliterated with pressure. Grade 3: Cannot be obliterated with pressure.

THRILLS Thrills are palpable vibrations from the murmurs or , ordinarily associated with grade 4/6 murmur or louder.

Method 1. The flat of the hand at the meta-carpo phalangeal joints or the finger tips are the best to appreciate thrills. 2. Thrills are most readily characterized according to their timing in the cardiac cycle (systolic, diastolic or continuous), their location, their direction of radiation, and their duration. High pitched murmurs such as those produced by valvular regurgitation, even when loud are not usually associated with thrills.

Causes 1. Aortic stenosis—systolic thrill is felt in the 2nd right intercostal space. 32 Manual of Cardiology

2. Pulmonary stenosis can produce a systolic thrill in the 2nd left intercostal space. 3. Ventricular septal defect—systolic thrill in the 3rd, 4th and 5th left intercostal spaces close to the sternum. 4. Hypertrophic obstructive cardiomyopathy—systolic thrill in the 4th and 5th intercostal spaces between the apex and the sternum. 5. Mitral regurgitation—systolic thrill in the mitral area. 6. Mitral stenosis—diastolic thrill at the apex. Heart Sounds 33

HEART SOUNDS 6

FIRST HEART SOUND The first heart sound (S1) signals the onset of LV contraction which occurs 50 to 60 msec after the electrical initiation of LV systole.

Components 1. The first component is due to closure of (M1). 2. Second high frequency component is related to tricuspid valve closure (T1).

Mechanism The first heart sound is due to abrupt arrest of the leaflet motion of mitral and tricuspid valves.

Intensity of S1 1. Velocity of valve closure—This is the most important factor. If the speed with which the valve closes is rapid, then the sound is loud, e.g. mitral stenosis. 2. Integrity of valve closure—If the valve coaptation is not correct then the S1 is soft as seen with mitral regurgitation. 3. Mobility of the valve—If the valve is calcified then the S1 is soft. 4. Status of ventricular contraction—This relates to the rate of pressure development in the LV. Exercise, adrenaline, 34 Manual of Cardiology

high output states often associated with tachycardia leads to increase in the intensity of S1. S1 is soft in cardiomyopathy and in acute myocardial infarction. 5. Transmission characteristics of the thoracic cavity and the chest wall: Obesity, emphysema, large pleural or pericardial effusions decrease the intensity of S1.

Loud First Heart Sound First sound is said to be loud when it is louder than the second heart sound in the right second intercostal space, in the absence of aortic stenosis or hypertension. Causes 1. Mitral stenosis 2. Left atrial myxoma 3. Tachycardia 4. Hyperkinetic states, such as anemia, thyrotoxicosis 5. Short PR interval less than 160 msec. Mitral Stenosis The pressure gradient across the valve is increased and so the valve is kept open till the end of diastole, and so, when it closes it closes from a wide position, with increased velocity—Wide open door theory.

Soft First Heart Sound First heart sound is said to be soft when it is softer than S2 at the apex or left sternal border. Causes 1. Long PR interval—more than 200 msec 2. Depressed LV contractility 3. Pericardial effusion 4. Mitral regurgitation 5. LBBB. Heart Sounds 35

SECOND HEART SOUND The second heart sound was labeled by Leatham as the key to auscultation of the heart, is of frequency 120 to 150 Hz, and coincides with the down stroke of the carotid pulse. Two components, the earlier one is due to closure (A2) and the later one is due to pulmonary valve closure (P2).

Mechanism A second sound is produced by closure of the aortic and pulmonary valve.

Intensity/Loudness The second heart sound can be loud or soft depending on the underlying cause. In addition, the loudness may affect the individual components of second heart sound namely, aortic component—A2 or pulmonary component—P2. The following table summarises the various causes.

TABLE 6.1: Increased intensity of A2 1. Systemic hypertension (drum like or tambour S2) 2. Coarctation of aorta 3. Ascending aortic aneurysm 4. Relative anterior placement of the aorta Tetralogy of fallot Transposition of great arteries Decreased intensity of A2 1. Aortic regurgitation 2. Valvar and aortic stenosis The decrease in intensity of A2 is due to lack of apposition of leaflets and decrease in arterial diastolic pressure.

Increased intensity of P2 Normally P2 is not audible at the apex. If P2 is louder than A2 in 2nd left intercostal space or if it is audible at the apex, it is said to be loud and indicates elevated pulmonary pressures except in ASD where because of the enlarged right 36 Manual of Cardiology ventricle, P2 may be audible at the apex even in the absence of elevated PA pressures. However, if P2 is very loud or it increases after mild exercise, the presence of an ASD with pulmonary hypertension and elevated pulmonary vascular resistance (PVR) should be suspected.

Soft P2 1. Pulmonary stenosis. 2. Pulmonary regurgitation due to absent pulmonary valve.

Splitting of S2 Normally second sound is heard as two components in inspiration and in expiration, it is heard as a single sound. If split sounds are heard both in inspiration and in expiration it is called as split second heart sound. This splitting can be of the following types: 1. Persistent split. 2. Wide and fixed. 3. Narrow split and fixed. 4. Reversed split/Paradoxical split.

Persistent Split In this the second heart sound is heard as a two components both in inspiration and in expiration. However, the split widens slightly in inspiration compared to expiration. This is observed in the following conditions: 1. Pulmonary stenosis. 2. Right bundle branch block.

Wide and Fixed In this the second sound is heard as two components, in both inspiration and expiration. The split does not vary with respiration. This is heard in atrial septal defect. The reasons for the same are as follows: Heart Sounds 37

1. The split is wide because of the increased capacitance of pulmonary vasculature. 2. The split is fixed because the amount of flow through the PA does not vary with respiration. With inspiration there is increased venous return to the right side. The result is that there is corresponding reduction in the left to right shunt as well.

Reversed or Paradoxical Splitting In this, the second sound is single on inspiration and in expiration it is split. The commonest reason for the same is because A2 is delayed and occurs later than P2.

FIGURE 6.1: Paradoxical splitting Causes 1. Left bundle branch block 2. RV paced beats 3. LVOT obstruction 4. Angina 5. PDA.

Narrow Split In pulmonary hypertension the second heart sound is split closely and the respiratory variation is not much. This happens only with severe pulmonary hypertension. 38 Manual of Cardiology

FIGURE 6.2: Summary of splitting of second sound

THIRD HEART SOUND

Mechanism There are several theories regarding the mechanism of third heart sound. One theory states that it is due to impact of the ventricle on the chest wall. The second theory states that it is due to distension of the ventricular wall, and the papillary muscles. The most accepted is that it is due to the valve itself.

Gallop Rhythm The term Gallop is used if third heart sound is heard with the heart rate of 100 or more. This produces a triple sound that sounds like a horse galloping. Causes 1. Increased cardiac output • Anemia • Mitral regurgitation • Hyperthyroidism • VSD • Aortic regurgitation • PDA • Exercise • Pregnancy. Heart Sounds 39

2. Left ventricular failure

FIGURE 6.3: S3 is a low pitched sound just after the S2.

FOURTH HEART SOUND Sound generated during atrial filling phase within the recipient ventricle is called as the (S4). It is also called as atrial or presystolic gallop or triple rhythm. It is a low- pitched sound that varies considerably in intensity and heard better with the bell of the stethoscope with light pressure applied to form a skin seal.

Mechanism It is caused by increase in stiffness of the ventricles; atrial contraction as a consequence produces sudden increases in pressure in the ventricle, rather than the ventricle increasing in its volume to accommodate the extra blood delivered by atrial systole.

Left Ventricular S4 LV S4 is heard well at the point of maximal LV impulse with the patient in the left lateral position. It may be palpable in this position. It may even be seen by examining the movement of a lightweight pencil held over the point of maximal impulse. It can be induced or augmented when resistance of LV discharge is increased by sustained handgrip especially in presence of coronary artery disease.

Causes 1. Atrioventricular block In complete heart block, atrial gallop sounds are frequently heard. When atria and ventricles contract independently, 40 Manual of Cardiology

S4 or summation sounds occur randomly in diastole because relationship between P wave and QRS of ECG is random. Careful auscultation in a quite room is necessary to detect these sounds, which resemble faint footsteps on a carpet. 2. Coronary artery disease It is unusual not to hear a faint S4 in a patient who has had previous myocardial infarction (MI). S4 is louder during an episode of acute MI and pain or during initial phases of MI. 3. Cardiomyopathy 4. Systemic hypertension 5. Aortic stenosis S4 in Aortic stenosis occurs particularly in patients with higher degrees of obstruction. It correlates with systolic gradient of 70 mm of Hg across the aortic valve or a LVEDP of 15 mm of Hg. It may also be heard in AR. 6. Elderly Older patients may have S4 when there is minimal to moderate aortic valve gradient due to associated coronary artery disease. In patients under 40 years of age, there is better correlation of S4 with severity of AS.

FIGURE 6.4: S4 is a low pitched sound heard just before the first heart sound Acute Rheumatic Fever 41

Section II VALVULAR HEART DISEASE 42 Manual of Cardiology

The prevalence of valvular heart disease is on the decline. The main etiology of valvular heart disease in countries like India is still rheumatic fever. In the last 20 years or so, there has been a dramatic decline in the incidence of acute rheumatic fever which in turn has resulted in less number of patients having rheumatic valvular heart disease. With the advancing age of the population, degenerative valvular heart disease is on the increase. These changes results in narrowing of the aortic valve or leaking of the same. In this section, acute rheumatic fever and its effects on the valvular heart disease are described. In rheumatic heart disease the following valvular lesions are observed: • Mitral valve alone—50 percent. • Mitral and aortic valve—40 percent. • Mitral, aortic and tricuspid—5 percent. • Aortic valve alone—2 percent. • Other combinations—3 percent. Acute Rheumatic Fever 43

ACUTE RHEUMATIC FEVER 7

Rheumatic fever (RF) is generally classified as a connective tissue disease or collagen . Its anatomical hallmark is damage to collagen fibrils and to the ground substance of connective tissue. The clinical manifestations of acute RF follow a group A streptococcal infection of the throat after a latent period of approximately three weeks.

GABHS—GROUP A BETA HEMOLYTIC STREPTOCOCCI • Streptococci contain certain antigenic determinations that cross react with various tissues in the body. • Cross reactivity or antigenic mimicry in RF Cell wall M protein—myocardium Carbohydrate—valvular glycoprotein Protoplast membrane—subthalamic nucleus and caudate • M type of 1,3,5,6,14,18,19,27 and 29 are rheumatogenic. • The attack rate of rheumatic fever: 3 percent in an epidemic. otherwise it is around 0.3 percent. • A strong relationship has been found with a B-cell antigen designated 883; this is found in patients with rheumatic fever suggesting that there could be a genetic predisposition.

Aschoff’s Body This was described in the year 1904. It is made up of perivascular infiltrate of large cells with polymorphous nuclei and basophilic cytoplasm arranged in a rosette around an 44 Manual of Cardiology avascular center of fibrinoid. Some of the cells may be multi nucleated or may have an owl eyed nucleus with an eccentric dot and fibrillae radiating to the nuclear membrane or caterpillar nucleus. These cells are called as Antischkow monocyte. Aschoff’s bodies are present in the myocardium; these are most marked in the interventricular septum and left atrial appendage. It is not present in areas such as brain or joints.

Prevalence of Rheumatic Heart Disease in India In order to understand the prevalence of rheumatic heart disease, we can get data either from school surveys or from hospital admissions. The Table 7.1 gives the school survey data regarding the prevalence of rheumatic heart disease in India. The prevalence varies from 0.68 to 4.54 per thousand school children.

TABLE 7.1: School survey data of rheumatic heart disease from India Author Place Year Age Population Prevalence/ studied 1000 ICMR 1 Vellore 1982-1990 5-15 13509 2.9 Padmavati 2 Delhi (urban) 1984-1994 5-10 40000 3.9 Grover 3 Raipurrani 1988-1991 5-15 31200 2.1 Avasthi 4 Ludhiana 1987 6-16 6005 1.3 Patel 5 Anand 1986 8-18 11346 2.03 Lalchandani 6 Kanpur 2000 7-15 3963 4.54 Jacob Jose7 Vellore 2003 5-18 229829 0.68

Diagnosis of Acute Rheumatic Fever For the diagnosis of acute rheumatic fever, we have been following the Jone’s criteria, since 1944. However, there are many patients who do not fulfill the criteria of the Jone’s criteria and presently, a new dimension has been added with advent of . The sensitivity of Jone’s criteria is 77 percent and the specificity is 97 percent. Acute Rheumatic Fever 45

The current guideline was put forward in the year 1992 as an update. These criteria are to be used only for the initial attacks of rheumatic fever. This does not apply for patients with past history of rheumatic fever or rheumatic heart disease.

TABLE 7.2: 1992 updated: For the diagnosis of initial attacks of rheumatic fever8 Major manifestations Minor manifestations 1. Carditis Clinical findings 2. Polyarthritis 1. Arthralgia 3. Chorea 2. Fever 4. Erythema marginatum Laboratory findings 5. Subcutaneous nodules 3. Elevated acute phase reactants Erythrocyte sedimentation rate C-reactive protein 4. Prolonged PR interval

If supported by evidence of preceding group A strep infection (a positive throat culture or elevated or rising strep antibody titer), the presence of two major manifestations or of one major and two minor manifestations indicates a high probability of acute rheumatic fever.

Exceptions to Jone’s Criteria: 1. Chorea. 2. Indolent carditis. 3. Recurrent rheumatic fever. 4. Post streptococcal arthritis. With the above four conditions Jone’s criteria do not apply.

TABLE 7.3: WHO criteria for the diagnosis of rheumatic fever in RHD First episode 2 major or 1 major and 2 minor Recurrent without RHD 2 major or 1 major + 2 minor Recurrent with RHD 2 minor with preceding strep infection Rheumatic chorea Preceding strep infection not required WHO criteria is useful for the diagnosis of recurrent rheumatic fever in patients with rheumatic heart disease. 46 Manual of Cardiology

ARTHRITIS 1. Occurs in 70 percent of cases. 2. Asymmetrical and migratory in nature. 3. Large joint arthritis (Knees, ankles, elbows and wrist are involved). 4. Pain, swelling, heat, and redness are noted. 5. Lasts for 2 to 3 weeks. 6. Rapid response to salicylates within 48 hours. 7. No residual deformity. Jaccoud’s arthritis: This is a permanent deformity of small joints, secondary to rheumatic fever, which is very rare.

CARDITIS Carditis occurs in 50 percent of patients with acute rheumatic fever. In India the incidence of carditis is higher than the western data (range 70-86%). Since carditis includes some or one of the following, the features are given in increasing order of severity. 1. Tachycardia (out of proportion to the degree of fever) is common; its absence makes the diagnosis of myocarditis unlikely. 2. A of valvulitis (caused by mitral regurgitation (MR) and/or aortic regurgitation (AR)) is almost always present; without the murmurs of MR and/or AR, carditis should not be diagnosed. 3. Pericarditis (friction rub, pericardial effusion, chest pain, and ECG changes) may be present. 4. Cardiomegaly on chest X-ray films is indicative of pericarditis, pancarditis, or congestive heart failure (CHF). 5. Signs of congestive heart failure (, distant heart sounds, cardiomegaly) are indications of severe carditis. Acute Rheumatic Fever 47

Valve involvement in acute rheumatic fever: • Mitral—70 to 75 percent • Mitral and Aortic—20 to 25 percent • Aortic—Isolated 5 percent Carditis—role of echo: It is difficult to clinically diagnose mild carditis. Echo is a valuable tool for the diagnosis of a valvular lesion. Echo is capable of evaluating the presence and degree of mitral and aortic regurgitation. The first report regarding the role of echo was by Steinfield in the year 1986. He found that Doppler showed mitral regurgitation even when there was clinically no murmur.9 Echo Doppler is extremely useful in the diagnosis of carditis by identifying valvular disease even in patients without clinical evidence of carditis. Echo can also identify the presence of pericardial effusion and in some cases identify the fine rheumatic valvar vegetations. However, echo should not be used as sole criteria for the diagnosis of carditis.

CHOREA Also called as Sydenham’s chorea, or St. Vitus dance is the third most common major manifestation. It occurs in 15 percent of patients in recent outbreaks; however, the overall figure may be around 5 percent or less. The period of latency between the GABHS infection and the onset of chorea is around three months. Chorea can occur in two circumstances. 1. As an isolated manifestation of rheumatic fever and frequently recurs following a new bout of streptococcal pharyngitis. This is called as Pure chorea. 2. It may occur as a part of otherwise active rheumatic fever with manifestations such as joint pains, etc. After puberty, chorea is almost entirely seen only in women. It may last for a few weeks to few months. It seems to be due to 48 Manual of Cardiology immune mediated reaction to autoantibodies of the basal ganglia. Severe chorea responds to treatment with intravenous IgG. Chorea is closely related to Postinfectious Autoimmune Neurological Diseases (PANDA). These manifestations include Tic, Tourettes syndrome and obsessive—compulsive behavior, all of which are also observed in some patients with chorea. The clinical findings include involuntary and purposeless movements with muscle in co-ordination of the extremities and labile mood.

Signs 1. Hyperextension of the fingers, spooning when the arms are extended. 2. Pronation of hands when arms are raised vertically. 3. Milkmaid’s grip—irregular contraction of the hand muscles when the patient presses the hand of the examiner. 4. Wormian tongue—gross fasciculations of the tongue when extended. 5. Clumsiness in fine movements such as buttoning of the shirt.

Duration Usually self limited and last for 2 to 4 weeks; can last for a few months to two years.

Sequlae 1. Studies suggest that many patients with chorea may eventually have obsessive—compulsive behavior. No residual nerurological deficit is seen in most patients. 2. When chorea is associated with other signs of RF, the incidence of valve damage is comparable to that caused by other patients without chorea. 3. When chorea is an isolated event—pure chorea, the valve damage is less frequent and mitral stenosis is the late manifestation. Acute Rheumatic Fever 49

Treatment of Chorea 1. Phenobarbitol 15 to 30 mg every 6th hourly. 2. Haloperidol—2 mg every 8th hourly or as needed. 3. Valproate—20 mg/kg/day.

PRECEDING GROUP A STREP. INFECTION At the time of diagnosis of acute RF, only about 11 percent of patients have positive throat culture. Hence, throat culture is less reliable than streptococcal antibody tests. Antistreptolysin O (ASO) titer is the one that is used routinely. It is elevated in 80 percent of patients with acute rheumatic fever and in 20 percent of normal individuals. Only 67 percent of patients with isolated chorea have an elevated ASO titer. ASO titer of at least 333 Todd units in children and 250 Todd units in adults are considered as elevated. If the clinical suspicion is high but the ASO titer is low, this does not exclude the diagnosis of acute rheumatic fever. You must do one more test such as antideoxyribonuclease B test (ADNB) or repeat the ASO titer after a week. A rising titer of ASO can be taken as evidence for acute rheumatic fever. Streptozyme test is relatively simple agglutination test but it is less standardized and less reproducible than the other antibody test. It should not be used as a definitive test for evidence of preceding group A streptococcal infection.

Treatment 1. Eradication of streptococci: Injection Benzathine Penicillin G is given intramuscularly. The dose is 0.6 million units if the weight is < 27 kg or 1.2 million units if the weight is > 27 kg. In patients allergic to penicillin, erythromycin 40 mg/kg/ day is given in two or three divided doses for a period of 10 days. 50 Manual of Cardiology

2. Anti-inflammatory drugs: Salicylates or Steroids must not be started until a definitive diagnosis is made. Early therapy with these drugs may interfere with definitive diagnosis of acute rheumatic fever. a. Prednisolone is used in a dose of 2 mg/kg/day in 4 divided doses for a period of 2 to 6 weeks in cases with severe carditis. b. For mild moderate carditis aspirin can be used in a dose of 90 to 100 mg/kg/day in 4 to 6 divided doses. This dose is continued for a period of 4 to 8 weeks depending on the clinical response. After improvement, the therapy is withdrawn gradually over 4 to 6 weeks. c. For arthritis alone: Aspirin is continued for two weeks and gradually withdrawn for a period of three weeks. In acute rheumatic fever there is rapid resolution of joint symptoms within 24 to 36 hours.

TABLE 7.4: Duration of anti-inflammatory agents Clinical manifestation Prednisolone Aspirin Arthritis alone 0 1-2 weeks Mild carditis 0 3-4 weeks Moderate carditis 0 6-8 weeks Severe carditis 2-6 weeks * 6 weeks to 4 months

* For severe carditis, prednisolone should be tapered and aspirin be started during the final week and the total duration of anti-inflammatory therapy can be between 6 weeks to 4 months, depending on the need.

3. Bed rest of varying duration is recommended. The duration depends on the severity of the clinical manifestation. ESR is a helpful guide to the rheumatic activity and, therefore, the duration of restriction of activities. The following table will give you a general guideline for bed rest/restricted ambulation period. Acute Rheumatic Fever 51

TABLE 7.5: General guidelines of restricted activities in acute rheumatic fever Clinical feature Duration of bed rest/limited ambulation Arthritis alone Only 2 weeks Mild carditis 3-4 weeks Moderate carditis (definite 4-6 weeks but mild cardiomegaly) Severe carditis (marked Bed rest as long as patient has heart cardiomegaly, cardiac failure and indoor ambulation for a period failure, pericardial effusion) of 2-3 months

4. Secondary Prevention of Rheumatic Fever: The following Tables give the dose and the duration for secondary prophylaxis.

TABLE 7.6: Drugs used in secondary prophylaxis Drug Dose Route 1. Benzathine Penicillin G 12,00,000 units every 3-4 weeks IM 2. Penicillin V 250 mg twice daily PO 3. Sulfadiazine 0.5 gm once daily for patients PO < 27 kg (60lb) 1.0 gm once daily for patients > 27 kg (60lb). 4. Allergic to penicillin Erythromycin 250 mg twice daily PO and sulfadiazine: Erythromycin

TABLE 7.7: Duration of secondary prophylaxis CATEGORY DURATION 1. RF with carditis At least 10 years after last episode and residual valvular disease and at least until age 40. Some times lifelong prophylaxis 2. RF with carditis but not 10 years or up to 21 years of age residual valvular disease 3. RF without carditis 5 years or up to 21 years of age, whichever is longer 52 Manual of Cardiology

Rheumatic Fever Recurrence Rates using Drugs • Three weekly benzathine penicillin 0.25/100 person years • Four weekly benzathine penicillin 1.29/100 person years • Sulfadiazine 2.8/100 person years • Oral penicillin 5.5/100 person years Since risk of recurrence is higher with oral penicillin, it is usually given for individuals, who have reached young adulthood and remained free of rheumatic attacks for at least five years.

CONCLUSION Acute rheumatic fever and rheumatic heart disease still forms a major health problem in countries like India. Early diagnosis and proper treatment of the disorder will prevent the marked disability left behind by this disease.

FURTHER READING 1. Avasthi G, Singh D, Singh C, Aggarwal SP, Bidwai PS, Avasthi R. Prevalence survey of rheumatic fever (RF) and rheumatic heart disease (RHD) in urban and rural school children in Ludhiana. Indian Heart J 1987;39:26-28. 2. Dajani AS, Ayoub EM, Bierman FZ, et al: Guidelines for the diagnosis of rheumatic fever: Jones Criteria, updated 1992. JAMA 268:2069. 3. Grover A, Dhawan A, Iyengar SD, Anand IS, Wahi PL, Ganguly NK. Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India. Bull World Health Organ 1993;71:59-66. 4. Jose et al. Declining Prevalence of Rheumatic Heart Disease in Rural School children in India:2001-2002: Indian Heart Journal - March-April 2003;158-60. 5. Lalchandani A, Kumar HRP, Alam SM. Prevalence of rheumatic fever and rheumatic heart disease in rural and urban school children of district Kanpur [Abstr]. Indian Heart 2000;52:672. 6. Padmavati S. Present status of rheumatic fever and rheumatic heart disease in India. Indian Heart J 1995;47:395-98. Acute Rheumatic Fever 53

7. Patel DC, Pael NI, Patel JD, Patel SD. Rheumatic fever and rheumatic heart disease in school children of Anand. J Assoc Physicians India 1986;34:837-39. 8. Pilot study on the feasibility of utilizing the existing school health services in Delhi for the control of RF/RHD. ICMR final report, 1990. 9. Steinfield, Ritter S, Rapport H. Martinez E-Silent Rheumatic Mitral regurgitation unmasked by Doppler studies. Abstract—Circulation 1986;(74 Suppl-11):385. 54 HandbookManual of Cardiology of Cardiology for Undergraduates

MITRAL STENOSIS 8

ETIOLOGY 1. Rheumatic (common) 2. Congenital (less frequent) 3. Rarely: a. Rheumatoid b. SLE c. Hunter-Hurler d. Malignant carcinoid e. Methysergide therapy.

PATHOPHYSIOLOGY Mitral stenosis is produced due to fusion of the mitral valve apparatus. Fusion occurs at multiple levels as shown below: - Commissural fusion 30 percent - Cuspal fusion 15 percent - Chordal fusion 10 percent - All of the above 45 percent As a result of mitral stenosis the left atrial pressure is increased. There is a pressure gradient between the left atrium and the left ventricle in diastole (Figure 8.1).

Features of LA Tracing 1. Mean LA pressure increased 2. Slow ‘y’ descent 3. Prominent ‘a’ wave Mitral Stenosis 55

FIGURE 8.1: Simultaneous left ventricle and LA, pressure tracing is shown. The shaded area represents the gradient between the LA and LV in diastole The normal mitral valve area is around 4 to 6 sq cm. When the valve area is narrow to 2.5 or less, we call it as mitral stenosis. The Table below shows the relationship between the valve area and the severity of mitral stenosis.

TABLE 8.1: Severity of mitral stenosis in relation to valve area Valve Area cm2 Severity 2.1 to 2.5 Very mild 1.6 to 2 Mild 1.0 to 1.5 Moderate < 1 cm Severe 1.2 cm or less Pulmonary congestion 0.3 to 0.4 Smallest area compatible with life.

Valve Area and Gradients At a MVA of 1 cm2, the mitral diastolic gradient to maintain normal cardiac output at rest is around 20 mmHg. The Table 8.2 shows the relation between valve area and gradients.

TABLE 8.2: Severity of mitral stenosis in relation to gradient Severity Gradient mm of Hg Valve area sqcm Mild < 5 > 1.5 Moderate 5-10 1.0-1.5 Severe > 10 < 1.0 56 Manual of Cardiology

Pulmonary Hypertension Pulmonary hypertension can develop in mitral stenosis due to the following two reasons: 1. Passive—this is due to backward transmission of elevated LA pressure 2. Reactive—the PA pressure is elevated much more than what is expected due to passive transmission of the elevated LA pressure; this occurs when the LA mean is at least 20 mmHg.

ATRIAL FIBRILLATION1

TABLE 8.3: Relationship between age and atrial fibrillation Age in years Incidence percent 11 - 20 0 21 - 30 17 31 - 40 45 41 - 50 60 > 51 80

History 1. Dyspnea: This symptom is due to pulmonary congestion. 2. Hemoptysis: There are several reasons for the same and they are given below: a. Acute pulmonary edema. b. Paroxysmal nocturnal dyspnea. c. Pulmonary infarction. d. Bronchitis. e. Pulmonary apoplexy—due to rupture of small bronchial vein. 3. Chest pain: It occurs in 15 percent of patients and clinically resembles angina due to coronary artery disease. a. Right ventricular ischemia secondary to pulmonary hypertension. b. Associated coronary artery disease. c. Coronary embolism. Mitral Stenosis 57

4. Hoarseness of voice (ORTNER’s syndrome): This is due to compression of the left recurrent laryngeal nerve due to dilated pulmonary artery. 5. Embolic manifestations: This can be in the form of embolism to the cerebral vessels manifesting as cerebro- vascular accident or to the periphery—ischemic limb. There is a direct correlation between age of the patient, left atrial size and atrial fibrillation in developing embolic symptoms. More than 50 percent of all emboli lodge in cerebral vessels. Emboli are recurrent in 25 percent of patients.

AUSCULTATION In mitral stenosis there are four important auscultatory findings: 1. Loud first heart sound. 2. Opening snap. 3. Mid-diastolic murmur. 4. .

Loud First Heart Sound S1 is loud because the elevated LA pressure keeps the mitral valve opened for a long time till the end of diastole and as a result when the valve closes it has a wider excursion. Loudness and calcification: A loud first heart sound indicates that the valve is pliable and it is non-calcific. When the valve is calcified the first heart sound may be soft. Calcification limited to the posterior mitral leaflet will not alter the loudness of the first heart sound if the body of the anterior mitral leaflet is mobile.

Opening Snap Mechanism: Sudden tensing of the anterior mitral leaflet after the valve cusps have completed their opening excursion. 58 Manual of Cardiology

S2-OS Interval: With increasing severity of mitral stenosis the opening snap becomes closer to the second heart sound and this is called as narrow 2-OS interval (< 0.08 secs).

FIGURE 8.2: First heart sound is loud; after the opening snap, the murmur starts in diastole and has presystolic component as well

TABLE 8.4: S2-OS interval and its relationship to the severity of mitral stenosis 2 Severity S2-OS interval Mild 100-120 msec Moderate 80-100 msec Severe < 80 msec

The following are the reasons for a long 2-OS interval: 1. Mitral stenosis is mild. 2. Hypertension. 3. . 4. Poor LV function.

Differentiating Split second heart sound (A2—P2) from 2-OS sounds • Standing: 2-OS widens • Inspiration: 2-OS widens, can be heard as triple sound— A2, P2 and opening snap • Exercise: 2-OS shortens. Mitral Stenosis 59

Mid Diastolic Murmur This is the third auscultatory feature of mitral stenosis: Site: Best heard at the apex. Pitch: Low pitched. Conduction: None. Character: Rough and rumbling. Loudness: May be associated with thrill. Exercise: The murmur increases. Posture: The murmur is best heard in the left lateral position and decreases during standing. Valsalva: The murmur may disappear or decrease in intensity. Respiration: With inspiration sometimes the heart rate increases and the murmur may get exaggerated (false positive CARVALLO’s sign). Usually the murmur is best heard in expiration because of increasing venous return.

Differential Diagnosis of Mid-Diastolic Murmur 1. Carey Coombs. 2. Austin Flint. 3. LA Myxoma. : This is the short mid diastolic murmur heard in acute rheumatic fever. S1 is not loud and this murmur is never associated with the thrill. (AFM): This murmur is present in patients with severe aortic regurgitation. The differentiating points are given below:

TABLE 8.5: Differences between Mitral MDM and AFM Mitral stenosis Austin flint S1 loud Present No P2 loud Present Rare S3 Never May be present Opening snap Present No Amyl nitrate Increases Decreases 60 Manual of Cardiology

Left atrial myxoma: The murmur varies with positional changes. In addition there will be a tumor plop. Left atrial myxoma is usually occurs with systemic symptoms such as fever, arthralgia.

Pre-systolic Murmur (PSM) This murmur is heard in patients with mitral stenosis if the pressure gradient exceeds 3 mmHg at least in the end of diastole. Usually it is absent in atrial fibrillation, can be present in patients with severe MS.

Chest X-ray (See Figure 37.1) Cardiac silhoutte 1. Left atrial enlargement 2. Pulmonary artery is prominent 3. Right ventricle and right atrial enlargement. Lung fields 1. Kerley B lines 2. Kerley A lines 3. Upper lobe veins are prominent (Antler’s sign) 4. Pulmonary hemosiderosis 5. Pulmonary calcification.

ECG 1. Left atrial enlargement. 2. Right ventricular hypertrophy. 3. Right axis deviation. 4. Atrial fibrillation.

Echo This is the investigation of choice for the diagnosis and for assessing the severity of mitral stenosis. 2D Echo shows that the mitral valve is narrowed and the valve domes in diastole Mitral Stenosis 61 giving a picture of Hockey stick appearance. Doppler Echo will be useful in assessing the pressure gradients across the valve and from this the valve area can be calculated.

FIGURE 8.3: The figure on the left is a long axis view showing dilated left atrium and dooming of the mitral valve. The figure on the right shows the short axis view in which the valve area is measured. In this patient the valve area is 1.2 sq cm

Echo is useful in the differential diagnosis of mitral stenosis. Shown below is the image taken from a 2D Echo with left atrial myxoma.

FIGURE 8.4: Apical 4 chamber view showing a mass lesion in left atrium attached to the interatrial septum

Natural History It usually takes 5 to 10 years for patients to progress from mild to severe symptoms. In countries like India, the progression can be more rapid and mitral stenosis can develop within two years 62 Manual of Cardiology after the onset of acute rheumatic fever. The term Juvenile mitral stenosis is used if mitral stenosis is observed before 19 years of age. In one study from Greece, the mean interval from acute RF to appearance of symptoms was 16.3 ± 5.2 years and progression of mild to severe disability was 9.2 ± 4.3 years on an average. Two Echo follow-up studies suggest that the valve area decreases on an average of 0.09 cm2/year

Management 1. Rheumatic fever prophylaxis. 2. Infective endocarditis prophylaxis. 3. Drugs to control pulmonary congestion—diuretics 4. Rate reducing drugs such as beta blocker or verapamil and digoxin for patients with atrial fibrillation. 5. Anticoagulation for patients with atrial fibrillation. The risk of embolic stroke varies from 7 to 15 percent per year. 6. Balloon mitral valvotomy or mitral valve replace- ment.

TABLE 8.6: Indications for surgery or balloon mitral valvotomy3 INDICATION 1. Class II, III, or IV with MVA < 1.5 sq cm2 2. Asymptomatic patients with MVA < 1.5 sq cm with PA pressure > 50 mmHg at rest or 60 mmHg with exercise 3. Asymptomatic patients MVA < 1.5 sq cm2 with new onset atrial fibrillation Absolute Contraindications for BMV: 1. LA thrombus 2. Calcified mitral valve 3. Moderate mitral regurgitation (> 2 + MR) Results of balloon mitral valvotomy from India4 Between July 1997 and January 2002, one thousand one hundred and thirty percutaneous balloon mitral valvotomy Mitral Stenosis 63

(BMV) procedure were performed at CMCH. Based on that, the following were the major complications observed. 1. Death 0.5 percent 2. Cardiac tamponade 0.6 percent 3. Severe MR 2.6 percent 4. MVR 0.9 percent 5. Stroke, TIA 0.1 percent Arora et al5 reported their data in a group of 4850 patients. The mortality was 0.2 percent, cardiac tamponade 0.2 percent, embolism 0.1 percent, mitral regurgitation severe 1.4 percent, restenosis was observed in 4.8 percent of these patients on a 94 months follow-up.

Mitral Valve Replacement Patients with significant valve calcification or with left atrial clots may require valve replacement instead of balloon mitral valvotomy.

REFERENCES 1. Deverall PB, et al. Incidence of systemic embolism before and after mitral valvotomy. Thorax 1968;23:530. 2. Carabello. Modern management of mitral stenosis. Circulation 2005;112:432-48. 3. Bonow RA, et al: ACC/AHA guidelines. J Am Coll Cardiol 1998;32:1486-588. 4. Jomiva Balloon Catheter Instruction Manual, second edition 2002;23-24. 5. Arora, et al. Results of Balloon Mitral Valvotomy. Cardiac Catheter Interventions 2002;55:450-56. 64 Manual of Cardiology

MITRAL REGURGITATION 9

ETIOLOGY 1. Leaflet abnormalities: a. Rheumatic. b. Infective endocarditis. 2. Annulus: a. Dilated cardiomyopathy. b. LV aneurysm. c. Calcification. 3. Chordal Rupture: Rheumatic, infective endocarditis or spontaneous. 4. Papillary muscle: Ischemic, infiltrative diseases such as amylodosis, sarcoidosis.

PATHOPHYSIOLOGY 1. The regurgitation of blood from left ventricle to left atrium begins early in systole. The result is that 50 percent of blood that is leaking back enters the LA even before the aortic valve opens. 2. The volume of regurgitation depends on: a. Regurgitant orifice area. b. Pressure difference between left ventricle and left artery. 3. The left ventricle compensates by: a. Increased contractility. b. Dilatation. c. Eccentric ventricular hypertrophy. Mitral Regurgitation 65

This compensation is maintained for several years, before the left ventricle fails and the contractility decreases. 4. LV ejection fraction: Because LV is able to eject into aorta and LA (2 outlets), the load on the left ventricle to eject is less (after load). The result is that there is an increase in the ejection phase indices of contractility—as ejection fraction. Hence in MR, EF is actually increased compared to normal. If the ejection fraction is in the lower side such as 0.40 to 0.50, (normal > 50%) the LV contractility should be considered to be greatly depressed.

SYMPTOMS 1. Usually asymptomatic for a long time. 2. Symptoms of LV failure develop late in the course of MR, unless the mitral regurgitation has been acute. The symptoms are: a. Fatigue—low cardiac output. b. Palpitation—increased contractility. c. Dyspnea—pulmonary congestion.

PRECORDIAL MOTION 1. LV impulse is hyperdynamic with a normal contour and increased amplitude of early systolic outward motion.

Figure 9.1: Apical impulse movement: The apex beat moves up quickly and falls rapidly-hyperdynamic in character 66 Manual of Cardiology

2. Late systolic parasternal lift—due to left atrial enlargement. 3. Parasternal heave of RVH—develops later secondary to pulmonary hypertension.

Figure 9.2: Timing of parasternal lifts: In mitral regurgitation the left is late systolic in timining

HEART SOUNDS 1. First heart sound: The most frequent abnormality is a decrease in S1 amplitude. 2. Second heart sound: In severe cases it is audibly split in expiration, because of a shortened LV ejection time. 3. Third heart sound: Is common in MR of hemodynamic significance; it does not indicate LV dysfunction. 4. Fourth heart sound: Is never a feature of rheumatic mitral valve disease. The LA of chronic MR any etiology is dilated and compliant and is unable to generate an atrial sound or S4. In acute MR, it can produce S4.

MURMURS Pan Systolic Murmur This murmur is the typical auscultatory finding. It may have the following configuration: 1. Holo systolic with plateau configuration. Mitral Regurgitation 67

2. Late systolic murmur—this is seen in papillary muscle dysfunction because the papillary muscle is unable to sustain the valve competency throughout systole. 3. Late systolic taper—in severe acute mitral regurgitation the murmur decreases in late systole because of large left atrial V wave that reduces the regurgitation. 4. Mid systolic accentuation—ejection systolic murmur In RHD, the most common variant is mid or late systolic accentuation of the murmur. In more severe MR, murmur is more likely to have a mid systolic accentuation giving it a spindle shaped or ejection quality. In all these situations, careful auscultation usually will identify sound vibrations at the beginning and at end systole, which confirm that the murmur is truly regurgitant in quality as opposed to a more common ejection systolic murmur.

FIGURE 9.3: Note the high frequency pan systolic murmur, that extends from first to second heart sound. In addition there is a third heart sound which is followed by a mid diastolic murmur (MDM)

Conditions that may decrease the intensity of MR murmur: 1. CHF or LV dysfunction. 2. Low output states. 3. Associated MS. 4. Huge LA. 5. Obesity, thick chest wall, etc. 68 Manual of Cardiology

Bedside clues to severe MR 1. Rhythm: Atrial fibrillation. 2. Carotid pulse: Small volume quick rising. 3. LV impulse is hyperdynamic and displaced. 4. LA impulse—in the parasternal area. 5. S2 is wide split. 6. S3 is present. 7. Murmur very loud (this is not very useful).

Left Decubitus Position Will usually augment the murmur of MR and often increase its intensity by one to two grades. In addition this manoeuver occasionally accentuates the holo-systolic murmur of a late systolic tapering murmur. Use this position when an apical murmur is low amplitude and long duration.

Chest X-ray 1. Cardiomegaly, LV contour. 2. Left atrial enlargement. 3. Pulmonary venous hypertension.

ECG 1. Left ventricular hypertrophy. 2. Left atrial enlargement. 3. Right ventricular hypertrophy in 15 percent.

Echo 1. Color Doppler is the method of choice. It can precisely quantify the severity of mitral regurgitation. 2. 2D Echo will give information regarding the size and contractility of left ventricle. 3. 2D Echo will also be useful in measuring the left atrial enlargement. Mitral Regurgitation 69

Doppler Indicators of Severe MR 1. Color jet area of > 8 sq cm (> 1/3 LA area). 2. Vena contracta 5 mm. 3. Effective regurgitation orifice (ERO) area > 0.4 sq cm. 4. CW Doppler shows dense signal. 5. Pulmonary vein shows systolic flow reversal. 6. Transmitral E velocity > 1.5 m/sec.

Indications for Surgery Definite Indications: 1. NYHA class 3 or 4. 2. EF less than 60 percent. 3. LV dimension in systole > 45 mm by Echo. Emerging Indications 1. Flail mitral leaflet. 2. LA size > 45 mm by Echo. 3. Paroxysmal AF. 4. Exercise PA pressure of > 60 mmHg or resting PA pressure > 50 mmHg (Doppler method).

MEDICAL MANAGEMENT 1. Rheumatic fever prophylaxis. 2. Infective endocarditis prophylaxis. 3. Heart failure treatment with digoxin and diuretics. 4. Role of ACE inhibitors: These drugs are indicated if the patient is symptomatic or LV dysfunction is present. Currently, there is lack of convincing data that ACE inhibitors alter the course of MR and, hence, they are not recommended for chronic therapy except as mentioned above. 70 Manual of Cardiology

AORTIC STENOSIS 10

In aortic stenosis, there is obstruction to outflow of blood from left ventricle to the aorta. This obstruction may be at the valve, above the valve-supra-valvar or below the valve-sub-valvar.

ETIOLOGY OF VALVAR AS 1. Congenital. 2. Rheumatic. 3. Calcific (degenerative). 4. Rarely (homozygous hyperlipidemia; rheumatoid; ochronosis). Congenital AS: The valve may be unicuspid, bicuspid or tricuspid. Unicuspid valves produce severe AS in infancy. Tricuspid valves develop AS over a period of time and they manifest only at 30 years of age or more. Rheumatic AS: Results from adhesions and fusion of the commissures and cusps. Rheumatic AS is always associated with regurgitation of aortic valve and involvement of the mitral valve.

PATHOPHYSIOLOGY 1. LV systolic pressure is increased. 2. LV ejection time is prolonged. 3. LV end diastolic pressure is increased. 4. Aortic mean pressure is decreased. 5. There is a gradient between the left ventricle and aorta. Aortic Stenosis 71

TABLE 10.1: Grading of aortic stenosis Mean Gradient mmHg AVA sq cm Mild < 25 > 1.5 Moderate 25-50 1.1-1.5 Severe > 50 < 1.0 Critical > 80 < 0.75

History 1. Angina—angina is due to supply demand mismatch • LVH—increases the demand • Reduced aortic mean pressure, reduces the supply • Associated coronary artery disease-decreases the supply. 2. Syncope • On effort is due to fixed cardiac output • At rest is due to ventricle arrhythmias. 3. Dyspnea is a late manifestation and suggests of LV dysfunction.

Pulse Rate of rise is slow, peak is reduced—pulsus parvus et tardus; this is seen only with severe aortic stenosis.

Blood Pressure Systolic BP will be low in severe aortic stenosis.

Apex beat Heaving and sustained in character. The apex beat is not usually displaced because hypertrophy does not produce noticeable increase in cardiac size.

Auscultation 1. Ejection sound: This is a sharp high pitched sound heard in patients with valvar aortic stenosis in a mobile valve. Hence, it is commonly heard only in children and adolescents. 72 Manual of Cardiology

2. Ejection systolic murmur: This is the murmur of aortic stenosis. This murmur is medium pitched, rough, rasping and peaks late in systole. This murmur is best heard in the 2nd right intercostal space and gets conducted to the carotids. 3. Second heart sound: The aortic component of the second heart sound is diminished in intensity and is often absent. 4. S4 is heard in severe aortic stenosis. 5. S3 is heard, if there is LV dysfunction. 6. Paradoxical split of second heart sound: This is seen in patients with severe aortic stenosis. During inspiration the second sound appears single and on expiration the sound is split and heard as two components. This is the reverse of normal split.

FIGURE 10.1: Shows aortic ejection click (EC). The second sound is diminished intensity. The most prominent feature on auscultation is ejection systolic murmur (ESM).

Chest X-ray Normal sized heart with a dilated ascending aorta (post stenotic dilatation of aorta) calcification of the valve may be seen in fluoroscopy (Figure 10.2).

ECG LVH with strain pattern. (depressed ST segment and T inversion). Aortic Stenosis 73

FIGURE 10.2: Shows slightly enlarged cardiac size with prominent ascending aorta which forms the right upper border of the cardiac shadow

FIGURE 10.3: Show that the ascending aorta in aortic stenosis is dilated; a normal aorta is shown on the left

Echo The valve is thickened, immobile; calcification may be present. 2D Echo is also useful in assessing of the LV size and function in a given case of aortic stenosis. Doppler Echo will be able to assess the gradient across the valve and the valve area.

Cardiac Cath and Coronary Angio Cardiac cath can demonstrate the gradient across the valve. Since, Echo-Doppler can demonstrate the gradient non- 74 Manual of Cardiology invasively, cardiac cath is not done nowadays to assess the gradient. Cath is done only to demonstrate the coronary arteries. The later is required in all aortic stenosis patients undergoing surgery, if they are above the age of 35 years.

Natural History Symptoms to death Angina 5 years Syncope 3 years Dyspnea 2 years

Medical Management 1. a. Antibiotic prophylaxis. b. Rheumatic fever prophylaxis. c. Infective endocarditis prophylaxis. 2. Activities-restricted in severe aortic stenosis. 3. Cardiac medications. Digoxin and diuretics are given only when LV dysfunction is present.

Indications for Surgery 1. Symptomatic. 2. Asymptomatic with LV dysfunction, or abnormal response to exercise with hypotension.

Other Causes of LVOT Obstruction Supravalvar Aortic Stenosis In this condition there is a membrane above the aortic valve. Often this is associated with mental retardation and hypercalcemia—William’s syndrome.

Subaortic Stenosis This is a congenital condition in which there is a membrane or fibrous ridge below the aortic valve. Aortic Stenosis 75

Hypertrophic Cardiomyopathy In this condition the interventricular septum is thickened and mitral valve moves towards the septum during systole (SAM) to result in LVOT obstruction. 76 Manual of Cardiology

AORTIC REGURGITATION 11

Aortic Regurgitation results from failure of leaflet to coapt, which can be as a result of disease in the valve cusps or disease in the aortic root; AR can be acute or chronic.

ETIOLOGY 1. Valvular Disease 1. Rheumatic: Cusps infiltrated with fibrous tissue, retract. 2. Infective Endocarditis: Perforation/vegetation interfere with valve closure. 3. Trauma: Tear/loss of commissural support. 4. Bicuspid: Incomplete closure/prolapse. 2. Aortic Root Disease: In these disorders, the annulus is dilated and the cusps are unable to close. 1. Annulo aortic ectasia 2. Ankylosing spondylitis 3. Reiters 4. Psoaritic arthritis 5. Marfans, Osteogenetic imperfect 6. Rheumatoid arthritis. Acute Aortic Regurgitation 1. Acute rheumatic fever 2. Infective endocarditis 3. Dissection of aorta 4. Rupture of sinus of valsalva aneurysm. Aortic Regurgitation 77

PATHOPHYSIOLOGY In chronic aortic regurgitation there is a large increase in the end diastolic volume of the heart because blood leaks from the aorta to the left ventricle during diastole. The left ventricle enlarges and is able to accommodate large volumes at lower pressures. The stroke volume proportionately increases to produce a wide pulse pressure.

HISTORY 1. Palpitations (increased motion of heart within the chest, that is responsible for the palpitation, rather than the increased force of contraction). 2. Dyspnea depends on the left ventricular dysfunction, which usually occurs late. 3. Angina: Occurs at right or at rest and is often associated with vasoactive phenomena such as flushing (a pseudo Nofthnagl attack).

Pulse Collapsing pulse or water hammer pulse: In patients with severe aortic regurgitation, this type of pulse is observed. (See Figure 3.1).

Blood Pressure In patients with pure severe aortic regurgitation the systolic BP is between 140 to 160 and the diastolic pressure is around 60 or less. Sometimes sounds can be heard till 0 on auscultation. However sounds will get muffled at around 60 to 40. Hence in recording BP for patients with aortic regurgitation you must mention systolic BP/diastolic BP at muffling and at disappearance. For example, BP should be written as 140/60- 0 mmHg. 78 Manual of Cardiology

Apex Beat 1. Apical impulse: diffuse, hyperdynamic, displaced down and inferiorly. 2. Rapid filling wave: This is a palpable wave felt in the apex in diastole and it is seen in patients with severe aortic regurgitation.

Auscultation Ejection Systolic Murmur A functional ejection systolic murmur due to increase in stroke volume is best heard in the 2nd right intercostal space. It is high pitched and shorter in duration and seen in severe aortic regurgitation. Sometimes this may be accompanied by a thrill.

FIGURE 11.1: Early diastolic murmur (EDM) is best heard in the 3rd intercostal space

Early Diastolic Murmur This is the key point in auscultation for the diagnosis of aortic regurgitation. a. Starts immediately after the A2. b. Loudest over the left 3rd and the 4th intercostal space; if aortic regurgitation is due to aortic root problem, may be best heard in the 2nd and 3rd right intercostal space. c. Best heard in the patients sitting, leaning forward and expiratory apnea. Aortic Regurgitation 79 d. The duration of murmur is directly related to the severity of aortic regurgitation. The longer the murmur, severe the aortic regurgitation. e. The murmur is augmented by exercise and reduced by amyl nitrite. With increasing severity of aortic regurgitation the length of the murmur increases. With slow heart rate, the diastole is prolonged and so the amount of regurgitation is also more. The early diastolic murmur is also long with bradycardia. The Figure 11.2 below shows the differences in the length of the murmur in relation to the length of diastole. The longer the diastole, the murmur is long.

FIGURE 11.2: EDM of aortic regurgitation and length of cardiac cycle: If the heart rate is low, the murmur is long and with tachycardia, the murmur is shorter in duration

Austin Flint Murmur This murmur is a mid diastolic murmur heard in the mitral area in patients with severe aortic regurgitation. This is related to preclosure of a mitral valve because left ventricle is filling in diastole from two sources namely, from the left atrium and the aorta. 80 Manual of Cardiology

TABLE 11.1: Differences between MDM of mitral stenosis versus Austin flint murmur Mitral Stenosis Austin Flint S1 Loud - S2 P2 loud - OS + - LV Enlargement - + RV Enlargement + - Amyl nitrite + Decreases Atrial fibrillation + -

Peripheral Signs Head and Neck 1. Corrigan’s—dancing carotids. 2. Demusset’s—bobbing of the head with each heart beat. 3. Retinal arteriolar pulsations. 4. Muller’s sign—pulsation of Uvula.

Upper Limb 1. Collapsing pulse. 2. Wide pulse pressure—> 50 percent of peak systolic pressure or diastolic pressure 60 or less. 3. Quincke’s—capillary pulsations in the nail bed.

Lower Limb 1. Traube’s—pistol shot sound. 2. Duroeziz’s murmur—systolic and diastolic murmur heard in the femoral artery proximal to compression. 3. Hill’s sign—systolic BP in the lower limb is more than 20 mm of Hg, when compared to upper limb.

Uncommon Peripheral Signs 1. Light house sign—alternate flushing and blanching of forehead. 2. Landolfi’s sign—variation of pupillary size. 3. Rosenbach’s sign—pulsations of liver. Aortic Regurgitation 81

4. Gerhardt’s sign—pulsations of spleen. 5. Oliver Cardavelli’s sign—pulsations of larynx.

Chest X-ray Cardiomegaly with LV contour depending on the degrees of AR.

FIGURE 11.3: The above X-ray shows cardiomegaly with left ventricular enlargement. Note the convex shaped left heart border dipping down to the diaphragm.

ECG LVH with diastolic overload; tall R waves and tall T waves.

Echo Color Doppler will confirm the diagnosis and severity of aortic regurgitation. 2D Echo is useful in assessing the LV size and function.

Natural History of Aortic Regurgitation Severe chronic AR is tolerated well for many years. If the LVEF is normal, 45 percent will remain asymptomatic at 10 years. 82 Manual of Cardiology

Once the patient becomes symptomatic the condition can deteriorate rapidly with death occurring within four years if the presenting symptom was angina and within two years if the presenting symptom was dyspnea. 1. Asymptomatic with Normal LV function: Progression to symptoms/LV dysfunction 6% Progression to asymptomatic LV dysfunction 3.5% Sudden death 0.2% 2. Asymptomatic with LV dysfunction: Progression to death or LV dysfunction before the onset of symptoms > 25 percent 3. Symptomatic patients Mortality > 10 percent/year

Medical Management 1. Antibiotic prophylaxis a. Infective endocarditis prophylaxis. b. Rheumatic fever prophylaxis. 2. Activities Restricted in severe AR such as competitive sports. 3. Cardiac drugs a. Asymptomatic with normal LV function: 1. Mild AR—none. 2. Moderate AR—nifedipine long acting. 3. Severe AR—nifedipine long acting. b. Severe AR symptomatic: 1. Normal LV function—nifedipine long acting. 2. LV dysfunction—Digoxin, diuretics, ACE inhibitor.

Surgical Management Decision for surgery should depend on patients’ symptoms and LV function. The expected AVR mortality is 3 to 8 percent. Aortic Regurgitation 83

Indications for Surgery 1. NYHA Class 3 or 4 symptoms. 2. Class 2 with progressive LV dilatation or serial drop in the EF. 3. Class 2 angina with severe AR. 4. Asymptomatic patients with LV dysfunction (LVEF 25 to 49%). 5. Patients undergoing coronary artery bypass surgery or surgery for other valves with significant aortic regurgitation.

Summary Pulse : Large volume, collapsing BP : Wide pulse pressure Apex : Hyderdynamic Murmur : Early diastolic murmur, Austin Flint. 84 Manual of Cardiology

INFECTIVE ENDOCARDITIS 12

Infection of the endothelium of the heart is called as Infective Endocarditis. Sometimes the infection may involve the lining of the large artery and this is called as infective end arteritis.

CLASSIFICATION 1. Native valve endocarditis (NVE)—infection of the native valves such as mitral or aortic valve. 2. Prosthetic valve endocarditis (PVE)—infection of a prosthetic valve.

PATHOPHYSIOLOGY Two factors are essential for the development of endocarditis 1. Aseptic fibrino—platelet thrombus (Nonbacterial Thrombotic Endocarditis—NBTE): This develops as a result of damage to the endothelium of the heart. This can happen because of damage to the valve or impact of high velocity jet such as mitral regurgitant jet or VSD jet. 2. Bacteremia: Though bacteremia can happen transiently a sustained bacteremia exposes bacterial colonization of the above NBTE resulting in infection.

Micro-organisms 1. Streptococci—50 percent. 2. Enterococci—5 to 8 percent. 3. Staph aureus—30 to 40 percent. Infective Endocarditis 85

4. Coagulase negative staphylococci—3 to 5 percent 5. Gram negative bacilli—4 to 8 percent

Duke’s Criteria for Infective Endocarditis Definitive diagnosis is based on: • Two major criteria, or • One major and three minor criteria, or • All five minor criteria (if no major criterion is met).

Major Criteria • Positive blood culture with typical organism in two separate cultures, or Persistently positive blood cultures, e.g. two or more than cultures done >12hr apart or all of three drawn 1 hr apart. • is involved: Positive echocardiogram (vegetation, abscess, dehiscence of prosthetic valve), or New valvular regurgitation (change in murmur not sufficient).

Minor Criteria • Predisposition (cardiac lesion; IV drug abuse) • Fever >38°C • Vascular/immunological signs • Positive blood culture that do not meet major criteria • Positive echocardiogram that does not meet major criteria.

CLINICAL FINDINGS 1. Signs of Infection: Fever, arthralgia, clubbing, weight loss, anemia, splenomegaly. 2. Cardiac lesions: Fever + new murmur = endocarditis until proven otherwise. Any new murmur, or change in the nature of a pre-existing murmur should raise a suspicion of endocarditis. 86 Manual of Cardiology

3. Peripheral signs a. Osler’s nodes: Tender, pea sized nodules occurring at the pad of the fingers. b. Janeway lesions: Large, nontender, macules occurring on the palms and soles. c. Roth spots: Oval retinal hemorrhages with a pale centre. d. Petechiae: They are seen on the extremities, above the clavicle, on the buckle, mucosa or on the palpebral conjunctiva. e. Splinter hemorrhages are seen in the underneath the nail.

INVESTIGATIONS Diagnosis: Duke’s criteria is used for the diagnosis of endocarditis. Blood cultures: Take 3 sets of cultures from different sites at one hour gap or two cultures a day for 3 days. Blood tests: Normochromic, normocytic anemia, leukocytosis, high ESR. Urine: Microscopic hematuria. ECG: Prolonged PR interval. Echo: Trans thoracic echo may show vegetations especially if more than 2 mm. Transesophageal echo is more sensitive and better for prosthetic valve endocarditis (Figure 12.1).

COMPLICATIONS 1. Cardiac complications - heart failure: This is the principal cause of death in IE. This is caused by valve destruction leading on to aortic and mitral regurgitation. 2. Cardiac abscess: This is common in the aortic valve and in prosthetic valve endocarditis. Infective Endocarditis 87

FIGURE 12.1: The above 2D echo picture shows a vegetation (arrow) on the mitral valve of a patient with infective endocarditis

3. Systemic arterial embolism: This occurs into the central nervous system especially in the distribution of middle cerebral artery. 4. Extra cardiac abscess: Results from septic infarction and frequently seen in the spleen and in the kidneys. 5. Mycotic aneurysm: They cause severe localized headache or focal neurological deficits. 6. Renal complications: Renal abscess, glomerulonephritis.

Prognosis 30 percent mortality with staphylococci; 14 percent with bowel organisms; 6 percent with streptococci.

Antibiotic Treatment 1. Streptococci: a. Aqueous penicillin G 12—18 million units/24 hour/IV as a continuous infusion or every 4 hourly in divided doses for a period of 4 weeks. b. Ceftriaxone—2 gms once daily IV for 4 weeks. 88 Manual of Cardiology

c. Aqueous penicillin G 12—18 million units/24 hours/IV for 2 weeks plus Gentamycin 1 mg/kg/IM or IV every 8 hour for 2 weeks. d. Vancomycin in two equally divided doses for 4 weeks for individuals who are penicillin allergic. 2. Enterococci: a. Aqueous penicillin G 18—30 million units/24 hour/IV as a continuous infusion for 4 to 6 weeks plus Gentamycin 1 mg/kg/IM or IV every 8 hourly for 4 weeks. b. Ampicillin 12 gm/24 hour/IV for 4 to 6 weeks plus Gentamycin as above. c. Vancomycin can be substituted instead of Penicillin or Ampicillin if the patient is allergic to Penicillin. 3. Staphylococci: a. Nafcillin 2gm/IV every 4 hourly for 4 to 6 weeks. b. Vancomycin for 4-6 weeks if penicillin allergic. c. For methicillin resistant staphylococci (MRSA), vancomycin for 6 weeks.

Prevention of Endocarditis Antibiotics are given prior to some specific procedures in patients with heart disease so that endocarditis is prevented. They are mentioned below: Heart diseases for which prophylaxis is recommended: • Prosthetic valve • Previous endocarditis • Left to right shunts such as VSD, PDA, ASD • Mitral valve prolapse with mitral regurgitation • Acquired valve disease. Heart disease for which prophylaxis is not recommended: • Mitral valve prolapse with no MR • Functional murmurs • ASD, VSD, PDA, operated with no residual shunt. Infective Endocarditis 89

Procedures for which prophylaxis is recommended: • Dental procedures • Esophageal dilatation • Sclerotherapy of varices • Surgery for gallbladder, lower bowel, genitor urinary tract. Procedures for which prophylaxis is not recommended: • Flexible bronchoscopy • Diagnostic GI endoscopy • Transesophageal echo • Cardiac catheterization • Cesarean section.

Antibiotic Regimen for Dental Procedures • Amoxicillin 3 g, 1 hour before the procedure • If penicillin allergy - clindamycin 600 mg oral.

Antibiotic Regimen for Genitourinary and GI Procedures 1. Injection: Amoxicillin 1g IV + Gentamicin 1.5 mg/kg followed by amoxicillin 500 mg orally 6 hours or later. 2. If penicillin allergy, give injection vancomycin 1g IV over 1 hour.

Section III ISCHEMIC HEART DISEASE 92 Manual of Cardiology

Ischemic Heart Disease (IHD) is the most common form of heart disease and it is a single most important cause of premature death in the world. IHD is caused by atherosclerosis of the coronary arteries. However, in a small proportion of patients, it can be due to arteritis or embolism of coronary arteries. The manifestations of ischemic heart disease are summarized in the following Table S-III-1 and Figure S-III.1.

TABLE S-III.1: Clinical presentations of IHD Clinical Presentation Reason Stable Angina (SA) Ischemia due to fixed stenosis of 1 or more of the coronary arteries Unstable Angina (UA) Ischemia due to dynamic obstruction of a coronary artery or due to plaque rupture Non ST segment Myocardial necrosis due to acute occlusion of a Elevation Myocardial coronary artery due to plaque rupture and Infarction (NSTEMI) thrombosis ST segment Myocardial necrosis due to acute occlusion of a Elevation Myocardial coronary artery due to plaque rupture and Infarction (STEMI) thrombosis Heart Failure LV dysfunction due to infarction or ischemia Arrhythmia Secondary to ischemia Sudden death Ventricular arrhythmia, asystole or massive myocardial infarction

FIGURE S-III.1: The spectrum of acute coronary syndromes

RISK FACTORS Nonmodifiable: Age, male, family history of ischemic heart disease (myocardial infarction in the first relative less than 55 years). Modifiable: Hypertension, smoking, diabetes, dyslipidemia, obesity, physical inactivity. Ischemic Heart Disease 93

Newer: Fibrinogen, apoprotein A, homocysteine, ACE genotype, hyperinsulinemia.

TABLE S-III.2:2 Risk factors and relative risks Factor Relative risk 95% Confidence Prevalence % Intervals Hypertension 1.2 0.8-1.8 26.3 Smoking 3.6 2.2-5.5 46.7 Diabetes > 120 mg% 2.7 1.4-5.3 02.6 HDL < 35 mg % 1.8 1.2-2.6 19.2 Cholesterol > 240 mg% 1.8 1.2-2.7 14.3 Lp (a) 1.9 1.2-2.9 11.3

DYSLIPIDEMIA/LIPID CONTROL Dyslipidemia is one of the modifiable risk factors in patients with ischemic heart disease. 1. 10 percent increase in serum cholesterol increases the risk of by 20 to 30 percent. 2. A reduction in serum cholesterol by 10 percent reduces cardiovascular death by 10 percent. 3. Treatment for 5 year reduces cardiovascular events by 25 percent.

Management of Dyslipidemia All patients with ischemic heart disease should be screened for serum cholesterol levels. It is recommended that this kind of screening also be done in adults over the age of 20 years. The current guidelines recommend different targets depending on the patients overall risk and it is summarized as below:

TABLE S-III.3: Target LDL level Variable Target LDL Diabetes/Ischemic heart disease LDL cholesterol 100 mg or less Two or more risk factors LDL cholesterol 130 mg or less One risk factor or less LDL cholesterol < 160 mg 94 Manual of Cardiology

TABLE S-III.4: Drugs used for lipid control Drug group Name of the Drug HMG-CoA reductase inhibitors Simvostatin Atorvastatin Rosuvastatin Fibric acid derivatives Fenofibrate Cholesterol absorption inhibitor Ezetamibe Vitamin Nicotinic acid Bile acid sequestrants Cholestyramine

Statins have become the primary drug for lowering LDL cholesterol. Statins decrease the LDL level to a greater extent. 5 mg of atorvastatin or 10 mg of simvostatin will reduce the total cholesterol by 22 percent and LDL is lowered by 27 percent. Each doubling of the dose will result in additional 5 percent reduction in total cholesterol and 7 percent reduction in LDL cholesterol. This is called as “the rule of 5 and 7”. Hence doubling the dose does not double the reduction in the cholesterol level. The most important side effects of statins are myositis and rhadomyolysis. The overall incidence is 1 case per 10,000 patient years. 1 percent of patient taking statins experience elevation of serum liver enzymes more than 3 times the upper limit of normal. In some patients’ symptoms of hepatitis may occur and resemble dose of influenzae like syndrome. Mild elevation—less than twice normal do not require cessation of statins. It is better to do liver function test before starting the therapy and after 2 to 3 months. The liver enzymes never increase after the first few months of therapy unless the dose has been increased or some other new drug has been added that interferes with drug metabolism. Ischemic Heart Disease 95

METABOLIC SYNDROME (International Diabetes Federation 2005) 1. High waist circumference >90 cm in men or >80 cm in women with 2. Any of two of the four factors: a. Triglycerides > 150 mg. b. HDL < 40 in men and < 50 in women. c. Blood pressure > 130/85 mmHg. d. Fasting sugar > 100 mg percent or diabetic. 96 Manual of Cardiology

ANGINA 13

STABLE ANGINA Angina can be classified as given below: 1. Stable angina. 2. Unstable angina. 3. Variant angina (Prinzmetal angina). The term stable angina refers to the predictable occurrence of pressure or choking sensation in the chest, on effort. CLINICAL CLASSIFICATION OF CHEST PAIN Typical angina has three important features 1. P—Precipitated by exertion. 2. Q—Quality of chest pain is choking or pressure type. 3. R—Relived by rest or nitrates.

Atypical Angina Two of the above 3

Non-cardiac Chest Pain One of the above 3

Classification of severity of Angina (Canadian Cardiovascular Society Classification—CCS) Class I • No angina with ordinary physical activity (e.g., walking, climbing stairs) • Angina with strenuous or prolonged exertion. Angina 97

TABLE 13.1: Symptoms of angina Classic (Typical) Atypical, Non-cardiac Sensations in chest of squeezing, Pain that is pleuritic, sharp, pricking, heaviness, pressure, weight, vise- knife-like, pulsating, lancinating, like aching, burning, tightness choking Radiation to shoulder, neck, jaw, Involves chest wall; is positional, inner arm, epigastrium (can occur tender to palpation; can be infra- without chest component); band- mammary; radiation patterns highly like discomfort variable Relatively predictable Random onset Lasts 3-15 min Lasts seconds, minutes, hours, or all day Abates when stressor is gone or Variable response to nitroglycerin nitroglycerin is taken

Class II Early-onset limitation of ordinary activity (e.g., walking rapidly or walking > 2 blocks; climbing stairs rapidly or climbing >1 flight); angina may be worse after meals, in cold temperatures, or with emotional stress.

Class III Marked limitation of ordinary activity.

Class IV Inability to carry out any physical activity without chest discomfort or angina occurs at rest.

INVESTIGATIONS FOR STABLE ANGINA 1. Resting ECG. 2. Echocardiography. 3. Treadmill testing. 4. Dobutamine stress echo. 5. Sestamibi test. 6. Multislice CT 7. Coronary angiogram. 98 Manual of Cardiology

Resting ECG Routine ECG done for a patient with stable angina is most likely to be normal. However, it is useful in showing whether the patient has had an old infarction. ST depression with or without T inversion especially if seen during an episode of angina is a strong evidence of ischemia.

Echocardiography Echo is useful in the assessment overall left ventricular function. In addition, they can identify abnormalities such as regional wall motion abnormalities that are secondary to old myocardial infarction.

Treadmill Testing (TMT) Indications 1. Patients with atypical chest pain. 2. Patients with typical chest pain in whom there is a need to understand the prognosis. 3. Following PCI or CABG to find out residual ischemia. 4. For patient with a typical chest pain, there is no need to do a TMT; however can be done to assess the effort tolerance.

Contraindications 1. Unstable angina. 2. Recent ST segment elevation MI within 5 days. 3. Severe aortic stenosis. 4. Uncontrolled hypertension or heart failure. Morbidity: 24 in 1,00,000 patients. Mortality: 10 in 1,00,000 patients.

Positive Result 1. Horizontal or downsloping ST depression > 1 mm is consi- dered as positive for ischemia. Angina 99

2. Sensitivity—68 percent 3. Specificity- 70 percent 4. This is the most widely used test for the diagnosis of angina and also indirectly used for prognosis. When interpreting Treadmill testing, we should keep in mind the probability of the prevalence of coronary artery disease in the population—pre-test probability. Over all false positive or false negative results are observed in one-third of cases. A negative result does not exclude coronary artery disease although it makes a likelihood of three vessel or left main CAD extremely unlikely.

Strongly Positive TMT 1. 1 mm or more ST depression before completion of Stage II. 2. 2 mm ST depression at any stage. 3. ST depression for more than 5 minutes after exercise. 4. Decline in systolic BP > 10 mmHg during exercise.

Dobutamine Stress Echo Indications 1. For patients, who are unable to exercise on a Treadmill. 2. For patients, who have baseline abnormal ECG. Positive Result Development of new left ventricular wall motion abnormality. Sensitivity—85 percent. Specificity—85 percent.

Advantages 1. Higher specificity. 2. Versatility—more extensive evaluation of cardiac anatomy and function. 3. Greater convenience/efficacy/availability. 4. Lower cost. 100 Manual of Cardiology

Sestamibi Testing In this patient is made to walk on a treadmill or dobutamine infusion is given to increase the heart rate. At peak exercise, sestamibi—a radio isotope is injected intravenously. After that, patient undergoes scanning by gamma camera in the nuclear medicine department.

Indications For patients unable to exercise adequately or patients with resting ECG abnormalities. Positive Result Perfusion abnormalities. Sensitivity—90 percent. Specificity—90 percent.

Strongly Positive 1. Multiple perfusion defects. 2. Increased lung uptake. 3. Decrease in ejection fraction during exercise.

Advantages 1. Higher technical success rate. 2. Higher sensitivity—especially for single vessel coronary disease involving the left circumflex. 3. Better accuracy in evaluating possible ischemia when multiple resting LV wall motion abnormalities are present. 4. More extensive published data base—especially in evaluation of prognosis.

Multislice CT (MSCT) This is a promising noninvasive technique to visualize the coronary arteries. In this method; contrast is injected through a peripheral vein and the imaging is done using a high Angina 101 resolution CT scan. The major limitation of this technique has beent the presence of severe calcium in the coronary arteries. This test may replace invasive coronary angiogram in the future.

Coronary Angiography This is the gold standard for the anatomical diagnosis of IHD. This is done in a cardiac catheterization lab. A catheter is passed into the heart via the femoral artery (Judkin’s Method) or via radial artery or via brachial artery (Sone’s Method).

Indications 1. Severely symptomatic despite medical therapy. 2. There is need to confirm or rule out the diagnosis of ischemic heart disease. 3. Patients, who have survived cardiac arrest. 4. Patients with angina and noninvasive testing suggest that it is strongly positive.

Risks 1. Death. 2. Myocardial infarction. 3. Stroke. 4. Vascular access complications.

PROGNOSIS Five-year mortality rate in IHD is based on the number of vessels involved and the left ventricular function 1. Single vessel disease 2 percent. 2. Two vessel disease 8 percent. 3. Three vessel disease 11 percent. 4. Left main mortality is 15 percent per year.

TREATMENT The management plan has the following components: 1. Treatment of risk factors. 102 Manual of Cardiology

2. Drug therapy. 3. PTCA. 4. CABG.

Treatment of Risk Factors a. Blood pressure control. b. Diabetic control. c. Stop smoking. d. Lipid lowering—LDL cholesterol < 100 mg percent.

Drug Therapy Nitrates Glyceryl trinitrate (GTN) can be given in the form of tablets or spray in the buccal mucosa. This rapidly relieves angina. For prophylaxis one of the following preparations may be used. a. Isosorbide dinitrate (ISDN) oral 10 to 60 mg Q8H. b. Isosorbide 5 mononitrate (ISMO) 20 to 30 mg twice daily. c. Transdermal NTG patch 0.4 to 1.2 mg per hour for 24 hours. Side Effects Headache, flushing. Contraindication: GTN should not be given to patients, who have used Sildenafil in the last 24 hours or to patients with low blood pressure. Mechanism of Action This group of drugs causes powerful venodilatation and thus reducing myocardial wall tension and oxygen requirement.

Beta blocker These drugs represent an important component in the treatment of angina. They reduce the myocardial oxygen demand. The drugs are: Atenolol, metoprolol, bisoprolol. Angina 103

Calcium Channel Blocker Diltiazem, verapamil, amlodipine, slow release nifedipine can all be used. These drugs are indicated when beta blockers are contraindicated, poorly tolerated.

Antiplatelet Drug Aspirin 75 mg is the standard therapy. Clopidogrel 75 mg can be given if aspirin sensitivity is present.

Percutaneous Coronary Intervention (PCI) A balloon is inflated across the narrowed segment of the coronary artery. After this, a stent is placed at this site. There are now bare-metal stents and drug coated stents (Figure 13.1).

FIGURE 13.1: The above figure shows angiographic picture of left coronary artery system. Note there is a tight lesion in the LAD as shown by arrow in the picture on the left. On the right, this lesion has been dilated with balloon and stented

Success of this procedure is more than 95 percent. Restenosis occurs in 20 percent of cases within 6 months with the use of bare metal stents and angina will recur within 6 months in 10 percent of cases. With the use of drug coated stent the incidence of restenosis has come down to 6-7 percent. The drugs used for coating of the stents are: Sirolimus or 104 Manual of Cardiology

Paclitaxol. Risks: Death < 0.5 percent, emergency bypass surgery < 1 percent, myocardial infarction < 2 percent.

Advantages 1. Less invasive. 2. Shorter hospital stay—2 days. 3. Can be easily repeated. 4. Effective in reliving symptoms.

Disadvantages 1. Restenosis. 2. Incomplete revascularization. 3. Poor outcome in diabetics.

Coronary Artery Bypass Grafting (CABG) In this procedure, a block in the coronary artery is bypassed using internal mammary artery or radial artery or saphenous vein or gastroepiploic artery. This surgery is done using a heart lung machine or without this machine. The later is called as off-pump CABG. Risks: Death <1 percent, myocardial infarction 4 percent. Success: Angina is abolished in more than 90 percent of cases, survival is increased in patients with left main and triple vessel disease with LV dysfunction. Follow-up: Occlusion of venous grafts is observed in 20 percent of patients during the first year and in 2 percent per year for the first 5 years; thereafter 4 percent per year. Internal mammary artery is associated with longer patency.

Indications 1. For significant left main disease. 2. Three vessel disease and reduced LV function—<50 percent. 3. Two vessel disease including LAD and reduced LV function. Angina 105

Advantages 1. Effective in relieving symptoms. 2. Improved survival. 3. Possible to achieve complete revascularization.

Disadvantages 1. Higher cost. 2. Increased risk of procedure. 3. Longer duration of hospitalization—5 to 7 days.

TABLE 13.2: Comparison of PCI versus CABG PCI CABG Death 0.5% 1.5% Myocardial infarction 1% 4% Recurrent angina 30% at 6 months 10% at 1 year Repeat revascularization 20% at 2 year 2% at 2 year Neurological complications Rare Common Hospital stay 24-48 hours 5-8 days Return to work 7 days 6 weeks

UNSTABLE ANGINA CLINICAL PRESENTATIONS The history is the most important one and the following clinical scenarios are included as unstable angina. 1. New onset: Angina of class 3 or 4 within the last two months. 2. Crescendo angina: Angina on effort that has increased atleast one class-to-class 3 or more in the last two months. 3. Rest angina: Angina that is occurring at rest and prolonged for more than 15 minutes occurring within one week of presentation. 4. Postinfarction angina: Angina that develops within two weeks after myocardial infarction. These patients are a very high risk group and the probability of death or myocardial infarction in the next one year is around 20 percent. 106 Manual of Cardiology

EARLY RISK STRATIFICATION All patients with unstable angina should be risk categorized based on clinical, ECG, troponin and hsCRP if available. Clinical: Rest angina, age > 75 years, hypotension or pulmo- nary edema suggest that the patient is a higher risk. ECG: ST deviation more than 0.5 mm or T inversion more than 3 mm or left bundle branch block are associated with higher risk. Troponin: Elevated troponin T or I suggest microinfarction and so they carry a higher risk. HsCRP: This is one of the newer markers that appear to be promising to identify patients, who are at high risk.

MEDICAL THERAPY The aim of therapy is to stabilize the patient and passivate the coronary lesion responsible unstable angina and then proceed for invasive therapy. 1. Aspirin: A dose 150 mg is given as a initial dose and this is followed by 75 mg. 2. Clopidogrel: Based on a large trial—CURE, all patients with unstable angina are given a loading dose of 300 mg followed by 75 mg once daily. 3. Enoxaparin (Clexane): This is a low molecular heparin and it is given 1 mg per kg body weight twice daily. Heparin may be substituted; however, this needs APTT monitoring and so we prefer enoxaparin. Enoxaparin is similar in efficacy and safety profile (Table 13.3).

TABLE 13.3: Comparison of enoxaparin with heparin, meta analysis End point Enoxaparin % Heparin % Death at 30 days 3% 3% Death or MI at 30 days 10% 11% Transfusion upto 7 days 7.2% 7.5% Major bleeding 4.7 4.5% Angina 107

4. Glycoprotein 2b/3a Inhibitors: These drugs block the 2b/3a receptors in the platelets and these agents are used only for high risk subsets and if they are going for PCI. As per the ACC guidelines the following drugs are used: a. Reo-pro IV or eptifibatide IV can be given prior to PCI. b. Eptifibatide or tirofiban can be given for high risk subsets, who are not planned for interventional PCI. 5. Statins: All patients should be started with atorvastatin 40 to 80 mg per day. The lower the LDL achieved, better are the outcomes. 6. Nitrates: Should be given sublingually or by buccal spray; if pain persists, after 3 tablets are given at 3 minute intervals, intravenous nitroglycerin is recommended. 7. Beta blockers: All patients should be given beta blockers unless there is a contraindication. 8. ACE Inhibitors: These groups of drugs are useful in the long term and so can be started in the hospital, if blood pressure is stable. 9. Invasive therapy: All patients after medical stabilization should undergo coronary angiography to decide regarding further treatment. The following subgroups are ideally suited for early invasive therapy within 48 hours of admission: a. Recurrent chest pain. b. Troponin elevation. c. ST segment elevation in anterior leads or dynamic ST depressions. d. Left ventricular failure. e. Hemodynamic instability. f. Prior CABG.

CHECK LIST The following is a check list for prescription writing. At admission, there are 7 drugs and at discharge a minimum of 5 drugs. 108 Manual of Cardiology

Check list for drugs at admission: 1. Aspirin 2. Clopidogrel 3. Clexane—enoxaparin 4. Nitrates 5. Beta blocker 6. Statins 7. ACE inhibitors. Check list for drugs at discharge: 1. Aspirin 2. Clopidogrel 3. Statins 4. Beta blocker 5. ACE inhibitors.

FIGURE 13.2: Treatment algorithm for UA/NSTEMI Angina 109

PRINZMETAL (VARIANT ANGINA) This type of angina has the following clinical features: 1. Chest pain occurs at rest. 2. ST segment is elevated. 3. May be associated with cardiac arrhythmias such as ventricular tachycardia or ventricular fibrillation.

Mechanism The reason for this type of angina is coronary artery vasospasm. Coronary vasospasm can be demonstrated in the cardiac cath lab by using ergonovine or acetylcholine.

Management 1. Nitrates are the main form of therapy and they relieve angina very quickly. 2. Prazosin—a selective alpha blocker has also been found to be useful. 3. Nicorandil—a coronary vasodilator due to potassium channel activation is also useful. 4. Aspirin may actually increase the severity of ischemic episodes. 110 Manual of Cardiology

ACUTE MYOCARDIAL 14 INFARCTION

Incidence 5 per 1000 per year.

EPIDEMIOLOGY 1. Around 50 percent of patients die of sudden death within first one hour. 2. Of the remaining patients, who enter the hospital 4 percent will have the diagnosis missed in the hospital. 3. In the hospital the expected mortality is around 5 to 15 percent. 4. Approximately 75 percent of patients who are admitted with myocardial infarction are stable. 5. About 18 percent are likely to develop post infarction angina during the hospitalization. 6. Two-thirds of the patients are likely to have non ST segment elevation myocardial infarction and one-third are likely to have ST segment elevation myocardial infarction.

PATHOLOGY Myocardial infarction is the result of complete occlusion of a coronary artery. The occlusion is caused by a thrombus. The initiating event is the plaque rupture. This leads on to platelet activation and aggregation which ultimately results in thrombus formation. Acute Myocardial Infarction 111

RISK FACTORS Nonmodifiable: Age, male, family history of ischemic heart disease (myocardial infarction in the first degree relative less than 55 years). Modifiable: Hypertension, smoking, diabetes, dyslipidemia, obesity, physical inactivity. Newer: Fibrinogen, lipoprotein(a), homocysteine, ACE genotype, hyperinsulinemia.

DIAGNOSIS The diagnosis depends on two of the three criteria are met. They are namely, clinical, ECG and enzyme evidence.

Clinical 1. The pain is severe and prolonged, lasting more than 20 minutes to several hours. 2. This pain is compressing in quality. 3. The pain is retrosternal in location. 4. The pain may radiate down the ulnar aspect of the left arm, fingers. The pain may also radiate to the neck, jaw, interscapular region. 5. The pain may be associated with severe sweating, extreme weakness or syncope or nausea or vomiting.

ECG The ECG is very sensitive for confirming the diagnosis of myocardial infarction. However, the ECG in the initial phase may be entirely normal and in one-third of patients it may not be diagnostic. Hence we need to rely on serial ECG’s. Based on the ECG changes the myocardial infarction may be classified as 1. ST segment elevation MI (STEMI) and 2. Non ST segment elevation MI (NSTEMI). 112 Manual of Cardiology

NSTEMI The diagnosis of NSTEMI is based on the cardiac enzyme elevation and typical chest pain. The ECG may show ST depression or deep T inversions (Figure 14.1).

FIGURE 14.1: This ECG was taken from a patient who had chest pain for 30 minutes. Note there is marked ST depression in lead I, II and V3-V6. In addition, there is ST elevation in aVR. ST elevation in aVR indicates proximal LAD artery involvement. His cardiac enzymes were elevated

STEMI Based on the leads in which ECG ST segment elevation is present, the myocardial infarction is categorized as anterior, inferior, posterior and right ventricular infarctions (See Figure 38.4 and 38.5). 1. Within hours of infarction, T waves become tall and peaked and the ST segment begins to rise. 2. ST segment is elevated with convexity upwards. 3. Within 24 hours, T waves start to invert and the ST segment comes towards the baseline. 4. Within a few days after MI, pathological Q waves begins to form. Based on the leads in which ST segment elevation is seen, myocardial infarction can be classified into the following types: 1. Anterior: V1 to V6. 2. Anteroseptal: V1 to V4. Acute Myocardial Infarction 113

3. High lateral: Lead I, aVL. 4. Extensive anterior: Lead I, aVL, V1 to V6. 5. Inferior: Lead II, III, aVF. 6. Posterior: R waves are tall in V1 with ST depression and T is upright. This usually occurs along with inferior wall myocardial infarction. Posterior leads will usually help in the diagnosis. 7. RV Infarction: Leads recorded from the right chest such as V3 R, V4 R shows the ST changes.

Enzymes One of the most important features of myocardial infarction is demonstration of elevated cardiac enzymes in the blood. The following enzymes are usually done (Figure 14.2). 1. Creatine kinase—MB (CK-MB): The cardiac muscle contains predominantly this enzyme. However, CK-MB is also present in small quantities in the small intestine, tongue, diaphragm, uterus and prostate. CK-MB starts to raise at 4 to 6 hours peaks at 12 hours and comes down to normal within 48 hours.

FIGURE 14.2: CK-MB enzyme raises quickly and comes down also quickly. On the other hand, the levels of troponin increase several fold and remains elevated for a week or more 114 Manual of Cardiology

2. Troponin T or I: This enzyme starts to raise 4 to 6 hours after myocardial infarction and peaks around first day and remains elevated 7 to 10 days after myocardial infarction. This prolonged elevation is useful for the late diagnosis of MI. It is possible that in some patients troponins may be elevated but CK-MB values are within normal limits. This is because troponins are capable of detecting minor myocardial necrosis. KILLIP—Classification Patients admitted into CCU can be classified into four classes based on their hemodynamic status. This particular classification correlates with CCU mortality (Table 14.1).

TABLE 14.1: KILLIP classification Class Hemodynamic status Incidence Mortality I No heart failure 40% 5% II Mild heart failure 40% 20% III Pulmonary edema 10% 40% IV 10% 90%

EARLY MANAGEMENT Time is the essence of management and it is important that immediate therapy is targeted for opening up of the infract related artery in the form of thrombolysis or primary PTCA. (Figure 14.3 and 14.4).

FIGURE 14.3: Time line for treatment Acute Myocardial Infarction 115

FIGURE 14.4: Algorithm for initial management of acute myocardial infarction

1. Analgesia—Morphine IV 3 to 6 mg, gives immediate relief of pain and can be repeated as required. 2. Aspirin—300 mg is given in soluble form immediately. 3. High-flow oxygen—Four litres per minute is given via nasal cannula. 4. Thrombolytic therapy—In order to open the infarct related artery drugs that can dissolve the thrombus are given. This therapy is indicated for patients with ST segment elevation myocardial infarction presenting within 12 hours of onset. Follow the steps given below to decide whether the patient is fit for thrombolysis. Step 1: Chest discomfort > 15 minutes and < 12 hours— proceed to step 2. Step 2: If any one of the following is present check with the consultant before proceeding for thrombolysis. 116 Manual of Cardiology

1. Systolic blood pressure > 180 mmHg. 2. Diastolic blood pressure > 110 mmHg. 3. Right versus left arm systolic BP difference > 15 mmHg. 4. Past history of cerebrovascular accident. 5. Recent—within 6 weeks of major surgery or gastroin- testinal bleeding. 6. CPR > 10 minutes. 7. Pregnant, female. 8. Serious diseases such as liver, kidney disease, terminal cancer. Step 3: If the patient is in severe heart failure or cardiogenic shock then primary PTCA may be preferable, rather than thrombolysis.

THROMBOLYTIC DRUGS 1. Streptokinase 1.5 million units given over 1 hour. 2. Tissue Plasminogen Activator (t-PA): 15 mg bolus, 50 mg over 30 minutes and 35 mg over 60 minutes. 3. Reteplace 10 units followed by another 10 units bolus.

Markers of Opening up of Infarct Related Artery 1. Prompt and persistent relief of chest pain. 2. ST resolution within 120 minutes. 3. Arrhythmias such as accelerated idio-ventricular rhythm.

Choice of Thrombolytic Agent 1. Within 4 hours of chest pain t-pa is preferred because rapidity of reperfusion. 2. Between 4 to 12 hours, both streptokinase and t-pa are equivalent options.

Complications of Thrombolysis 1. Intracranial hemorrhage is the most serious complication. The overall incidence is 0.5 percent. Hence, it is important Acute Myocardial Infarction 117

to identify whether there are any contraindications for thrombolysis such as old cerebrovascular accident. 2. Hypotension: BP fall during the infusion of streptokinase is common and this can be reversed easily by infusing fluids or leg end elevation of the patient’s bed; only rarely, streptokinase infusion need to be stopped.

Benefits of Thrombolysis 1. Prompt thrombolysis within 12 hours reduces the mortality significantly. 2. In addition, improves the left ventricular function by reducing the infarct size. 3. By opening up, the infarct related artery, the remodeling of the left ventricle results in less left ventricular dysfunction.

TABLE 14.2: Criteria for starting thrombolytic therapy

1. Chest pain consistent with myocardial ischemia - > 20 minutes with 2. ECG changes: ST segment elevation > 1 mm in > 2 contiguous limb leads or ST segment elevation > 2 mm in > 2 contiguous precordial leads or new left bundle branch block 3. In the absence of contraindications

Primary PTCA This mode of treatment is an effective alternative therapy for patients with STEMI. The benefits are as follows: 1. Higher rate of reperfusion—opening up of the infarct related artery > 90 percent. 2. The residual lesion can also be treated at the same time— less incidence of post infarct angina and reinfarction. 3. It is an alternative in patients in whom thrombolysis has failed. 4. It is the treatment of choice in patients in whom there is a contraindication for thrombolysis. The Figure 14.5 shows an occluded right coronary artery proximally. This patient underwent a primary PTCA for his 118 Manual of Cardiology inferior wall infarction and the picture on the left shows a fully opened up right coronary artery.

FIGURE 14.5: Coronary angiogram of the right coronary artery before and after primary PTCA

Primary PTCA is preferred in the following circumstances 1. Skilled cath lab is available and door to balloon is < 90 minutes; this means that the time from contact with the patient in the hospital to inflation of the balloon in the cath lab. If this time is kept to minimum, the amount of myocardium saved is more. 2. High risk STEMI such as cardiogenic shock or in heart failure. 3. Contraindications to thrombolysis.

CORONARY CARE UNIT All patients with STEMI are to be admitted in a dedicated coronary care unit (CCU) and observed for 48 hours. During this period, patient’s vital signs such as heart rate, rhythm, blood pressure, oxygen saturation are monitored. Nowadays this kind of monitoring is done through a central unit so that doctors and nurses can monitor the patients from a remote staff room. The CCUs have been one of the important factors that brought down the mortality to less than 15 percent. With Acute Myocardial Infarction 119 addition of thrombolysis or primary PTCA currently, the expected CCU mortality is in the range of 5 to 10 percent.

DRUG THERAPY IN CCU 1. Aspirin: All patients are given aspirin on a maintenance dose of 75 to 150 mg. Enteric coated preparations are preferred to minimize the gastrointestinal irritation. 2. Beta blockers: If there are no contraindications such as hypotension, bradycardia, past history of asthma, patients are started on beta blockers such as metoprolol on a twice daily basis. 3. Clopidogrel: A starting dose of 300 mg if not given in the emergency department is given in the CCU and this is followed with 75 mg once daily. 4. Statins: These drugs have plaque stabilization effects in addition to lipid lowering effects. Hence atorvastatin 20 mg to 80 mg is given from day one in the hospital. 5. ACE Inhibitors: This group of drugs to be given to patients with pulmonary congestion or if left ventricular ejection fraction is < 40 percent by Echo. Before giving make sure that the blood pressure is 100 mmHg or more. Ramipril is preferred because of lower incidence of hypotension. 6. Nitroglycerin: a. Sublingual nitroglycerin is given every 5 minutes as needed for chest discomfort. b. Intravenous nitroglycerin for a period of 24 to 48 hours is given only if heart failure or hypertension or persistent chest pain is present for a period. 7. Anxiolytics: Alprazolam or diazepam are given routinely to reduce anxiety based on the needs. 8. Stool softner: This is given so that patients do not strain for stools. The drugs that are commonly used are fybogel or cremaffin. 9. Heparin/low molecular heparins: Patients in cardiogenic shock, atrial fibrillation or left ventricular thrombus are given 120 Manual of Cardiology

anticoagulation. In addition, patients in whom tissue plasminogen activator has been given for thrombolysis should also receive heparin.

COMPLICATIONS OF INFARCTION 1. Arrhythmias (Table 14.3) 2. Cardiac failure 3. Cardiogenic shock 4. LV thrombus 5. Pericarditis 6. Ventricular septal rupture 7. Papillary muscle dysfunction/rupture 8. Left ventricular aneurysm 9. Right ventricular infarction.

TABLE 14.3: Arrhythmias Arrhythmias Treatment Ventricular premature beats Check potassium, magnesium, use betablocker/lidocaine Ventricular tachycardia Antiarrhythmic drugs/DC shock

Ventricular fibrillation DC shock

Atrial fibrillation Verapamil, Digoxin, Amiodarone infusion

AV block Pacemaker

RIGHT VENTRICULAR INFARCTION Approximately 30 percent of patients with inferior wall myocardial infarction have RV infarction. The features are as follows: 1. Elevated jugular venous pressure. 2. Lungs are clear. 3. Hypotension. Acute Myocardial Infarction 121

4. ECG shows ST segment elevation of 1 mm or more in right sided chest leads (V4R). 5. IV fluids are given to increase the right ventricular preload to angioplasty. 6. Angioplasty of the right coronary artery will improve the RV function quickly.

POST MYOCARDIAL INFARCTION CARE 1. Hospital discharge: The discharge timing of the patient depends on the condition of the patient in relation to chest pain or left ventricular failure. Most patients are likely to be ready for discharge by 5th day or so. 2. Risk stratification: a. Patients who are stable at discharge and if ejection fraction is > 40 percent, can go for elective non- invasive testing such as Treadmill or Sestamibi testing to decide regarding further treatment. If these tests show significant ischemia then these patients are referred for coronary angiogram. b. Patients in whom ejection fraction is < 40 percent or post infarction angina or significant left ventricular dysfunction clinically should go for early coronary angiography. 3. Activities: Patient should undertake light activities for a period of 6 weeks and avoid air travel for 2 months. Patient can join for work after 2 months. 4. Risk factor modification: All patients should be encouraged to control risk factors such as diabetes, hypertension and stop smoking. 5. Secondary prevention: 1. Aspirin: All STEMI patients are given 75 mg of aspirin indefinitely. There is a 22 percent reduction of death, re- infarction and stroke. If patient cannot tolerate, then 122 Manual of Cardiology

clopidogrel is given (clopidogrel started in CCU is given for only one month post infarction usually). 2. Beta blocker: Beta blockers have shown a 23 percent reduction in mortality following STEMI. They are given for a period of 2 to 3 years and if patient can tolerate, continue the drug for life long. 3. Cholesterol reducing agents - Statins: These drugs are given life long so as to keep the LDL at 70 mg or less. A dietician’s advise is preferable. 4. Diet control: A low fat diet is a must and saturated fat component is limited to 7 percent and cholesterol to 200 mg or less. 5. Exercise: Exercise helps in increasing the HDL cholesterol and overall physical fitness. 6. ACE inhibitors: Based on the results of HOPE and EUROPA studies indefinite treatment with ACEI is justified, provided there is no contra indication. ARB’s such as VALSARTAN may be substituted as an alternative to ACEI. 7. AICD: (Automatic Internal Cardioverter Defibrillator). This is a pacemaker like machine which is inserted transvenously. This discharges DC shock to patient if it identifies a serious ventricular arrhythmia. As a result patients with malignant ventricular arrhythmias following myocardial infarction can effectively be treated. Multiple trials have shown, this device to reduce the mortality. The algorithm for the same is given below in Figure 14.6. In patients following myocardial infarction if their LV dysfunction is severe, ejection fraction < 30 percent, AICD can be fixed without any further study. The cost of AICD is approximately Rs.3,00,000/. This has been the major limitation of its use in our country. Acute Myocardial Infarction 123

FIGURE 14.6: Algorithm for AICD selection in post infarction patient

Summary of Secondary Prevention of STEMI: A simple way to remember the headings for secondary prevention of myocardial infarction is A B C D E.

TABLE 14.4: Secondary prevention of STEMI A = Aspirin + ACE inhibitors B = Beta blockers C = Cholesterol reduction—statins D = Diet control E = Exercise

Section IV HEART FAILURE 126 Manual of Cardiology

Heart Failure can be defined as failure of the heart to pump blood at a rate commensurate with the requirements of the body. Circulatory Failure is not equal to heart failure because both cardiac and noncardiac conditions such as a massive GI bleed can lead on to circulatory collapse, while cardiac function is preserved. Myocardial Failure is a term that is used to denote systolic or diastolic dysfunction. Right Heart Failure is a term used for systemic venous congestion. Left Heart Failure is a term used due to symptoms of pulmonary congestion secondary to a disease affecting the left side of the heart. Congestive Cardiac Failure is a term used when both pulmonary and systemic venous congestions are present. Diastolic Heart Failure means that the patient has features of clinical heart failure in the presence of preserved systolic function. Low Output Heart Failure is a term is used for conditions that cause systemic venous congestion and pulmonary venous congestion with low cardiac output. Heart Failure 127 HEART FAILURE 15

The incidence of heart failure increases with advancing age. The average incidence is 1 to 2 percent between 50 and 59 years and 10 percent in patients above 75 years.

TABLE 15.1: Causes of heart failure Causes Example Myocardial dysfunction Hypertension, Ischemic heart disease, cardiomyopathy Obstruction to flow Mitral stenosis, aortic stenosis Regurgitant lesions Mitral regurgitation, aortic regurgitation High output states Anemia, thyrotoxicosis Restrictive ventricular Constrictive pericarditis, cardiac tamponade, filling restrictive cardiomyopathy Arrhythmias Atrial fibrillation

PRECIPITATING CAUSES OF HEART FAILURE 1. Inappropriate reduction of therapy: This is the most common cause of deterioration in a known heart failure patient. 2. Arrhythmias: Arrhythmias are common in patients with heart failure and these are likely to precipitate heart failure. 3. Myocardial Ischemia 4. Infections: In patients with heart failure any infection is likely to increase the work load on the heart, which results in heart failure. 5. Drugs: Drugs such as verapamil, diltiazem, and doxorubicin can precipitate heart failure. 128 Manual of Cardiology

SYMPTOMS Exertional Dyspnea This is the most important clinical symptom of left ventricular failure. At first, this occurs on more than normal exertion. Gradually, as the disease progresses, symptoms can be present at rest.

Orthopnea This symptom can be defined as dyspnea that occurs in the recumbent position. When a patient lies down blood gets displaced from the periphery to the lungs. In heart failure, this results in pooling of blood in the lungs which manifests in the form of dyspnea on lying down. This is relieved immediately after getting up or else patient sits up with several pillows so that the head is an elevated position.

Paroxysmal Nocturnal Dyspnea (PND) In this patient gets dyspnea suddenly 2 to 4 hours after sleeping. Patient is forced to get up and seek fresh air. It usually takes 30 minutes or more to get relieved. These attacks need to be differentiated from bronchial asthma (Table 15.2). The following are the possible mechanisms for the same: 1. Depression of respiratory center in the night. 2. Increased venous return in sleeping position. 3. Decreased adrenergic drive in the night.

SIGNS 1. Cardiomegaly: This is a nonspecific finding and not all patients with heart failure have heart enlargement. For example, patients with constrictive pericarditis or restrictive cardiomyopathy or diastolic heart failure are likely to have Heart Failure 129

TABLE 15.2: Differentiation of PND versus bronchial asthma Features PND Asthma Cough Cough and dyspnea Starts with dyspnea simultaneous Wheezing Rare Usual Sweating Marked Rare Accessory muscles of respiration Not active Active Crepitations Marked Rare Rhonchi Occasional Prominent LV-S3 gallop May be present RV-S3 late feature Peripheries Cold Warm

not much cardiomegaly. On the other hand patients with dilated cardiomyopathy are likely to have enlarged heart. 2. Gallop sounds: Pathological third heart sound is a feature of heart failure. 3. Lung crepitations: With pulmonary congestion, there are fine crepitations heard in the lung fields. 4. Jugular venous pressure is elevated: With systemic venous congestion the jugular veins become engorged and the mean pressure is elevated. 5. Liver is enlarged and tender. 6. Pedal edema: This is a cardinal manifestation of heart failure; a minimum of 4 litres of fluid accumulation is required before peripheral edema is seen. In patients who are bedridden this edema is seen over their sacrum.

CHEST X-ray 1. Cardiac size may be increased. 2. Pulmonary venous congestion: This is seen in the form of prominent upper lobe veins, perivascular cuffing or subpleural accumulation of fluid. In severe forms, there is alveolar edema, which is seen as bats wings appearance. 130 Manual of Cardiology

Echo 1. Cardiac size and function can be made out. 2. Ejection fraction can be calculated. The normal ejection fraction is more than 50 percent. 3. Diastolic function can be assessed by assessing the mitral Doppler flow.

BRAIN NATRIURETIC PEPTIDE (BNP) OR NT PRO BNP In patients with heart failure increased levels of BNP are found in the blood. Hence checking the BNP level can be a useful test for the diagnosis of heart failure when in doubt.

Treatment Angiotensin-Converting Enzyme (ACE) Inhibitors (Table 15.3) ACE inhibitors are recommended as a first line therapy for patients with reduced LV systolic function.

TABLE 15.3: Recommended dose of ACE-inhibitors Drug Initiating dose Maintenance dose Captopril 6.25 mg t.i.d. 25-50 mg t.i.d. Enalapril 2.5 mg daily 10 mg b.i.d. Lisinopril 2.5 mg daily 5 to 20 mg daily Ramipril 1.25 to 2.5 mg daily 2.5 to 5 mg b.i.d. Trandolapril 1 mg daily 4 mg daily

Angiotensin Receptor Blockers ARB (Losartan, Irbesartan, Valsartan) are similar to ACEI, but they do not interfere with the bradykinin pathway, hence they do not produce cough. These drugs are equivalent to ACEI but are not superior. Hence they are recommended when ACEI are contraindicated. Recently a combination of ACEI and ARB has been tried with the hope that a complete inhibition of Renin angiotensin system will improve the prognosis. This combination did result in a significant reduction in hospitalization but no difference in mortality. Heart Failure 131

Diuretics These drugs are recommended for symptomatic treatment if pulmonary congestion or peripheral edema is present (Table 15.4). Loop diuretics: These drugs are commonly used. Frusemide (Lasix), Torsemide (Dytor), Bumetanide (Bumux) are the ones available. Mechanism of action: They act at the thick ascending limb of the loop of Henle; inhibition of the Na+/K+ /2Cl– cotransporter.

TABLE 15.4: Dosages Initial (mg) Maximum dose (mg) Loop Frusemide 20-40 250-500 Torsemide 5 100-200 Bumetanide 1 5-10 Thiazides Bendroflumethiazide 2.5 10 Metolazone 2.5 10 Potassium sparing* Amiloride 5 40 Triamterene 50 200 Spironolactone 50 100-200

* If potassium sparing diuretics are used along with ACEI, the dose should be half of the recommended dose. Aldosterone Antagonists: Based on the results of RALES study, Spironolactone (Aldactone), an aldosterone antagonist is now recommended to all patients who are in NYHA Class 3 or 4. Thirty percent reduction in mortality was observed in that study. The main side effect of this drug is gynecomastia, impotence, and hyperkalemia.

Digitalis Mechanism of action: Inhibits Na+K+ATPase which results in high levels of intra cellular sodium, which in turn results in more calcium for contraction. 132 Manual of Cardiology

Dose: About 0.125 mg to 0.25 mg per day for five days or 6 days in a week. Side Effects 1. Anorexia, Nausea. 2. Arrhythmias such as ventricular premature beats, AV block, atrial tachycardia with block. 3. Altered vision—xanthopsia—yellow vision. Digoxin levels: The serum level of digoxin can be monitored. A level of 2.5 nmol/L or more suggests toxic level.

Beta blockers These drugs should be considered for patients with reduced LV ejection fraction on standard treatment. a. Start with a very low dose. For example, carvedilol 3.125 mg, bisoprolol 1.25 mg, nebivolol 1.25 mg. Increase the dose once in two to three weeks. b. Look for worsening of heart failure, hypotension or bradycardia. c. Do not give to patients with known asthma or hypotension or bradycardia.

Bi-Ventricular Pacing—Cardiac Resynchronization Therapy (CRT) In a small group of patients with heart failure, the left ventricular contraction is altered in the sense that lateral wall contracts much later than the other segments. The net result is that there is no contribution to cardiac output by the late contraction of the lateral wall and in fact there is waste of energy. Hence, if the lateral wall is made to contract simultaneously along with the septum, then the cardiac output will increase. This is done by placing three electrical wires in the heart. One in the atrium, second in the right ventricle and the third in the coronary vein to stimulate the lateral wall of left ventricle. Heart Failure 133 Heart Failure Digoxin antagonists care) care) Betablocker Aldosterone retention intolerant with orwithout ACEI if fluidwith orwithout ACEI of diuretics combination specialist with or (under without ACEI of diuretics combination specialist (under Severe • Summary of drug therapy for heart failure ACEinhibitors ARB Diuretics TABLE 15.5: Asymptomatic LV dysfunctionSymptomatic HF (NYHA II) Indicated Indicated If ACEWorsening HF (NYHA III-IV) Indicated Not Indicatedindicated Post MIEnd-stage HF (NYHA IV) Indicated Recent MI Indicated Atrial Fib Indicated Indicated Indicated, Recent MI • Atrial fib Indicated Indicated Indicated Indicated, Indicated Indicated Indicated Indicated 134 Manual of Cardiology

In order to identify which patients are likely to benefit from this form of therapy, Tissue Doppler is used. ECG may be helpful, especially if there is LBBB. The following are the indications for bi-ventricular pacing and the clinical effects are summarized in Tables 15.6 and 15.7.

TABLE 15.6: Indications for biventricular pacing

Severe NYHA class III-IV heart failure despite optimal drug treatment Aetiology non-ischemic or ischemic heart disease QRS > 130 ms LVEDD > 55mm LVEF < 35 percent

TABLE 15.7: Effects of biventricular pacing Improves: Quality of life Exercise tolerance NYHA class LV ejection fraction LV dimensions Heart rate variability Reduces: Total and heart failure related hospitalizations Heart rate

Cardiac Transplantation This procedure has become the treatment of choice in patients with end stage heart failure whose life expectancy is shortened. The expected 5 year survival is around 70 percent. The main benefit is the quality of life. However as in any transplantation, the complications following the procedure are numerous and they are given below: 1. Allograft rejection 2. Infections 3. Allograft vascular disease 4. Malignancy 5. Hypertension. Heart Failure 135

ACUTE PULMONARY EDEMA It is a life threatening medical emergency. Patient is severely dyspneic, unable to lie down; the sputum is pink and frothy; cyanosis may be present. On auscultation, the lung is flooded with crepitations.

Treatment 1. Sitting position. 2. Oxygen by mask. 3. Morphine 2 mg—IV. 4. Frusemide 40 mg IV or Bumetamide 1 mg. 5. After load reduction with sodium nitroprusside drip if systolic BP > 100 mmHg. 6. Admit the patient to ICU or High Dependency Unit (HDU) for observation and monitoring. 7. Treat the cause of acute pulmonary edema, such as ischemia or valvular heart disease.

Section V CARDIOMYOPATHY 138 Manual of Cardiology

The cardiomyopathies are a group of diseases of the heart muscle associated with cardiac dysfunction. It is not secondary to hypertension or valvular, coronary or pericardial diseases.

CLASSIFICATION Cardiomyopathies are classified as: 1. Dilated cardiomyopathy. 2. Restrictive cardiomyopathy. 3. Hypertrophic cardiomyopathy. 4. Ischemic cardiomyopathy: Extensive coronary artery disease can result in multiple myocardial scars which can result in LV dysfunction. 5. Arrhythmogenic RV cardiomyopathy—rare form. The characteristic features of the main types of cardio- myopathy can be identified with two dimensional and Doppler Echo on the basis of chamber size, wall thickness and function of the ventricle (Table S-V.1).

TABLE S-V.1: Comparison of dilated, hypertrophic, and restrictive cardiomyopathies Feature Cardiomyopathy Dilated Hypertrophic Restrictive Cavity size Enlarged Small Normal Wall thickness Normal Marked Normal Systolic function Severely Hyperdynamic Normal/reduced depressed Diastolic function Abnormal Abnormal Abnormal Others Outflow tract obstruction Dilated Cardiomyopathy 139 DILATED 16 CARDIOMYOPATHY

The WHO has defined Dilated Cardiomyopathy (DCM) as myocardial disease characterized by dilatation and impaired contraction of the left ventricle or both left and right ventricles.

ETIOLOGY 1. Idiopathic. 2. Late sequelae of acute viral myocarditis. 3. Postchemotherapy—doxorubicin. 4. AIDS. 5. Peripartum cardiomyopathy. 6. Associated with muscular dystrophy. 7. Tachycardia induced. 8. Alcohol induced.

CLINICAL FEATURES 1. Occurs more frequently in males than females—3:1. 2. Symptoms of dyspnea and swelling of the legs. 3. Cardiomegaly. 4. 3rd and 4th heart sounds are common. 5. Mitral and tricuspid regurgitation may occur.

Chest X-ray Moderate to severe cardiomegaly, pulmonary venous hyper- tension. 140 Manual of Cardiology

ECG Sinus tachycardia or atrial fibrillation, left atrial enlargement, diffuse nonspecific ST changes.

Echo Left ventricle is dilated and the ejection fraction is reduced.

TREATMENT The treatment of DCM is essentially the treatment of heart failure 1. ACE inhibitors. 2. Diuretics. 3. Digoxin. 4. Beta blockers: Carvedilol and metoprolol have been found to be useful in patients with heart failure. However these drugs should be started on a small dose and gradually increased. 5. Spironolactone should be added for patients in class III and class IV 6. Biventricular pacing: Patients with intraventricular conduction delay benefit with this form of pacemaker. 7. Patients who are refractory to medical therapy, cardiac transplantation should be considered. Hypertrophic Cardiomyopathy 141

HYPERTROPHIC 17 CARDIOMYOPATHY

Hypertrophic cardiomyopathy (HCM) is defined as an unex- plained hypertrophy of the left ventricle.

PREVALENCE The prevalence of HCM is thought to be 0.2 percent by ECG screening, 0.5 percent by echocardiographic screening of the population.

PATHOGENESIS 1. There is left ventricular hypertrophy, often with preferential hypertrophy of the interventricular septum—Asymmetrical septal hypertrophy (ASH). 2. In addition there is mid systolic apposition of the anterior mitral wall leaflet against the hypertrophic septum—Systolic anterior motion (SAM). 3. In about 25 percent of patients SAM is responsible for left ventricular outflow tract obstruction. This LVOT obstruction is usually dynamic. The obstruction varies depending upon the loading conditions of the heart. If there is an increase in the preload, then there is a decrease in gradient. If there is an increase in the contractility, the gradient increases. 4. In the remaining 75 percent of patients, the left ventricular hypertrophy results in increased stiffness of the ventricle, 142 Manual of Cardiology

increased diastolic filling pressure, leading onto diastolic dysfunction.

TYPES OF HCM 1. Subaortic obstruction. 2. Midventricular obstruction. 3. Apical obstruction. 4. Nonobstructive.

GENETIC CAUSES About 50 percent of all patients have a family history of HCM. 10 different genes have been identified. 1. 40 percent of these are related to cardiac beta myosin heavy chain on chromosome 14. 2. About 15 percent have mutation on cardiac troponin T gene on chromosome 1. 3. In 20 percent, there is mutation of myosin binding protein C in chromosome 11.

CLINICAL FEATURES 1. The most common symptom is dyspnea—90 percent. 2. Angina is present in 75 percent of cases. 3. Apical impulse—has a double or triple in nature; there are two peaks in systole, as shown in the Figure 17.1.

FIGURE 17.1 Showing that the apex beat has 2 peaks in systole Hypertrophic Cardiomyopathy 143

4. JVP: prominent A wave. 5. Carotid pulse: Rapid and jerky. 6. S4 may be present. 7. S3 may be present. 8. Second sound is paradoxically split in severe cases. 9. Systolic murmur is crescendo—decrescendo, radiating to base and apex. Systolic murmur is best heard at the third and fourth intercostal spaces. 10. Mitral regurgitant murmur. 11. Valsalva maneuver: Forced expiration against closed glottis for 10 seconds. During the strain phase of valsalva, the murmur is increased. 12. Standing: Since the preload is reduced the murmur increases. Effect of Valsalva in HCM murmur: Valsalva strain—Murmur increases Valsalva release—Murmur decreases

TABLE 17.1: Methods to differentiate obstructive versus non obstructive HCM Obstructive Nonobstructive JVP A wave Prominent Prominent Carotid pulse Jerky Normal Palpable S4 Common Common Systolic thrill Present Not present Double apex beat Present Not present Murmur Long, louder Shorter or absent

ECG 1. ST—T changes: ST depression and T inversions. 2. LVH by voltage criteria. 3. Giant T wave inversions. 4. Prominent q waves: inferior and in precordial leads. 5. Left axis deviation. 6. Left atrial enlargement. 7. Pre-excitation. 144 Manual of Cardiology

Echo Echo and Doppler is useful in confirming the diagnosis and in assessing the severity of LVOT obstruction.

FIGURE 17.2: shows a long axis view of 2D echo in a patient with HCM. Note the marked thickness of interventricular septum, shown by arrow. The thickness of the interventricular septum is much more than the posterior wall of the ventricle. In addition the mitral valve is touching the interventricular septum causing systolic anterior motion of the mitral leaflet—SAM.

NATURAL HISTORY • 20 years of age murmur is detected • 30 years NYHA Class 2 • 35 years NYHA Class 3 • 40 years NYHA Class 4 or death Annual mortality is 3 percent per year, in younger individuals it may be 6 percent per year. Atrial fibrillation is common late in the course of disease and this leads on to severe increase in the symptoms. In 10 percent of individuals HCM may progress through dilatation of the ventricles. The major reason for mortality is sudden death. The risk factors for the same have been identified and they are summarized ahead. There is no correlation between the risk of sudden death and the severity of symptoms. Hypertrophic Cardiomyopathy 145

Risk Factors for Sudden Death Major Risk Factors 1. Cardiac arrest (ventricular fibrillation). 2. Spontaneous sustained ventricular tachycardia. 3. Family history of sudden death (one or more first degree relative younger than 40 years of age).

Minor Risk Factors 1. Age less than 30 years. 2. Syncope (Two or more episodes syncope within one year). 3. Holter—Non Sustained Ventricular Tachycardia (one or more runs are with a rate higher than 120 beats per min and duration of less than 30 secs. 4. Gene screening: High risk genes as given above. 5. Treadmill testing: Abnormal blood pressure response to exercise. Failure to increase the blood pressure to 25 mm of Hg during exercise or if there is a fall in BP of more than 10 mmHg with exercise 6. Echo: LVH—more than 30 mm 7. Sestamibi testing—Nuclear medicine: Showing perfusion defects suggestive of micro vascular obstruction.

Treatment (Figure 17.3) 1. Medical therapy. 2. Alcohol septal ablation. 3. MORROW procedure. 4. Dual chamber pacing. 5. Mitral valve replacement.

Medical Therapy 1. Beta blockers are generally the initial drug of choice for patients with symptomatic HCM and is effective in 60 to 80 percent of patients. 146 Manual of Cardiology

2. Verapamil is given for patients, who cannot tolerate beta blockers. Death has been reported in patients with severe symptoms, pulmonary hypertension and severe outflow tract obstruction. Hence, verapamil should not be used in patients with severe symptoms. 3. For patients who are not controlled with the beta blocker, addition of Disopyramide should be considered since its negative inotropic effect further decreases the gradient. It is recommended that disopyramide be given with a beta blocker to prevent a rapid ventricular response if atrial fibrillation occurs.

Alcohol Septal Ablation (PTSMA) In 1994, Sigwart injected a small quantity of absolute alcohol into the first septal artery of a 67-year-old woman with HCM and, hence, this procedure is also called as Sigwart procedure. The other term is PTSMA - Percutaneous Transluminal Septal Myocardial Ablation. This procedure is indicated if the patient is symptomatic despite medical treatment. This is currently the favored treatment in patients not responding to medical therapy.

Surgery—Myectomy In this procedure a small amount of inter ventricular septum is removed surgically. This procedure is also called as MORROW procedure. This procedure is indicated if the patient is symptomatic despite medical treatment.

Dual Chamber Pacing (DDD) In this procedure the ventricle is paced so that there is altered activation sequence of the ventricle. This results in reduced gradient across the left ventricle outflow tract. This procedure is effective in only around 50 percent of individuals. Hypertrophic Cardiomyopathy 147

Surgery—Mitral Valve Replacement If the mitral valve is removed surgically, then there is no obstruction of the left ventricular outflow tract due to anterior motion of the mitral leaflet. However, the patient will have to be on life long anticoagulation for the prosthetic valve. Hence this procedure is not routinely recommended except in individuals with severe associated mitral regurgitation.

FIGURE 17.3: Management plan for HCM 148 Manual of Cardiology

RESTRICTIVE 18 CARDIOMYOPATHY

RCM is defined by WHO as “characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near normal systolic function and wall thickness”: 1. Normal sized ventricular chambers. 2. Variable reduction in systolic function. 3. Restrictive diastolic function.

ETIOLOGY 1. Idiopathic. 2. Eosoniphilic endomyocardial disease. 3. Endomyocardial fibrosis (EMF). 4. Infilterative cardiomyopathies: Amyloid, hemochromatosis. 5. Scleroderma. 6. Postmediastinal irradiation.

FEATURES OF RCM Usually present with severe symptoms of failure of the right side of the heart and low output state. Fatigue, shortness of breath, edema and asities are the most common symptoms. On physical examination the jugular venous pressure is elevated with rapid X and Y descents. Murmurs of mitral and tricuspid regurgitation may be present. S3 is nearly always found. Restrictive Cardiomyopathy 149

EMF (Davies disease) This disease is common in some parts of Kerala and Orissa. In 50 percent of cases both the ventricles are involved; in 40 percent, only the left ventricle is involved; in 10 percent of cases right ventricle is involved. In right ventricular involvement, there are features of right heart failure. Right ventricular third heart sound and tricuspid regurgitant murmur are heard. Chest X-ray shows right atrial enlargement. In left ventricular involvement, there is left ventricular third heart sound and mitral regurgitant murmur.

Echo In EMF there is obliteration of the apex and dilatation of the atrium.

MEDICAL TREATMENT 1. Diuretics are helpful to treat congestive symptoms. 2. Digoxin may be tried in small doses. 3. Surgery may be done to remove the endocardium and replacement of mitral valve. 4. Heart transplantation should be considered for patients with refractory symptoms.

DIFFERENTIAL DIAGNOSIS The most important differential diagnosis for restrictive cardiomyopathy is constrictive pericarditis. 1. In constriction the pericardium is thickened and this can be well demonstrated by MRI/CT. 2. Respiratory variation of Doppler flow is an important finding in constriction. 3. The end diastolic pressures are elevated and there is a difference of more than 5 mmHg between left and the right ventricles in RCM. 150 Manual of Cardiology

The major differences are summarized in the Table 18.1 given below:

TABLE 18.1: Differentiating features of constriction versus restriction Investigation Constriction Restriction X-ray Calcification in 50 percent — ECG Not useful Not useful Echo Thick pericardium Small LV cavity, enlarged atria, increased wall thickness Mitral Doppler With inspiration, decrease of No respiratory variation more than 25 percent Cath 1. RVEDP more than 1/3 of 1. RV systolic pressure RV systolic pressure more than 60 2. Diastolic pressures are 2. more than 5 mm Hg equal in RV and LV difference between (difference < 5 mmHg LVEDP and RVEDP 3. Square root sign of RV pressure wave form Radio Nuclear 80 percent filling in first half 40 percent filling in first Studies of diastole half of diastole CT/MRI Pericardium is thickened No pericardial thickness Acute Pericarditis 151

Section VI PERICARDIUM AND AORTA 152 Manual of Cardiology

The pericardium is made up of two layers. The visceral pericardium is adherent to the heart surface and the parietal pericardium is about 2 mm thick and surrounds the heart. The space in between these two layers contains a small amount of serous fluid and this can be up to 50 ml. The pericardial diseases can be broadly categorized into 1. Acute pericarditis. 2. Pericardial effusion. 3. Cardiac tamponade. 4. Constrictive pericarditis. Etiology of pericarditis is common to all the diseases enumerated above and it is given below:

ETIOLOGY OF PERICARDIAL DISEASES a. Infections Viral—coxsackie virus, adenovirus, hepatitis B, infectious mononucleosis, HIV. Bacterial—pneumococcus, staphylococcus, streptococcus. Mycobacteria—tuberculosis. Fungal—histoplasmosis. b. Connective tissue disorders—rheumatoid arthritis, systemic lupus, scleroderma. c. Neoplastic disorders—leukemia, lymphoma, lung cancer, breast cancer. d. Trauma. e. Radiation—secondary to mediastinal radiation. f. Uremia. g. Myocardial infarction. h. Miscellaneous—hypothyroidism, aortic dissection. Acute Pericarditis 153 ACUTE PERICARDITIS 19

Acute pericarditis is due to pericardial inflammation of less than two weeks in duration.

HISTORY Chest pain is the classical feature. Site: Retrosternal. Quality: Pleuritic—the pain is aching in character and aggravated by respiratory movements. Radiation: The pain radiates to shoulder edge. Occasionally may radiate to the left arm. Relieving factor: The pain is almost always relieved by sitting forward. Associated symptoms: Cough, hiccoughs, sometimes dyspnea.

PHYSICAL EXAMINATION Pericardial rub is the most important feature. It consists of three components: 1. Systolic component—during ventricular systole. 2. Early diastolic component during rapid filling. 3. Presystolic component due to atrial contraction. The rub is loudest along with left sternal border and it is best heard with the patient sitting and leaning forward. 154 Manual of Cardiology

Most often only a two component rub is heard; the systolic component followed by the presystolic one. The rub is superficial and scratchy in quality.

ECG This is the most important investigation for acute pericarditis. 1. ST segment is elevated with the concavity upwards 2. ST segment elevation is seen in all leads except aVR and V1. 3. PR segment is depressed. 4. T wave inversions occur after the ST segment comes down to baseline.

FIGURE 19.1: The above ECG shows diffuse ST segment elevation in all the leads except V1 and aVR.

TABLE 19.1: Differential diagnosis of ECG in acute pericarditis versus acute STEMI Pericarditis STEMI ST elevation Concave upward Convex upward Leads All leads except aVR Region wise inferior/ and V1 anterior Evolution of ST ST is elevated for several T inversion within a and T days. T inversion after ST few hours, while ST returns to baseline is still elevated PR depression Yes No Acute Pericarditis 155

TESTS FOR ETIOLOGIC DIAGNOSIS* Stage 1 1. Basic tests such as Chest X-ray, ECG, Echo and complete blood count. 2. If the disease does not remit in one week, rheumatoid factor, anti DNA antibodies, sputum AFB. 3. If pleural effusion is present then tapping of the same is done and fluid is analyzed.

Stage 2 Pericardial fluid aspiration is pyogenic effusion or cardiac tamponade is present.

Stage 3 Subxiphoid pericardial drainage and biopsy. This is done if pericardiocentesis is ineffective or tamponade relapses or in patients in whom three weeks of intense investigation does not result in an etiological diagnosis.

TREATMENT Idiopathic pericarditis is a self limiting disease. Hence the treatment needs to be supportive. 1. Nonsteroidal anti-inflammatory drugs (NSAID’s) are preferred. Ibuprofen or indomethacin can be used. 2. Colchine if added to the above regimen and given for three months, reduces the recurrence rates considerably. (COPE trial) 3. For patients, who are not responding, a short course of steroids may be used.

COMPLICATIONS 1. Pericardial effusion. 2. Cardiac tamponade. 3. Constrictive pericarditis. 4. Recurrent pericarditis.

* Permanyer Miralda, acute pericardial disease: approach to aetiologic diagnosis. Heart 2004;90:252-4. 156 Manual of Cardiology

PERICARDIAL EFFUSION 20

In pericardial effusion, there is accumulation of fluid within the pericardium. The causes of pericardial effusion are the same as for acute pericarditis (Refer Chapter 19). The term cardiac tamponade is used if the pericardial effusion restricts the filling of the heart and impairs the outflow of the blood from the heart. The three common causes of large pericardial effusions are: 1. Tuberculosis 2. Viral 3. Idiopathic

HISTORY If the effusion develops slowly over a period of time then there will be no symptoms. If there is a rapid accumulation of fluid then patients’ present with dyspnea. If the effusion is large and constricting the filling of the heart, then patient may develop symptoms of low blood pressure such as giddiness and shock.

PHYSICAL EXAMINATION 1. Pulse: In patients with cardiac tamponade, there is paradoxical pulse. In this there is a fall of more than 10 mmHg systolic blood pressure with inspiration. 2. Jugular venous pressure is elevated and the Y descent is absent. 3. Heart sounds are faint or not heard. 4. Beck’s triad: In patients with acute cardiac tamponade, the jugular venous pressure is elevated, the heart sounds Pericardial Effusion 157

are faintly heard—quiet heart and fall of blood pressure is noted.

Echo This is the investigation of choice for patients with suspected pericardial effusion (Figure 20.1). In patients with cardiac tamponade echocardiogram will show the right ventricle and the right atrium collapses during ventricular systole.

FIGURE 20.1: The above figure is a long axis view of the left ventricle. The star is placed in the pericardial space which shows pericardial effusion.

Chest X-ray Cardiomegaly is seen in proportion to the amount of fluid. If more than 250 ml of fluid is present, the heart assumes a globular configuration (Figure 20.2).

ECG Low voltage (QRS complexes are 5 mm in height in limb leads and 10 mm in precardial leads) and electrical alternans may be seen. 158 Manual of Cardiology

FIGURE 20.2: The above figure shows marked cardiomegaly with a globular shaped heart suggestive of pericardial effusion.

Chest CT1 This test is useful if tuberculosis is suspected as a cause for the pericardial effusion. Enlarged mediasternal lymph nodes > 10 mm are detected in nearly all patients. The nodes involved are aorto pulmonary, para-tracheal and carinal ones. Hilar nodes are least affected.

Pericardial Fluid Analysis 1. Pericardial fluid is assessed to find out whether it is an exudate or transudate, using Light’s criteria. Exudates will have one of the following: a. Fluid protein divided by serum protein > 0.5. b. Fluid LDH divided by serum LDH > 0.6. c. Fluid LDH > 66% of normal for serum LDH. 2. PCR for Tuberculosis DNA: This test is less useful for the fluid rather than for tissue. 3. Adenosine deaminase (ADA): A value above >35 U/L is suggestive of tuberculosis as an etiology. 4. Lysozyme levels are elevated in tuberculous pericardial effusion. The cut off level is 6.5 μg/dL. 5. Interferon - γ (IFN - γ): This test is elevated in tuberculous effusion and the cut-off value is >200 pg/L. Pericardial Effusion 159

Pericardial Biopsy2 Pericardial biopsy may also be used for etiologic diagnosis. 1. ‘Diagnostic’ biopsy: In areas where tuberculosis is endemic, biopsy is not required before commencing treatment. Nonendemic areas it is useful. 2. ‘Therapeutic’ biopsy: This is done, when surgical drainage is done in patients with severe tamponade and they are undergoing pericardial drainage.

TREATMENT 1. Pericardiocentesis: If there is cardiac tamponade then it is a medical emergency and the fluid need to be drained percutaneously. If the needle cannot be passed, then the patient will require surgical drainage. 2. For patients, whom there is no cardiac tamponade, fluid is aspirated for diagnosis only. Based on the fluid analysis, further treatment is planned.

REFERENCES 1. Cherian G. Diagnosis of tuberculosis etiology in pericardial effusions. Postgraduate Medical Journal 2004; 80: 262-6. 2. Mayosi BM, Burgess LJ, Doobell. Tuberculosis Pericarditis. Circulation 2005; 112: 3608-16. 160 Manual of Cardiology

CONSTRICTIVE 21 PERICARDITIS

In this condition, the pericardium is thickened and may be calcified. As a result, this thickened pericardium forms a rigid shell around the heart restricting the filling of the heart. It is usually due to tuberculosis in countries like India but any condition that can cause pericarditis can lead onto constrictive pericarditis. The most important differential diagnosis is restrictive cardiomyopathy (Refer Table 18.1). The incidence of constriction requiring pericardectomy develops in 50 percent with tuberculosis, 35 percent with pyogenic pericarditis and 17 percent with neoplastic pericarditis. Constriction is very rare with idiopathic pericarditis.

CLINICAL FEATURES 1. Jugular venous pressure is grossly elevated. 2. Y descent of the venous waveform is prominent (Fredrick’s sign). (See Figure 4.4). 3. Kussmaul’s sign: There is an inspiratory increase of jugular venous pressure; normally during inspiration, the venous waveforms may become prominent but there is a slight fall in the mean pressure. 4. Pericardial knock: It is a high pitched sound heard after the second heard sound. It is heard all over the precardium. 5. Hepatomegaly due to congestion. 6. Splenomegaly. 7. Ascitis. 8. Pedal edema is mild in comparison to ascitis. Constrictive Pericarditis 161

INVESTIGATIONS 1. Chest X-ray: On fluoroscopy, calcification can be seen in 50 percent of individuals 2. ECG: Low voltage with T inversions 3. Echo—Doppler: The respiratory variation in the filling of the ventricle can be demonstrated. 4. CT Scan/MRI: Pericardial thickness can be measured accurately by these two methods. 5. Cardiac Cath and Angiogram: The pressures in the heart are equalized in all the chambers in diastole and specific patterns are seen that are useful in the diagnosis.

TREATMENT Constrictive pericarditis is a progressive disease. Surgical pericardiectomy is the treatment of choice. This is associated with 5 to 10 percent of mortality. Digoxin and diuretics are given for control of failure. 162 Manual of Cardiology

DISSECTION 22 OF AORTA

Dissection of aorta (DA) is a life threatening condition in which there is a tear in the intima of aorta, which allows blood to dissect into the wall of aorta.

TYPES OF DISSECTION Stanford Classification Type A: Ascending aorta. Type B: Limited to descending aorta.

DeBakey Classification Type I : Ascending and descending aorta. Type II: Ascending aorta. Type III: Descending aorta.

ETIOLOGY Cystic medial degeneration is the chief predisposing factor for DA. This is common in conditions such as Marfan’s syndrome.

SYMPTOMS Sudden onset of severe chest pain with ‘tearing’ or ‘ripping’ in quality; pain may radiate to the back or to legs.

SIGNS 1. Pulse deficits is seen in 30 percent of patients. Pulses are absent in the carotids or in the brachials. Dissection of Aorta 163

2. Aortic regurgitation is seen in one-third of patients. 3. Neurological deficits are seen infrequently and this may manifest in the form of cerebrovascular accident, or paraplegia.

Chest X-ray Widening of the mediastinum.

CT/MRI/TEE The above three tests can confirm the diagnosis of DA.

TREATMENT 1. Sodium nitroprusside (SNP) 20-400 μ/min/IV 2. Beta blocker a. Esmolol (or) 500 μ/kg/IV over 1 min; 50-200 μ/min/IV b. Labetalol 20 mg IV over 2 mins; 40-80 mg q 10 mins. The above medical treatment is given to all patients. If it is proximal type of dissection, the patient should go for surgery. If it is distal, then patient can be managed medically, stabilized and surgery done as needed later.

Section VII CONGENITAL HEART DISEASE 166 Manual of Cardiology

A heart defect present from birth but can manifest at any time in life.

INCIDENCE The overall incidence is 8 per 1000 live births. About 85 percent of all congenital heart disease is accounted by the following 8 lesions. (Table S-VII.1)

Table S-VII.1: Frequency of congenital heart disease Disease Frequency Ventricular Septal Defect 30% Atrial Septal Defect 10% Patent Ductus Arteriosus 10% Pulmonary Stenosis 7% Coarctation of Aorta 7% Aortic Stenosis 6% Tetralogy of Fallot 6% Transposition of Great Arteries 4%

ETIOLOGY In most patients no specific etiology can be made out. In 5 percent of patients chromosomal abnormalities are present. The details of the same are given below:

TABLE S-VII.2: Chromosomal abnormalities in CHD Abnormality / Name Incidence Type of heart disease 21 Trisomy – 50% AV canal defect Down’s syndrome 1 in 600 live births 18 Trisomy – 90% VSD, PDA, DORV Edward’s syndrome 1 in 8,000 live births 13 Trisomy – 90% Dextrocardia, VSD, PDA Patau’s syndrome 1 in 20,000 live births XO –Turner syndrome 15% Coarctation of aorta, AS 1 in 2,500 female live births CongenitalAtrial Heart Septal Disease Defect 167

The following inciting agents can lead onto heart defects.

TABLE S-VII.3: Maternal factors in CHD Inciting agent Type of heart disease Rubella PDA, PAS Mumps Endocardial fibroelastosis Alcohol VSD, TOF Vitamin D Supravalvar AS Phenytoin VSD, ASD Thalidomide PS Diabetes TGA SLE Congenital complete heart block

TYPES OF CONGENITAL HEART DISEASE 1. Left to right shunts: ASD, VSD, PDA. 2. Obstructive lesions: Aortic stenosis, Pulmonary stenosis. 3. Cyanotic lesions: Tetralogy of fallot.

LEFT TO RIGHT SHUNTS 1. Recurrent respiratory infections. 2. Increased sweating. 3. Failure to thrive. 4. Tendency of heart failure. 5. Cardiomegaly. 6. Shunt and flow murmur. 7. Chest X-ray – Increased pulmonary blood flow (plethora).

OBSTRUCTIVE LESIONS 1. Absence of cyanosis or history of recurrent infections. 2. Normal shape of precordium. 3. Forcible or heaving apex. 4. Delayed corresponding S2. 5. Ejection systolic murmur. 6. Absence of diastolic murmurs unless valvar stenosis is associated with leaking valve. 168 Manual of Cardiology

7. Chest X-ray: Normal sized heart. 8. ECG : Ventricular hypertrophy.

CYANOTIC LESIONS—CYANOSIS, CLUBBING, POLYCYTHEMIA Cyanosis is a word derived from Greek meaning bluish condition. Cyanotic lesions can be classified under four headings

TABLE S-VII.4: Physiology Example Pulmonary stenosis with right to Critical pulmonary stenosis with left shunt at atrial level shunt through PFO or ASD / Ebsteins Pulmonary stenosis with VSD – TOF/DORV/TGA/SV right to left shunt at ventricular level (TOF physiology) Transposition physiology Transposition of great arteries (Increased pulmonary blood flow) Eisenmenger Increased PA pressure with right to left shunt

Pulmonary Stenosis with Right to Left Shunt at Atrial Level 1. Jugular venous pressure shows dominant a wave. 2. Cardiomegaly. 3. Parasternal impulse is marked. 4. S2 widely split. 5. Pulmonary ejection murmur is prominent.

Pulmonary Stenosis with VSD—Fallot’s Physiology 1. Normal cardiac size. 2. Mild parasternal impulse. 3. Systolic thrill uncommon. 4. S2 single. 5. Ejection systolic murmur which varies inversely with the severity of pulmonary stenosis. CongenitalAtrial Heart Septal Disease Defect 169

Transposition Physiology (Increased Pulmonary Blood Flow) 1. Symptomatic in the neonatal period. 2. Cyanosis – mild to severe. 3. Failure to thrive. 4. Congestive cardiac failure. 5. Cardiomegaly. 6. S2 is single. 7. Cardiomegaly with increased pulmonary flow on chest X-ray.

Eisenmenger’s Syndrome In this condition there is severe pulmonary arterial hypertension and there is right to left shunt at the atrial, or ventricular or ductus level. The pulmonary vascular resistance is very high. These patients have severe right ventricular hypertrophy. 170 Manual of Cardiology

ATRIAL SEPTAL DEFECT 23

PREVALENCE ASD’s are the most common form of acyanotic heart disease. The overall frequency is around 5 to 19 percent of congenital cardiac defects after birth.

CLASSIFICATION OF ASD 1. Ostium secundum (fossa ovalis) defects constitute the most common type of ASD, accounting for 60 to 70 percent of all forms of ASD. 2. Ostium primum defects account for 15 to 20 percent of atrial septal defects. These defects occur in the inferoanterior portion of the atrial septum, frequently in association with a cleft in the anterior leaflet of the mitral valve. 3. Sinus venosus defects compose the remaining 5 to 10 percent of septal defects. The location of these defects typically is superior and posterior in relation to the fossa ovalis or, less frequently, located inferiorly to the fossa ovalis. 4. Coronary sinus defect is the least common variety and as the name implies it is located at the site normally occupied by the coronary sinus ostium.

Associated Defects 1. Anomalous pulmonary venous drainage is seen in 15 percent of the secundum and in 85 percent of the sinus venosus variety. Atrial Septal Defect 171

2. Mitral valve prolapse occurs in about 20 percent of individuals. This is due to abnormality of the LV shape in ASD. 3. Mitral regurgitation—the overall prevalence is around 6 percent. 4. Valvular heart disease such as mitral stenosis can co-exist. A combination of ASD with acquired rheumatic mitral stenosis is called Lutembacher’s syndrome.

Hemodynamic Features • The magnitude of the left to right shunt is not dependant on the size of the ASD. • The magnitude of the shunt is dependant on distensibility characteristics of the two ventricles. • The right ventricle is generally more compliant than the left, resulting in less resistance in filling to RA. • In a study done by Steele et al, on 702 patients found that 6 percent had PVR > 7 units/m2 or more. 85 percent of them were women.1 • The infrequency of PAH is due to deferred development of left to right shunt. • The overall incidence of Eisenmenger is around 10 percent.

Physical Appearance • Usually, children with ASD are thin and frail. • Left precordial bulge with Harrison’s grooves in children with large left to right shunt. • Holt-Oram syndrome: In this, the thumb is hypoplastic and triphalengeal, which makes the apposition of the fingertips difficult.

Symptoms • Most infants with ASD’s are asymptomatic. 172 Manual of Cardiology

• Symptoms are noticed in the late teens and twenties and by the age of 40, the majority are symptomatic. Over two- thirds of patients become symptomatic at a mean age of 41 years.2 The reason are as follows: 1. Decreased distensibility of left ventricle. 2. Atrial arrhythmias. 3. Pulmonary hypertension. • Dyspnea and fatigue are the earliest symptoms. Dyspnea occurs because of decreased pulmonary compliance due to large left to right shunt. • In 10 percent of patients the recurrent lower respiratory tract infections can be a feature. The recurrent respiratory infections are due to dilated pul- monary arteries causing compression of adjacent bronchioles, which results in recurrent lower respiratory infections

Precordial Palpation 1. The jugular venous pulse is normal in height with equal crests of A and V waves. If A wave is greater than V wave, it suggests either small shunt or associated pulmonary hypertension or LV failure. 2. On palpation the right ventricular impulse is hyperdynamic. 3. A dilated pulmonary trunk is palpable in the second left intercostal space. 4. A systolic thrill in the pulmonary area indicates associated pulmonary stenosis. Atrial Septal Defect 173

Auscultation 1. First heart sound is split with loud tricuspid component (T1). Mechanism of increased T1 amplitude is believed to be rapid and forceful tricuspid valve closure during early RV contraction. 2. The typical murmur of ASD is systolic murmur in the pulmonary area. a. Crescendo-decrescendo. b. Grade 2 or 3. c. Maximal in the second left interspace. d. Mechanism: Rapid ejection of a large right ventricular stroke volume into a dilated pulmonary trunk accounts for the murmur. The quality of the pulmonic systolic murmur is impure or “superficial” because of proximity of the dilated pulmonary trunk to the chest wall. e. A murmur exceeding Grade 3 means an unusually large shunt or associated pulmonic valve stenosis. 3. Wide, fixed splitting of the second heart sound. The second sound is heard as two components, in both inspiration and expiration. The split does not vary with respiration. The reasons for the same are as follows: a. The split is wide because of the increase capacitance of pulmonary vasculature. b. The split is fixed because the amount of flow through the PA does not vary with respiration. With inspiration there is increased venous return to the right side. The result is that there is corresponding reduction in the left to right shunt as well. 4. Tricuspid mid diastolic murmur is present, if the shunt is two times or more that of systemic flow. 5. An ejection click may be heard in the pulmonary area due to dilated pulmonary artery.

Complications3 1. Arrhythmias: The incidence of atrial arrhythmias such as atrial fibrillation increases with advancing age. The incidence 174 Manual of Cardiology

is about 13 percent in patients older than 40 years and 52 percent in patients 60 years or older. 2. Congestive heart failure: is common after the age of 40 years. 3. Pulmonary hypertension: occurs in 10 percent of patients with atrial septal defect. In a series from CMCH the prevalence was 13 percent. The incidence of PAH in sinus venosus defect is higher than ostium secundum (20% versus 6%)4. 4. In untreated ASDs’ the average age of death is around 36 to 40 years. The annual mortality is 5 to 7 percent in the group of patients above the age of 40 years.

Cyanosis in ASD The possible reasons are: 1. Eisenmenger’s syndrome. 2. Eustachian valve directing the IVC blood to LA. 3. Common atrium.

X-ray 1. Right atrial enlargement is characteristic of ASD. 2. Increased pulmonary blood flow, depending on the degree of shunt. 3. Main pulmonary artery is prominent (on fluoroscopy, Hilar Dance may be made out).

FIGURE 23.1: This chest X-ray shows enlarged pulmonary artery (large arrow) and prominent right descending pulmonary artery (small arrow head) Atrial Septal Defect 175

ECG 1. The most characteristic feature is the incomplete RBBB. 2. Notched R in inferior limb leads; this pattern is called “Crochetege”. 3. ECG is useful in identifying the location of ASD, based on the axis: Ostium secundum: Right axis of QRS (95 to 175 degrees). Ostium primum: Left axis of QRS (-30 to -120 degrees). Sinus venosus: Left axis of P wave. Common atrium: Left axis of P wave and QRS axis.

Echo 1. 2D ECHO can visualize the defect; the sensitivity is 100 percent for ostium primum, 90 percent ostium secundum and 50 percent for sinus venosus type of defect. 2. Color Doppler can visualize to shunt. 3. TEE is very sensitive for the diagnosis of all types of ASD.

FIGURE 23.2: Echo in the apical 4 chamber view, arrow showing large ASD 176 Manual of Cardiology

ASD WITH MITRAL REGURGITATION 1. MVP with MR. 2. Rheumatic MR. 3. Partial AV canal. 4. Complete AV canal.

ASD Closure—SURGICAL or DEVICE ASD can be closed either by surgery or by a device. Closure is advised between 3 and 5 years of age. Surgical closure: Either through a median sternotomy or through a limited lower thoracotomy, the chest is opened, and through the right atrium, the ASD is closed using Dacron patch. Device closure: Amplatzer septal occluder This device has two round disks made up of Nitinol wire mesh linked together by a short connecting waist. This device can be placed across the septum provided there is adequate rim of atrial septum to hold. A rim of 4 to 5 mm is essential. Fischer et al reported their experience with device closure in 236 patients. Only two patients had complication. In 18 patients they could not implant the device because the ASD was large or the device was unstable. At the median follow up of 2 to 3 years complete closure was detected in 94 percent. An overall success of device closure was 84.7 percent.5 Indications for closure: 1. Large shunt ASD—shunt more than 1.5: 1 2. Now a days if the defect is 5 mm or larger by echo, then closure can be recommended. The reason being that it will prevent the development of pulmonary vascular disease and also reduces the incidence of arrhythmias, such as atrial fibrillation, when carried out before 40 years of age.6 Contraindication for closure: Pulmonary hypertension with high pulmonary vascular resistance of > 10 units/m2 Atrial Septal Defect 177

ASD Spontaneous Closure Asymptomatic infants with secundum ASD less than 8 mm should be observed. More than 75 percent of such ASD’s close before 18 months of age and so it is better to wait. There is a relationship between size of ASD and the probability of closure. This is summarized in the Table 23.1.

Table 23.1: ASD size and spontaneous closure

Size of ASD Probability of closure < 3 mm 100% 4mm 94% 5- 6 89% >8 Low probability

Follow-up of Adults with Closure of ASD 1. Patients operated on or before 24 years of age have an expected survival identical to those without heart disease. Those operated after 25 years of age, are likely to have late supraventricular arrhythmias. 2. On follow-up look for the following: a. Residual shunt. b. RV dysfunction. c. Mitral regurgitation if ostium primum. d. Sinus node dysfunction. e. Supraventricular arrhythmias. 3. IE prophylaxis is not required for those more than 6 months after closure.

REFERENCES 1. Steele PM, Fuster V, Cohen M, Ritter DG, McGoon DC. Isolated atrial septal defect with pulmonary vascular disease. Circulation 1987;76:1037-42. 2. Gatzoulis MA, et al. Atrial Arrhythmia after Surgical Closure of Atrial Septal Defects in Adults. NEJM 1999, 340: 839-46. 3. Geggel RL, Mark E. 48 year old women with atrial septal defect and pulmonary hypertension. NEJM 1993;329:864-72. 178 Manual of Cardiology

4. Vogel M, Berger F, Kramer A, Alexi M, Lange PF. Incidence of secondary pulmonary hypertension in adults with atrial septal or venosus defects. Heart 1999;82:30-33. 5. Fischer G, Stieh J, Vebing A, Hoffmann U, Morf G, Kramer M H. Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder. A single centre study in 236 consecutive patients. Heart 2003;89:199-204. 6. Oliver JM, et al. Predisposing conditions for atrial fibrillation inatrial seqptal defect with and without operative closure. Am J Cardiol 2002; 89: 39-43. Ventricular Septal Defect 179

VENTRICULAR 24 SEPTAL DEFECT

PREVALENCE VSD is the most common form of congenital heart disease and accounts for 20 percent of all such defects.

EMBRYOLOGY VSD results from a delay in closure of the interventricular septum beyond the first 7 weeks of intrauterine life.

VSD Location 1. Membranous defects are the most common—80 percent. This part of the septum is a small area close to and under the aortic valve on the left side; on the right side it is related to a septal leaflet of the tricuspid valve. 2. Infundibular - subpulmonary—5 percent. These defects are located under the pulmonary valve when viewed from the right ventricle. 3. Muscular defects—5 to 20 percent. These defects can be located anywhere in the septum such as apical, mid or anterior part of the septum and it tends to be multiple. 4. Endocardial cushion defects or AV septal defects— 5 percent. These are located below the tricuspid valve.

Size of VSD Based on the size of VSD, they can be classified as given below: 1. Small—7 mm or less (shunt is less than 1.4:1). 180 Manual of Cardiology

2. Moderate—8 to 14 mm (shunt is 1.5 to 2.2:1). 3. Large—15 mm or more (shunt is > 2.2:1). Shunt = The amount of blood flow across the pulmonary artery in relation to aortic blood flow.

Hemodynamic Classification a. Restrictive VSD: RV pressure is less than the LV pressure. b. Nonrestrictive VSD: RV pressure is equal to LV pressure. c. Eisenmenger’s syndrome: A nonrestrictive VSD in which there is right to left shunt due to high pulmonary vascular resistance.

Symptoms 1. Small VSD—The child is asymptomatic. 2. Moderate or large VSD—symptoms of heart failure and recurrent respiratory tract infections occur. In addition there can be a delay in the growth and development. Only about 15 percent of all VSD’s are large enough to cause symptoms; however this is still the most common form of congenital heart disease requiring admission into the hospital after first two weeks of life.

Auscultation 1. Pansystolic murmur: The murmur is best heard in the lower left sternal border. Smaller defects produce the loudest murmurs and this murmur is called as Maladie de Roger. When the VSD is large, the murmur tends to be shorter. This murmur is present as early as in the first week of life itself. In VSD, there is increased blood flow across the pulmonary valve, which may produce an ejection systolic murmur. It is difficult to separate this murmur from the pan systolic murmur of VSD. However, because of the combination of the ejection systolic murmur and pansystolic Ventricular Septal Defect 181

murmur, the VSD murmur will appear to be equally heard well in the upper left sternal border; this is called as selective transmission of pansystolic murmur to the upper left sternal border. 2. Mid diastolic murmur: At the mitral area, when the blood flow across the mitral valve is increased more than 2 times this murmur appears in the mitral area and this may be preceded a third heart sound. This murmur is also called as flow rumble. 3. Second sound: Since there is left to right shunt; the P2 is delayed. Aortic components occur early, since the LV has 2 outlets—one VSD and second the aorta. Therefore the second sound is widely split, but varies with respiration in patients with large VSD.

ECG 1. Small VSD—ECG is normal. 2. Moderate VSD—left ventricular hypertrophy. In addition if there is significant volume overload of the right ventricle there will be associated rsR pattern in the right precordial leads, namely V1. (Figure 24.1). 3. Large VSD—in this there is hypertrophy of both the ventricles. In addition to the volume overload of both the ventricles. Hence there is ECG manifestation of biventricular hypertrophy. In infancy, biventricular hypertrophy manifests in the form of increased mid precordial voltages of more than 45 mm. (Katz Wachtel Phenomenon).

X-ray Small VSD—chest X-ray is normal. Moderate or large VSD—there is varying degrees of cardiomegaly which involves the left ventricle and left atrium. There is an increased pulmonary vascular marking related to increased blood flow across the lung. 182 Manual of Cardiology

FIGURE 24.1: The above ECG shows left ventricular hypertrophy and large equiphasic complexes in mid precordial leads V3 and V4. rSR complex in V1 suggests RV volume overload

FIGURE 24.2: This chest X-ray is from a child with VSD and increased pulmonary blood flow. Note the enlarged Main Pulmonary Artery (MPA—white arrow). In addition the heart is enlarged. The pulmonary arteries in the lung fields are also very prominent due to increased pulmonary blood flow Ventricular Septal Defect 183

Echo Two dimensional echo and color Doppler help to identify the location of the defect, size of the defect. Doppler Echo gives information regarding the pressure difference across the ventricles, and gives an indirect estimate of the RV pressure. The later helps us to understand whether patient has got pulmonary hypertension.

FIGURE 24.3: The Echo image shows aneurysm of the membraneous septum with small left to right shunt. The arrow shows the bulge in the membraneous septum

Cardiac MRI This modality of imaging can also be used in defining the anatomical location of VSD.

Cardiac Catheterization This test is reserved for cases in which anatomical localization of VSD is required prior to surgery. This is done only if sufficient information is not available from 2D Echo Doppler. Secondly, this test is done precisely quantify the shunt. Thirdly, it is useful in measuring the PA pressure accurately prior to surgery. This 184 Manual of Cardiology information is required in patients with pulmonary hypertension. If the pulmonary vascular resistance is > 7 wood units, the patient is usually inoperable. Further studies may be required to assess the feasibility for surgery.

Natural History Spontaneous closure of the VSD Ninety percent of the VSD’s that are destined to close will close by 8 years. It is due to reduplication of the tricuspid valve tissue, adhesion of the TV leaflet to the defect or prolapse of an aortic cusp are the more common mechanisms; previously it was believed that septal aneurysm was responsible. Small VSD’s are more likely to close than large VSD’s.

Pulmonary Hypertension About 15 percent of patients with VSD in the 20 years old group have pulmonary vascular disease. In some patients this can develop as early as 6 to 12 months of age and the next peak occurs in the second decade.

Aortic Regurgitation Aortic regurgitation, in association with VSD was first described in 1921. The mechanism of aortic regurgitation differs depending upon the type of VSD. a. With subaortic VSD, the incidence is 5 percent; this is due to sagging or herniation of the right or both right and non- coronary cusp. The structural basis for incompetence of the aortic valve is due to developmental fault at the right coronary and noncoronary commisure and contiguous aortic sinuses. Poor leaflet support is aggravated by the high velocity jet. b. The incidence is 30 percent with subpulmonic; here the reason is due to lack of leaflet support. Ventricular Septal Defect 185

Infective Endocarditis Infective endocarditis increases with age; the incidence is 1:200 to 1:1200 patient years.

Infundibular Pulmonary Stenosis Some patients with VSD have associated infundibular pulmonary stenosis. This is a variation of TOF. The infundibular PS develops in later part of life.

Medical Management 1. Control of heart failure. 2. Infective endocarditis for dental/surgical procedures. 3. Correction of anemia.

Indications for Surgery—VSD Closure 1. Before 3 months of age: Symptomatic. 2. Three months to 6 months of age: Symptomatic, growth failure, increasing pulmonary hypertension. 3. Six months - primarily based on pulmonary hypertension. If normal, then can wait till 1 year. Otherwise surgery can be done. 4. After one year of age a significant left to right shunt indicates that the patient requires closure.

Complications of Surgery 1. Complete heart block. 2. Residual VSD.

Device closure of VSD Using devices it is possible to close VSD’s that are muscular in location. 186 Manual of Cardiology

PATENT DUCTUS 25 ARTERIOSUS

PREVALENCE PDA is the 6th most common congenital heart disease. The overall prevalence is around 10 percent. It is three times more common in females. Children born of mothers, who had rubella have a high incidence of PDA.

HEMODYNAMICS 1. There is a persistence patency of the ductus arteriosus, which connects the left pulmonary artery and the descending thoracic aorta. The connection is around 5 to 10 mm distal to the left subclavian artery. 2. It is cone shaped with a small orifice with at the pulmonary artery level. 3. It is derived from left 6th embryologic arch.

HISTORY 1. Patients are usually asymptomatic if the ductus is small. 2. If there is a large shunt, it causes recurrent lower respiratory tract infections and heart failure.

PHYSICAL EXAMINATION 1. Apex Beat: Apex beat is hyperkinetic in patients with significant shunt. If the PDA is small the apex beat is normal. Patent Ductus Arteriosus 187

2. Pulse: Because of aortic run off to pulmonary artery there is bounding character of the pulse. 3. Blood Pressure: Wide pulse pressure with significant shunt. 4. Suprasternal pulsations are also seen. 5. Palpable thrill in the 2nd left intercostal space and first intercostal space may be felt.

AUSCULTATION 1. The classical murmur is a continuous murmur, loudest at the 2nd intercostal space. This murmur peaks around the second heart sound. It may be accompanied by a thrill. This murmur is also heard in the first left intercostal space and infraclavicular area. 2. EDDY sounds are heard when the ductus is large; these are multiple clicks heard during the continuous murmur. 3. Mid-diastolic Murmur at the mitral area. If the shunt is large, there is a mid-diastolic murmur at the mitral area due to increased flow across the mitral valve. This may be preceded by soft third heart sound.

FIGURE 25.1: Continuous murmur of PDA. The murmur marches through the second sound.

Chest X-ray 1. Increased pulmonary blood flow. 2. Ascending aorta and the main pulmonary artery are prominent. 3. Chest X-ray will be normal if the shunt is small. If the shunt is large, then there is cardiomegaly with left ventricular configuration. 188 Manual of Cardiology

FIGURE 25.2: Chest X-ray PA view: This patient had a 8 mm large PDA. Note the prominent PA and increased pulmonary blood flow. The heart size is also increased

FIGURE 25.3: This patient has undergone coil closure of the PDA of 2 mm in size. The arrow shows the position of coil. The heart size is not enlarged because the PDA was small Patent Ductus Arteriosus 189

ECG 1. Left ventricular hypertrophy is a feature of large shunt. 2. Biventricular hypertrophy is seen if there is associated pulmonary artery hypertension.

Echo 1. Color Doppler confirms the diagnosis of PDA with left to right shunt. 2. Doppler studies also give an indirect idea regarding the pulmonary hypertension.

DIFFERENTIAL DIAGNOSIS 1. Rupture of sinus of valsalva aneurysm (RSOV)—sudden onset of chest pain and signs of heart failure are typical. The murmur is usually very loud and may be best heard slightly lower in this sternal than in the 2nd left intercostal space. 2. Coronary arteriovenous fistula (CAF)—this continuous murmur is usually best heard either right of sternum or left of sternum and is soft in quality. 3. Venous hum (VH)—this occurs in children and is heard maximally in the right or left infra clavicular and supra clavicular areas. Best heard when the patient is sitting. It can be obliterated by pressure in the neck veins. 4. Pulmonary arteriovenous fistula (PAF)—a continuous murmur can be heard in this situation and is usually heard in the peripheral lung fields.

MANAGEMENT Medical 1. Indomethacin is useful in premature infants. 2. Infective endocarditis prophylaxis is required for all patients. 3. Digoxin and diuretics are given for heart failure. 190 Manual of Cardiology

Nonsurgical Closure This procedure is done between 6 months and 2 years or at any time thereafter. If the PDA is 3 mm or less, it can be closed using coils. If the PDA is 4 mm or more, it is better to use devices. The commonly used device is called as Amplatzer’s device.

Surgical Closure Using lateral thoracotomy, the PDA is divided and ligated. This is called as ligation and division. This can also be done using a very small incision with video assisted thoracoscopy.

Indications for Closure All PDA’s should be closed because there is always a risk of infective endocarditis.

NATURAL HISTORY 1. Asymptomatic throughout life. 2. Infective endocarditis. 3. Pulmonary arterial hypertension. 4. Congestive heart failure. Tetralogy of Fallot 191

TETRALOGY OF FALLOT 26

PREVALENCE Tatralogy of fallot occurs in 10 percent of all congenital heart defects. This is the most common cyanotic heart disorder seen. The frequency in the siblings is 3 percent.

PATHOLOGY As the name itself suggests, Tetralogy of fallot consist of the following four abnormalities: 1. Ventricular septal defect: This is usually situated in the perimembranous area and the VSD is large so that it equalizes the pressures in both ventricles. 2. Pulmonary stenosis: The level of obstruction is usually is in the infundibulum (45%). Infundibulum is the area that is just below the pulmonary valve, in the right ventricle. At the valvar level in 10 percent. The combination of infundibulum and valvar in 30 percent. In around 10 to 15 percent, the valve is atretic. The later form is called as VSD with pulmonary atresia. 3. Over riding of aorta: In this, the aorta is arising from both the ventricles. 4. Right ventricular hypertrophy: This is secondary to right ventricle outflow tract obstruction.

EMBRYOLOGY All the above four components of TOF is the result of one morphogenetic abnormality—malalignment of the 192 Manual of Cardiology infundibular septum. Because the infundibular septum is deviated, it encroaches on to the right ventricular outflow tract causing pulmonary stenosis, over riding of aorta and VSD.

HISTORY This malformation was reported by Niles Stensen in the year 1671. However, Fallot described the disease in detail in the year 1888 and published the same.

Associated Genetic Disorders TOF is associated with a number of genetic disorders and they are listed below: 1. CATCH 22. 2. Down’s syndrome. 3. Velocardiofacial syndrome. 4. CHARGE. 5. Goldenhar’s syndrome.

Associated Cardiac Defects 1. Right aortic arch-25 percent. 2. ASD. 3. Multiple VSD. 4. Coronary anomalies-5 percent.

Hemodynamics 1. Since the VSD is large, pressures in both the ventricles are the same. 2. The pulmonary stenosis results in elevation of RV pressure with a low pressure in the pulmonary artery. This is responsible for the ejection murmur. 3. Right to left shunt occurs at the VSD level and with overriding of aorta. The blood for RV shunts easily into the aorta, because it is easier for the blood to go to aorta Tetralogy of Fallot 193

through the VSD, than through the pulmonary stenosis, which acts as resistance zone. 4. Right ventricular hypertrophy is present because of pulmonary stenosis. 5. The severe the TOF is, more and more blood gets diverted to aorta through the VSD and less blood is flowing across the RVOT and the pulmonary artery. The result is that the ejection murmur shortens and softens.

CLINICAL FEATURES Cyanosis1 About 25 percent are cyanotic at birth and by the age of 1 year 75 percent are cyanotic. The late appearance of cyanosis is related to progressive increase in oxygen demand of the growing child and increasing pulmonary stenosis.

Squatting 1. With squatting systemic vascular resistance increases and diverts the RV blood into the pulmonary artery so that pulmonary blood flow increases. 2. Systemic venous return is even more important. Squatting decreases the venous return from leg, which has the lowest oxygen saturation.

Postures assumed other than squatting: 1. Sitting with legs drawn downwards. 2. Legs crossed while standing. 3. Mother holding infant with legs flexed upon. 4. Lying down. 194 Manual of Cardiology

Spell/Hypoxic Spell In this, the following features are seen with increasing severity: 1. Increase in the rate and depth of respiration. 2. Increasing cyanosis. 3. Hypotonia. 4. Syncope. 5. Seizures.

Mechanism of Spell 1. Vulnerable respiratory center. 2. Infundibular spasm. 3. Atrial tachyarrhythmia.

Age at which spells occur Six months to two years and in the early morning.

Precipitating factor for spell 2 1. Anemia. 2. Increase in the activity of child resulting in an increase in oxygen demand, especially on waking up or following exertion.

Complications of Spell 1. Hypoxic brain damage/mental retardation 2. Nasal speech

Clubbing This is usually seen by 6 months of age. PDGF released by the megakaryocytes that impact on the capillaries are responsible. If pain is a problem, then can give salsalate, which is a non- acetylated analog of aspirin that does not interfere with platelet function. Jugular Venous Pulse: JVP is normal. Tetralogy of Fallot 195

Precordial Palpation 1. A gentle RV impulse is felt in the 4th and 5th left intercostal spaces, close to the sternum. 2. LV impulse is absent and the apex is formed by RV. 3. Right sternoclavicular junction may show an impulse in patients with right aortic arch. 4. Thrill may be palpable in the following situations: a. If the RVOT obstruction is mild. b. If valvar and infundibular PS is present. c. If restrictive VSD is present.

Auscultation Ejection Systolic Murmur 1. The murmur originates at the zone of right ventricular outflow stenosis, not across the ventricular septal defect. 2. The murmur is equally prominent in the 2nd and 3rd left inter spaces, but is typically maximum in the third inter space because the stenosis subvalvar. 3. The length and loudness of murmur varies inversely with the severity of TOF. In severe TOF, the murmur is only faintly heard or there is no murmur at all. 4. Thrill may be present in 30 percent of cases.

Second Heart Sound is Single and Loud. Aortic Component is the one that is Heard P2 is soft or absent. P2 is soft because of decreased pulmonary blood flow. It is delayed or absent because of the delay in the closure of the pulmonary valve due to pulmonary stenosis and delayed relaxation of the infundibulum. 196 Manual of Cardiology

Summary of Clinical Features 1. Cyanosis. 2. Clubbing. 3. Squatting. 4. Spells. 5. Quiet precordium. 6. Ejection systolic murmur. 7. Single second heart sound.

Features of adult TOF 3 1. Congestive heart failure—15 percent. 2. Hypertension—6.7 percent. 3. Aortic regurgitation—6.7 percent. 4. ECG may show LVH—0.7 percent. 5. Absence of RVH—3.4 percent. 6. q in lateral leads—8.8 percent.

ECG ECG shows right ventricular hypertrophy. 1. Dominant R in V1. 2. Transition zone is in V2. 3. P waves are typically peaked, but are seldom increased in amplitude. The duration of the P wave is normal or short, because an under filled and therefore relatively small left atrium writes the terminal portion of the P wave. P is normal in height only in two thirds of cases. In 20 percent of patients the P wave height may be 3 mm or more and this is especially seen in older children. 4. QRS axis is rightward; the mean axis is between +90 and +150. Figure 26.1 shows ECG axis in TOF in relation to two other conditions. Tetralogy of Fallot 197

FIGURE 26.1: ECG axis

CHEST X-ray Chest X-ray is unique in TOF—boot shaped heart (Figure 26.2). 1. PBF normal or reduced; the middle and center 2/3rd show no vascular markings. The main pulmonary segment is concave. 2. The left heart border is straight on top, rounder underneath, with an elevated blunt shoulder. This is responsible for the boot shaped heart. This configuration results from the combination of a concave PA segment and a horizontal ventricular septum concave by RVH, in the presence of LV that is smaller then normal.

FIGURE 26.2: The left heart border shows in the middle, a convexity, which gives a configuration of a boot. In addition, the lung vascular markings are only faintly seen suggestive of decreased pulmonary blood flow 198 Manual of Cardiology

FIGURE 26.3: This is a long axis view of a 2D Echo. This shows that there is a large VSD – arrow. In addition, note that the aorta is arising partially from the left ventricle and partially from the right ventricle – overriding of the aorta

Echo This is the noninvasive test of choice (Figure 26.3 and Figure 26.4, Plate 2) echo can identify the VSD, override of aorta and me severity of RVOT obstruction.

ANGIOGRAM This imaging method is essential in localizing the right ventricular outflow tract obstruction and pulmonary artery anatomy. Most patients undergo the same prior to surgery.

Complications of TOF 1 1. Brain abscess. 2. CVA. 3. Depressed IQ. 4. Scoliosis. 5. Gout. 6. Gallstones. 7. Infective endocarditis.

Differential Diagnosis The diagnosis of TOF is clinically made, when there is cyanosis with normal sized heart and ejection murmur in left sternal Tetralogy of Fallot 199 border. Similar findings clinically can occur in the following conditions: 1. Double Outlet Right Ventricle (DORV) with associated pulmonary stenosis. 2. Single ventricle with pulmonary stenosis. 3. Transposition great arteries with pulmonary stenosis. 4. Corrected Transposition of Great Arteries (CTGA) and pulmonary stenosis. The clinical features of this condition usually differ from TOF, because of prominent aortic impulse felt in the 2nd and 3rd left intercostal space.

TREATMENT OF A SPELL 1. Knee chest position. 2. Oxygen is given in a tent or by mask. 3. Morphine is the principle pharmacological agent used; the dose is 0.1 mg/kg. 4. Soda bicarbonate: 1 mEq/kg IV. The same dose can be repeated in 10 to 15 minutes. Soda bicarbonate reduces the respiratory center stimulating effect of acidosis. If the spells do not respond to the above 4 measures, the following medications can be tried. 5. Beta blocker—Propranolol, 0.01 to 0.25 mg/kg given intravenously; the average dose is usually 0.05 mg/kg. Half of the dose should be given rapidly, remainder slowly over the next few minutes. 6. Ketamine: 1 to 3 mg/kg (average 2 mg/kg) given IV over 60 seconds works very well. It increases the systemic vascular resistance and sedates the infant. 7. Phenyl ephrine (Neo—synephrine) 0.02 mg/kg given intravenously is quite effective. 8. Emergency shunt. 200 Manual of Cardiology

NATURAL HISTORY Survival of Un-operated TOF 1. 50 percent at 3 years. 2. 25 percent at 10 years. 3. 12 percent at 20 years. 4. 6 percent at 30 years. 5. 3 percent at 40 years.

Causes of Death 1. Spells. 2. CVA. 3. Infective endocarditis. 4. Cardiac failure. 5. Pulmonary hemorrhage.

SURGERY 1. Intracardiac repair: In this, VSD is closed and the right ventricular outflow tract is enlarged by cutting or using a patch. For this surgery we need to have a good sized pulmonary artery. There are several methods to measure the adequacy of pulmonary artery, which are mentioned below. This surgery can be done at any age, if the anatomy is suitable. Usually this surgery is done, when the child is over 6 months of age or so, because then the risk of surgery is low. Over all the expected surgical risk is 5 percent.

Assessment of the Adequacy of PA Size 1. McGoon Ratio: Diameter of the right pulmonary artery and diameter of left pulmonary artery is divided by the size of descending thoracic aorta at the level of diaphragm. Normal McGoon ratio is 2 to 2.5. A ratio of more than 1.2 is adequate for ICR. Tetralogy of Fallot 201

2. Nakata index: This is the pulmonary artery area index (RPA + LPA) assessed by cineangiogram. Patients with an index < 60 percent of normal had severe problems with low cardiac output and congestive cardiac failure or died in the postoperative period. 3. Black stone formula: This is derived from diameter of right and left pulmonary artery and the pulmonary valve annulus measured from preoperative cine angiogram normalized to the patients descending thoracic aorta. This ratio should be > 1.5:1.

Survival after Intracardiac Repair4 In one series the rate of survival at 32 years after surgery was 86 percent among patients with repaired TOF and 96 percent in an age matched control population. Ventricular arrhythmias may be detected in Holter in 40 to 50 percent with postoperative TOF. This is most likely to occur in patients who are older at the time of surgery and those with moderate or severe pulmonary regurgitation.

Chronic Complications following Intracardiac Repair 1. Pulmonary regurgitation—60 to 90 percent. 2. RVOT aneurysm. 3. Residual pulmonary stenosis. 4. Residual VSD. 5. Aortic regurgitation, which is usually mild. 6. Arrhythmias. Shunt surgery: If the pulmonary artery is small and the child is sick, it is possible to do a shunt surgery, in which a systemic artery to pulmonary artery connection is made. On a later date, if possible, Intracardiac repair is undertaken.

Types of Shunts 1. Classical Blalock-Taussig Shunt (1945) (BT shunt): It is done on the side opposite the aortic arch; subclavian 202 Manual of Cardiology

artery is dissected down and anastomosed to the side of the PA. Blalock was a vascular surgeon of John Hopkins Hospital in USA who implemented the idea of a Pediatrician namely Taussig. 2. Modified BT: It is side-to-side anastomosis of subclavian artery to PA using Gortex conduit; can be done in small infants also. This is the one that is preferred. 3. Potts shunt (1946): Descending aorta to LPA. Long-term follow-up showed a significant incidence of pulmonary vascular disease. 4. Waterston Shunt (1962): Ascending aorta to RPA; it may be the shunt of choice in small infants. 5. Glenn: SVC to RPA.

REFERENCES 1. Fyler DC. Tetralogy of Fallot. Nadas’ Pediatric cardiology. 1992; Chapter 30;471-92. 2. Pediatric Cardiac Society of India April 2000. 3. Abraham KA, et al. Am J of Med 1979;66:811-6. 4. Bricker MV, Hillis LD and Lange RA. Medical Progress: Congenital Heart Disease in adults. NEJM Feb 2000;342:335-6. Coarctation of Aorta 203

COARCTATION OF 27 AORTA

In coarctation of aorta there is narrowing of aorta just beyond the left subclavian artery at the insertion of ductus arteriosus. As a result of this narrowing collateral circulation develops across the coarctation. These collateral arteries are able to support the distal aortic flow.

CLINICAL FEATURES 1. Headache. 2. Claudication of the legs. 3. Radio femoral delay. 4. Blood pressure in the upper limb more than a lower limb. 5. Ejection systolic murmur along the spine posteriorly at the level of thoracic vertebrae 4. 6. In addition there can be an ejection systolic murmur across the aortic valve secondary to bicuspid aortic valve. 7. Collateral arteries around the scapula may give raise to pulsations especially, with the patient bending forward— Suzman’s sign.

INVESTIGATIONS 1. Chest X-ray—shows a dilated aorta and sometimes an indentation may be made out at the descending aorta—3 sign. In addition dilated collateral arteries may erode the under surfaces of the ribs—rib notching (Figure 27.1). 204 Manual of Cardiology

Figure 27.1: The above chest X-ray shows rib notching on the under surface of the ribs, marked by arrow. Rib notching is an important radiological sign of coarctation. In page 203 the figure along with legend is being given once again

2. CT/MRI—both these tests can accurately demonstrate the coarct segment. 3. Angiography—this is a confirmative test and it is done just prior to a procedure.

TREATMENT Treatment is indicated if the pressure difference across the coarct segment is more than 20 mmHg. This can be done by balloon dilatation or surgery. Nowadays most patients are treated with balloon dilatation. Section VIII ARRHYTHMIAS

ATRIAL FIBRILLATION 28

Atrial fibrillation (A Fib) is the most common arrhythmia. It can occur in three forms: 1. Paroxysmal Atrial Fib (PAF)—duration < 7 days (mostly 24 hours). 2. Persistent—usually more than 7 days. 3. Chronic A Fib—more than 3 weeks.

PATHOPHYSIOLOGY 1. In atrial fibrillation the atrial rate is in the range of 350 to 450/minute. 2. All the atrial fibrillatory impulses are not conducted down to the ventricles. They get blocked at the AV nodal level. As a result, the ventricular rate is in the range of 100 to 160 per minute. 3. Ventricular rate is irregularly irregular. Some of the atrial impulses get blocked in the AV node and alters the conduc- tion of the AV conduction. This effect of nonconducted atrial impulses influencing subsequent conduction of atrial impulses is called as concealed conduction.

ETIOLOGY 1. Rheumatic heart disease. 2. Hypertension. 3. Chronic lung disease. 4. Atrial septal defect. 208 Manual of Cardiology

5. Thyroxicosis. 6. Pericarditis. 7. Lone atrial fibrillation: This term is used when there is no underlying cause is made out for atrial fibrillation. 8. Following emotional stress or surgery. 9. Acute alcoholic intoxication (holiday heart syndrome). 10. Hypoxia, secondary to any cause possibly with pneumonia.

SYMPTOMS 1. Palpitations, secondary to increased heart rate. 2. Fatigue, due to fall in cardiac output, because the atrial contribution to ventricular filling is lost. 3. Embolization to brain, limbs, etc; due to stasis of blood in the atria, since there is no effective atrial contraction. 4. Angina can be precipitated in patients with coronary artery disease. 5. Dyspnea secondary to pulmonary congestion in patients with LV dysfunction or valve stenosis.

SIGNS 1. Pulse is irregularly irregular. 2. Pulse deficit: heart rate counted simultaneously is more than the pulse rate, because some of the ventricular contractions are sufficiently strong enough to open he aortic valve. 3. Loss of a wave in Jugular venous pressure. 4. First heart sound varies in intensity.

ECG 1. No P waves are seen. 2. The base line shows undulating pattern or sometimes atrial deflections above 350 per minute. Atrial Fibrillation 209

FIGURE 28.1: The R-R intervals are irregular and the baseline in the middle strip shows undulating wave pattern suggestive of atrial fibrillation (see arrow)

Chest X-ray This test is done routinely to assess the lung parenchyma and vasculature.

Echo This test is mandatory in all patients with atrial fibrillation to identify valvular heart disease, left and right atrial size, LV size and function and left atrial thrombus. Transthoracic Echo has low sensitivity for left atrial thrombus. TEE is superior to identify LA thrombus.

Thyroid Function Test This is done especially: 1. After the first episode of atrial fibrillation. 2. Rate is difficult to control. 3. When A fib recurs unexpectedly after DC cardioversion. 4. Routinely in all patients with non-valvar atrial fibrillation.

Trans Esophageal Echo (TEE) This is done to identify LA thrombus prior to cardioversion. 210 Manual of Cardiology

TREATMENT 1. Rate control. 2. Rhythm control. 3. Anticoagulation. 4. Ablative therapy.

Rate Control Drugs are used to control the ventricular rate, between 60 to 90 at rest. These drugs are: 1. Digoxin. 2. Beta blockers: Metoprolol, atenolol. 3. Calcium channel blockers such as verapamil or diltiazem. The following Table 28.1 gives the orally given drugs for rate control

TABLE 28.1: Drug Loading dose Onset Maintenance dose Digoxin 0.25 mg 2 hours 0.125 to 0.25 mg/daily Diltiazem - 2-4 hours 30 mg thrice daily to 360 mg max/daily Metoprolol - 4-6 hours 25 to 100 mg twice daily Verapamil - 1-2 hour 120 to 360 mg daily in divided doses Amiodarone 800 mg for 1 week 1-3 weeks 100 to 200 mg/daily 600 mg for 1 week 400 mg for 4 weeks

Rhythm Control This is done to convert atrial fibrillation to sinus rhythm. It is useful if atrial fibrillation has been present for less than 3 months and there is no .

Pharmacological Conversion Amiodarone: 150 mg IV over 20 minutes (5 mg/kg) then 1.2 gm over 24 hours. Atrial Fibrillation 211

Electrical-DC Cardioversion DC electrical shock is an effective form of treatment to restore sinus rhythm quickly. In this electrical energy is delivered to the heart via chest and we expect the heart to recover out of the A fib and sinus rhythm to be established. Usually about 200 joules of energy is delivered 1. If atrial fibrillation has been present for less then 48 hours DC cardioversion can be undertaken. 2. If atrial fibrillation has been present for more than 48 hours, the cardioversion is done, after anticoagulation for 3 days. 3. If atrial fibrillation has been present for more than 48 hours, we can do a TEE and if no thrombus present, then a DC cardioversion can be done. 4. After DC cardioversion anticoagulation should be continued for four weeks at least. 5. DC cardioversion is successful in 75 percent of cases but relapse occurs in 20 to 50 percent at one month and 70 to 90 percent at year.

Anticoagulation All patients with A Fib are prone to develop embolism. Hence they are given anticoagulation using Warfarin. Guidelines have been drawn looking at the risk of embolism versus risk of hemorrhage secondary to warfarin excess dose.

Warfarin Dose The INR is kept between 2 and 3. In patients above the age of 75 years, it is preferable to maintain around 1.6 to 2. The following factors have been identified as risk factors for embolism in AFib: 1. Past history of stroke/transient ischemic attack. 2. Mitral valve disease. 3. Age > 65 years. 212 Manual of Cardiology

4. Hypertension. 5. Diabetes mellitus. 6. Heart failure. 7. Echo—Ejection fraction is low or mitral annular calcification.

TABLE 28.2: Risk of stroke according to risk factors Risk Category Risk Factors Risk of Embolism/year Low Risk Age < 65 1.2% Moderate Risk Age < 65 with other risk factor 4% High Risk Age > 65 6.5% Very High Risk Past H/o stroke/TIA 12%

Ablative Treatment 1. This form of therapy is now employed for Paroxysmal atrial fibrillation. Pulmonary vein isolation is one of this procedure. Selectively pulmonary veins are entered, and using special catheters the ostial area of these veins are electrically burned. 2. The MAZE procedure is a surgical approach in which multiple scars are produced in the atria so that fibrillatory waves are disconnected. 3. AV nodal ablation is the last procedure to be attempted, in which using radio frequency the AV node is disconnected from the conduction system. The result is that the patient will have complete AV block. This is used as a last resort in patients in whom the control of rate is a problem.

ATRIAL FIBRILLATION IN WOLFF PARKINSON-WHITE SYNDROME Patients with WPW have accessory conduction pathway in which electrical pulses are conducted from the atria to the ventricle bypassing the AV node. As a result, if a person with WPW syndrome develops atrial fibrillation it is possible that all the atrial impulses can be conducted antegrade through this accessory pathway. The net result will be a very fast ventricular Atrial Fibrillation 213 rate which is irregular and it is likely that the patient will develop hypotension. • The treatment of choice is to DC shock to convert the rhythm. • These patients should not be treated with digoxin or beta blockers. • Procainamide is the agent of choice for drug therapy. 214 Manual of Cardiology

ATRIAL FLUTTER 29

1. In Atrial Flutter (AFL) the atrial rate is between 250 to 350/ min. 2. Usually these flutter waves get blocked in the AV node, resulting in 2:1/4:1 rates.

ECG The baseline shows flutter waves which appear as saw tooth (Figure 29.1).

FIGURE 29.1: Saw tooth appearance of the baseline suggestive of atrial flutter

TREATMENT 1. If hemodynamically unstable—DC cardioversion. The energy required is around 50 joules. Atrial Flutter 215

2. If present for more than 48 hours, treat like atrial fibrillation. 3. Ibutilide is a new drug that is effective for conversion of atrial flutter to sinus rhythm. 4. To control the heart rate use either a beta blocker or calcium channel blocker. 5. Radio frequency ablation is the treatment of choice for recurrent symptomatic atrial flutter. 216 Manual of Cardiology

NARROW QRS 30 TACHYCARDIA

In this form of tachycardia, ECG shows a rate of >100 bpm and QRS complex duration is <120 ms or less (< 3 small squares on ECGs done at the standard speed of 25 mm/second).

DIFFERENTIAL DIAGNOSIS • Sinus tachycardia: Normal P wave followed by normal QRS. • AV nodal re-entry tachycardia (AVNRT). • AV re-entry tachycardia (AVRT) secondary to accessory pathway, e.g. WPW. The commonest cause is usually AV nodal re-entrant tachycardia.

FIGURE 30.1: Figure shows a narrow QRS tachy with a heart rate of 180/minute. The P waves are not seen and they are submerged within the QRS Narrow QRS Tachycardia 217

These have a sudden onset and sudden offset. Physical examination during an episode will show prominent jugular venous pulsations – “frog sign”.

TREATMENT – IMMEDIATE (TABLE 30.1) 1. Reflex Vagal maneuvers: a. Carotid sinus massage: The carotid sinus is gently stimulated by rotatory pressure against the transverse process of C-3 vertebra. Check for before doing this. Carotid sinus massage is used in clinical practice for the following reasons: i. Breaks the tachycardia involving the AV nodal junction ii. It may reveal P waves in atrial arrhythmias iii. In patients with carotid sinus hypersensitivity there is fall in blood pressure and heart rate. b. Valsalva maneuver: Forced expiration against glottis, causes transient AV block and about 50 percent of patients respond to this. c. Induction of vomiting by placing a finger in tongue. d. Drink a glass of ice cold water. e. Face is immersed into a basin of cold water – Diving reflex.

TABLE 30.1: Drugs used for PSVT Drug / Dose Side effects Contra indications Adenosine 6 mg Flushing, chest pain Asthma rapid bolus Postheart transplant Verapamil 5 to 10 mg Hypotension, Heart block Diltiazem 0.25 mg/kg Hypotension, Heart block Metoprolol 5 mg, Hypotension, Heart block Bronchial asthma repeat after 5 min

2. If unsuccessful, give adenosine 6 mg with saline flush, if unsuccessful give 12 mg after 2 minute interval. Adenosine can precipitate broncho spasm in patients with asthma. 218 Manual of Cardiology

3. If there is a contraindication to give adenosine such as asthma, verapamil (Isoptin 5 mg IV slowly can be given). 4. If hemodynamically unstable, such as blood pressure < 90 mmHg, or chest pain or heart failure or heart rate > 200 give synchronized DC cardioversion with 100 joules.

FIGURE 30.2: Treatment algorithm for Immediate management of PSVT

TREATMENT – LONG TERM 1. AV nodal blocking drugs such as verapamil or beta blockers are given on a long-term basis. 2. If these drugs fail, Class Ic or Class 3 antiarrhythmic drugs (propafenone, sotalol or amiodarone) can be used. These drugs can prevent the recurrence in 80 percent of patients. Despite their efficacy, long-term therapy with these drugs are not recommended, because of side effects. 3. Pill in the pocket1 is a concept that can be tried in the following situations: 1. Infrequent episodes that are well tolerated (< 5 episodes in one year). Narrow QRS Tachycardia 219

2. Single episode of SVT: In this method, the patient takes the tablet as soon as he gets an attack. The drugs that can be used are: 1. Verapamil. 2. Beta blockers. 3. Flecainide. 4. Propafenone. 4. Radio Frequency Catheter ablation is curative in most patients. The Table below gives the indications for referral to an electrophysiologist, for further treatment. TABLE 30.2 Referral to EP for SVT2

1. Syncope or severe symptoms 2. Drug resistant 3. Drug intolerance 4. Does not like to be on long-term drugs 5. Pre-excitation syndrome (with or without SVT)

FIGURE 30.3: Treatment algorithm of PSVT

REFERENCES 1. Alboni P, Tomasi C, Menozzi C, et al. Efficacy and Safety of out of hospital self administered single dose oral drug treatment in the management of infrequent well tolerated paroxysmal supraventricular tachycardia. JACC 2001;37:548-53. 2. Etienne Delacretaz. Supraventricular Tachycardia. NEJM 2006;354:1039-51. 220 Manual of Cardiology

VENTRICULAR 31 TACHYCARDIA

QRS is wide with duration of 120 ms or more at a rate of 100 or more. The term sustained is used if VT persists for 30 seconds or more. The term monomorphic is used if all the complexes are of the same type. If the QRS complex is varying in size and shape then it is called as polymorphic ventricular tachycardia.

ETIOLOGY 1. The commonest cause for VT is ischemic heart disease. 2. Can also occur with dilated cardiomyopathy. 3. Metabolic disturbances. 4. Drug toxicity such as digitalis. 5. Long QT syndrome.

DIFFERENTIAL DIAGNOSIS It is important to differentiate between a ventricular tachycardia from a supraventricular tachycardia that manifests itself with a widened QRS.

TREATMENT a. If hemodynamically stable: 1. Lignocaine 50 mg IV over 2 minutes, repeat after 5 minutes; start infusion 2 mg per minute after first bolus dose. Ventricular Tachycardia 221

STEP 3 Look for the presence of AV dissociation or a fusion complex

STEP 4 Look for morphology as shown in the diagram below: 222 Manual of Cardiology

2. If this fails, procainamide 100 mg IV over 2 minutes, repeated at 5 minutes up to a maximum of 1gm. 3. Instead of procainamide we can also give amiodarone. 300 mg over 5 to 15 minutes preferably by central line and then 600 mg over 1 hour. b. If hemodynamically unstable: 1. Give DC shock. c. After correction of VT: 1. Maintenance antiarrhythmic therapy, in the form of IV lignocaine for 24 hours. Amiodarone oral can also be given. 2. AICD (Automatic Internal Cardioverter and Defibrillator): In this a device is placed within the patients’ chest with connections to heart so that defibrillation is done within the heart as required. 3. Radio frequency ablation is done in selected cases if the patient is symptomatic despite medical therapy and the site of origin can be identified by electrical mapping. Heart Block 223

HEART BLOCK 32

Atrioventricular conduction block can be classified as given below: 1. First degree. 2. Second degree • Mobitz type 1 • Mobitz type 2. 3. Complete AV block.

FIRST DEGREE HEART BLOCK This is manifest in ECG as prolonged PR interval. The interval is more than 0.20 seconds.

SECOND DEGREE HEART BLOCK This can be of two types: Mobitz type 1 block: It is characterized by progressive lengthening of PR interval and this is followed by a dropped beat. The block is usually in the AV node. Mobitz type 2 block: In this there is block without any change in the preceding PR interval. The level of block is in the His- Purkinje system.

THIRD DEGREE COMPLETE AV BLOCK In complete AV block, no P waves are conducted down to the ventricles. Hence the rhythm is dependent on the escape 224 Manual of Cardiology

TABLE 32.1: Etiology

Congenital Corrected TGA, Maternal SLE Idiopathic Lenegre’s disease Lev’s disease Ischemic Acute myocardial infarction Infections Lyme disease Chagas’ disease Infective endocarditis Following surgery Post VSD surgery rhythm, from the bundle or ventricle. The ventricular rate is around 40 to 50 per minute. ECG is diagnostic of complete AV block (Figure 32.1).

FIGURE 32.1: The above ECG shows that P waves (see arrows) are not followed by QRS complexes. P waves are independent of the QRS complexes, suggesting complete AV block.

TREATMENT Drugs: Atropine 0.5 to 2 mg IV or isoprenaline 1 mcg /min IV are useful to increase the heart rate in an emergency.

Pacemakers Temporary: This is done temporarily to tide over the crisis. A pacemaker wire is passed into the right ventricle from the patient’s groin or through the internal jugular vein. An energy Heart Block 225 giving device called as generator is placed externally connected to this wire. Permanent Pacemaker: This offers a long term solution to bradycardia. The pacemaker is placed in the patient’s body itself. The wire is placed in the atria or ventricle or both. The commonly used ones are in which the wire is placed in the right ventricle. Shown below in Figure 32.2, is a chest x-ray of a patient who has had a permanent pacemaker. The system will work only if the patient’s heart rate falls below the set limit. For example, if the set rate is 60/min, then it will pace the heart only when the patient’s heart rate falls below 60/min. The following codes are used in pacing: VVI: Ventricle is paced; Ventricle is sensed, and Inhibited by the patient’s rhythm. DDD: Both atria and ventricle is paced and sensed. D stands dual; the last symbol D stands for response to sensing and in this, it is both triggered and inhibited.

FIGURE 32.2: The above chest x-ray shows a pacemaker (small arrow head) that is placed in the right pectoral region and the wire leading from the same to the right ventricle (bigger arrow) 226 Manual of Cardiology

CARDIAC ARREST AND 33 SUDDEN DEATH

CAUSES OF SUDDEN ARRHYTHMIC CARDIAC DEATH 1. Coronary artery disease 85 percent, such as myocardial infarction. 2. Structural heart disease—10 percent, such as aortic stenosis, hypertrophic cardiomyopathy. 3. No structural heart disease—10 percent, such as long QT syndrome, Brugada, Wolff-Parkinson-White syndrome.

REASONS FOR CARDIAC ARREST 1. Ventricular fibrillation or pulseless ventricular tachycardia. 2. Asystole. 3. Electromechanical Dissociation (EMD).

MANAGEMENT OF CARDIAC ARREST After confirming the diagnosis of cardiac arrest—patient unconsciousness, not breathing, absent carotid pulse, must proceed onto the following two steps: 1. Basic life support. 2. Advanced life support.

Basic Life Support Consists of the following components: 1. Airway—lift the chin up, clear the mouth. 2. Breathing—give breathing using the Ambu bag or mouth to mouth breathing; start with two breaths—each inflation two seconds long. Cardiac Arrest and Sudden Death 227

Ventricular Fibrillation and Pulseless Ventricular Tachycardia

FIGURE 33.1: Algorithm for cardiac arrest management 3. Chest compression—for 1 operator do 15 chest compres- sions followed by two breaths (15:2 ratio). If 2 operators do 5 chest compressions and one breath (5:1 ratio). This should not be interrupted except to give shocks or intubations.

Advanced Life Support The main feature of advanced life support is to identify the rhythm and give defibrillator if necessary. Connect the 228 Manual of Cardiology defibrillator paddles to the patient and assess the rhythm. Based on the rhythm seen on the monitor follow the algorithm given below in Figure 33.1. It is important to know when to stop the CPR and also to know when not to resuscitate. In most situations the following general rule may be followed: 1. If after 20 minutes of CPR, patient’s continues to be in asystole, then can be stopped. 2. If the patient’s condition is due to a terminal cancer or the patient does not wish to have CPR as declared before the cardiac arrest event, then also CPR need not be done. Hypertension 229

Section IX HYPERTENSION 230 Manual of Cardiology

BLOOD PRESSURE The arterial blood pressure is a measure of the lateral force per unit area of vascular wall. It is quantified as mm of Hg or dynes/cm.

White Coat Hypertension Blood pressure more than 140/90 mmHg when measured in doctor’s clinic but less than 135/85 mmHg during day time ambulatory recording. The usual prevalence is 15 to 30 percent of the population. This is more common in the elderly.

Pseudohypertension With increasing age, the vessel wall gets thickened and the blood pressure cuff does not obliterate the arterial wall properly and there can be higher blood pressure recordings. This is called as .

Orthostatic Hypotension Blood pressure is measured in supine position, immediately after standing and within 3 minutes after standing. A 20 mm drop in systolic BP or 10 mm drop in diastolic BP is defined as orthostatic hypotension.

Isolated Systolic Hypertension In this condition systolic BP is more than 160 mmHg and diastolic pressure is less than 90 mmHg.

Malignant Hypertension This refers to severe hypertension (systolic > 200 and diastolic > 130 mmHg) along with retinal hemorrhages, exudates or papilloedema. Symptoms such as headache and visual disturbances are present. Hypertension 231

Accelerated Hypertension Recent increase in BP levels with exudates and hemorrhages in retinal exam.

METHODS OF MEASURING ARTERIAL BLOOD PRESSURE A. Direct Method In this a catheter is passed into the artery and the pressure is measured directly.

B. Indirect Method Invention of the pneumatic cuff manometer (Riva, Rocci- 1896) and Korotkoff sounds (1905) permitted indirect measurement of arterial blood pressure.

KOROTKOFF SOUNDS (KS) This represents oscillations of the partially occluded arterial wall as a result of distension with each cardiac impulse.

Five Phases 1. Phase I—clear tapping sounds occur when cuff pressure has fallen to arterial peak systolic level. 2. Phase II—murmurs of swishing sounds occurs at least 10 to 15 mmHg below peak systolic pressure. Auscultatory gap is the period of abnormal silence or diminished intensity of Phase II KS. The pulse is palpable during this time. This is seen in 5 percent of people. The Korotkoff sounds re-appear at a pressure 10 to 20 mmHg lower than the systolic blood pressure. 3. Phase III—loud sounds. 4. Phase IV—muffling of sounds. 5. Phase V—complete cessation of sounds. 232 Manual of Cardiology

AHA RECOMMENDATIONS Systolic BP is recorded at the point where tapping sounds are heard for two consecutive beats and diastolic BP is at the point where Korotkoff sounds is inaudible. In patients with aortic regurgitation, Phase IV is to be taken as the diastolic blood pressure.

TABLE S-IX.1: AHA Recommendations for BP cuff dimensions Mid-arm Bladder width Bladder length circumference (cm) (cm) (cm) Newborn 5-7.5 3 5 Infant 7.5-13 5 8 Child 13-20 8 13 Small adult 17-26 11 17 Adult 24-32 13 24 Large adult 34-42 17 32 Thigh 42-50 20 42

It is recommended that the width of the bladder be 40 percent at arm circumference, and the length of the bladder be long enough to encircle at least 80 percent of the arm in adults. In children the occluding bladder should be long enough to encircle the arm completely (100%). Overlapping the ends of the bladder in children doesn’t appear to introduce an error in measurement. The British hypertension society has recommended the use of one large cuff, width 12.5 to 13 cm and length 35 cm, for all adults with an arm circumference exceeding 33 cm in order to avoid the need for multiple cuff sizes.

GUIDELINES FOR MEASURING BP Posture 1. Usually blood pressure is recorded in a resting stage, preferably in a sitting posture. 2. Arm position: The recommended position is patients’ elbow at the level of the heart—usually 4th intercostal space Hypertension 233

at the sternum. If the patients’ arm is higher by 6 cm, the systolic and diastolic pressure will be around 5 mmHg lower. These errors are explained by hydrostatic effect. It is essential that the arm is supported during blood pressure measurement. This is best achieved in practice by having the observer hold the arm at the elbow. 3. Which arm? For the first time, you should measure in both the arms. If the difference is more than 20 mm of Hg for systolic and 10 for diastolic between both the arms then the patient needs further evaluation. It has been our preference to use the right arm.

Circumstances 1. No caffeine for preceding hour. 2. No smoking for preceding 15 minutes. 3. No exogenous adrenergic stimulants, e.g. phenylephrine in nasal decongestants or eye drops for pupillary dilation. 4. A quite, warm setting. 5. Home readings taken under varying circumstances and 24- hour ambulatory recordings may be preferable and more accurate in predicting subsequent cardiovascular disease.

Equipment 1. Cuff size: You must use appropriate cuff size. See the Table S – 1×.1. 2. Manometer: Aneroid gauges should be calibrated every 6 months against a mercury manometer. The manometer should not be more than 3 feet (92 cm) so that the scale can be read easily. The mercury column should be vertical and at eye level. 3. For infants: Use ultrasound equipment, e.g. the Doppler method. 234 Manual of Cardiology

Technique 1. On each occasion, take at least two readings, separated by as much time as is practical. If readings vary by more than 5 mmHg, take additional readings until two are close. 2. For diagnosis, obtain at least 3 sets of readings at least a week apart. 3. Inflate the bladder quickly to a pressure 20 mmHg above the systolic, as recognized by disappearance of the radial pulse and deflate the bladder 3 mmHg every second.

VALSALVA MANEUVER AND BLOOD PRESSURE Force expiration against a closed glottis for 20 seconds. The normal response consists of four phases. 1. Phase I—systolic and diastolic pressures will rise proportionate to rise in intrathoracic pressure. 2. Phase II– systolic and diastolic pressures will fall because of reduced venous return and there is reflex tachycardia. 3. Phase III—immediately after release, there is an abrupt transient decrease in arterial pressure. 4. Phase IV—there is an overshoot of pressures and reflex bradycardia.

FIGURE S-IX.1: Different phases of valsalva maneuver Hypertension 235

HYPERTENSION 34

Joint National Committee (JNC) on prevention, detection, evaluation and treatment of high blood pressure has proposed the following classification for blood pressure in adults aged 18 years or more.

TABLE 34.1: JNC—7 Classification1 Category SBP DBP Normal <120 <80 Pre Hypertension 120-139 80-89 Stage 1 Hypertension 140-159 90-99 Stage 2 Hypertension >160 >100

TYPES OF HYPERTENSION 1. Systolic Hypertension Increased cardiac output such as aortic regurgitation, AV fistula, thyrotoxicosis. 2. Systolic and Diastolic Hypertension a. Primary/Essential/Idiopathic—95 percent of cases. b. Renal—glomerulonephritis, diabetic nephropathy, polycystic kidney, renal artery stenosis. c. Endocrine—acromegaly, hypothyroidism, hyperthy- roidism, hypercalcemia, cushing syndrome. d. Coarctation of aorta. e. Stress—alcohol withdrawal, perioperative. f. Pregnancy induced hypertension—preeclampsia, eclampsia. g. Drugs—oral contraceptives, steroids. 236 Manual of Cardiology

INAPPROPRIATE HYPERTENSION Since 95 percent of patients with hypertension do not have an identifiable cause, it is important for clinicians to look for causes that can lead on to hypertension and which may be treatable. The features of hypertension that may suggest that it could be secondary are given below: 1. Onset of hypertension before the age of 35 or after 55 years of age. 2. Blood pressure of >180/110 mmHg. 3. Abdominal bruit. 4. Unexplained hypokalemia. 5. Varying blood pressures with sweating, tremor.

HYPERTENSIVE TARGET ORGAN DAMAGE (TOD) 1. Heart: Left ventricular hypertrophy. 2. Hypertensive encephalopathy: Headache, altered senso- rium, vomiting, seizures. 3. Retinopathy: Exudates, hemorrhages, papilloedema. 4. Stroke/Intra cranial hemorrhage. 5. Renal: Proteinuria, impaired renal function. 6. Peripheral vascular disease.

INVESTIGATIONS—ROUTINE 1. Blood glucose. 2. Fasting lipid profile. 3. Serum creatinine. 4. Serum electrolytes. 5. Urine analysis for blood, protein. The above five investigations are done for all patients with a diagnosis of hypertension. The following investigations are restricted to patients in whom secondary hypertension is suspected—features of inappropriate hypertension are present. Hypertension 237

1. Twenty four-hour ambulatory blood pressure recording— borderline or white coat hypertension. 2. ECHO for LV function. 3. Renal ultrasound and color Doppler—to assess the renal size and renal artery stenosis. 4. Twenty four-hour VMA or CATECHOLAMINES—to detect pheochromocytoma. 5. Urinary Cortisol/Dexamethasone suppression test—to detect cushing’s syndrome. 6. Plasma renin and aldosterone—primary aldosteronism. 7. Angiogram is done based on the above investigations, especially for renovascular hypertension. Shown below is the angiogram of a patient, 17 year old with right renal artery stenosis.

ANGIOGRAM—RIGHT RENAL ARTERY STENOSIS

FIGURE 34.1: Arrow shows the right renal artery narrowing at its origin.

TREATMENT Drug therapy for hypertension reduces the mortality and morbidity. Diuretics or beta blockers reduce the risk of coronary 238 Manual of Cardiology artery disease by 16 percent, stroke 38 percent and all cause mortality by 13 percent. There are several classes of drugs and they are namely: 1. ACE Inhibitors—captopril, enalapril, lisinopril 2. Beta blockers—atenolol, metoprolol, bisoprolol 3. Calcium channel blockers—nifedipine, amlodiine 4. Angiotensin receptor blockers—losartan, valsartan, irbesartan 5. Diuretics—bendroflumethiazide—2.5 mg 6. Alpha blockers—prazosin, doxazosin 7. Centrally acting drugs—methyldopa, clodinine The first thing to do is to set up a goal for therapy. The current recommendations are as follows:

TABLE 34.2: Target for blood pressure control No diabetes Diabetes Clinic Measurements (JNC 7 2003) <140/90 130/80 24 hour—Mean Daytime measurements (British Hypertension Society Guidelines) <130/80 <130/75

Diuretics or beta blockers remain appropriate for the initial treatment of uncomplicated hypertension. ACE inhibitors and ARB are appropriate initial therapy in patients with diabetes, heart failure and renal disease. We prefer long acting calcium channel blockers because short acting once can cause drop in blood pressure and precipitate coronary ischemia. Alpha blockers are useful in patients with prosthetic hypertrophy.2 The algorithm for the treatment of hypertension is summarized in Figure 34.2.

Life Style Modifications 1. Lose weight, if overweight. 2. Salt restricted diet. 3. Daily exercise. 4. Stop smoking. 5. Limit alcohol intake. Hypertension 239

FIGURE 34.2: Algorithm for treatment of hypertension

TABLE 34.3: Choice of drug based on the patient’s condition Condition Drug of choice Diabetes ACE Inhibitors Heart Failure ACE Inhibitors/Diuretics Prostate problem Alpha Blockers Raynaud’s phenomenon Nifedipine Coronary artery disease Beta Blockers ACE Inhibitors

Hypertensive Crisis

FIGURE 34.3: Hypertensive crisis

This is a medical emergency and blood pressure need to be brought down immediately. This can be divided into two types. 240 Manual of Cardiology

Hypertensive emergency: In this condition, there is evidence of end organ damage. Example: Dissection of aorta, hypertensive encephalopathy. Usually the diastolic blood pressure is in the range of 140 mmHg or more. Hypertensive urgency: Diastolic BP is more than 120 mmHg but no evidence of immediate end organ damage. Example: Hypertensive patient with unstable angina.

TABLE 34.4: Drugs for Hypertensive crisis Drug Dose Onset/Effects Sodium nitro prusside 0.25-10 μg/kg/min IV Immediate, nausea, vomiting Nitroglycerin 5-100 μg/min IV 2 to 5 minutes. Headache, vomiting Enalaprilat 1-5 mg every 6 hour/IV 15 minutes. Labetalol 20-80 mg IV bolus; Vomiting, burning in repeat after 10 minutes throat if required followed by 2 mg/min/IV Frusemide 40 mg in 1 to 2 minutes 5 to 15 minutes. Hypokalemia

Note: Sublingual nifedipine should not be used, because a dramatic fall in BP can cause ischemia of coronaries and precipitate myocardial infarction or sudden death.

REFERENCES 1. JNC 7 Report. Hypertension 2003;42:1206-52. 2. August P. Initial Treatment of Hypertension. N Engl J Med 2003;348:610-7. Section X PULMONARY VASCULAR DISORDERS

PULMONARY ARTERIAL 35 HYPERTENSION

Pulmonary arterial hypertension refers to any condition in which PA systolic pressure consistently exceeds 30 mmHg, or 25 mmHg mean.

ETIOLOGY Five types are identified on the basis of cause: 1. Pulmonary venous hypertension secondary to diseases affecting the left side of the heart, such as left ventricular failure, mitral regurgitation, mitral stenosis. 2. Chronic hypoxia with secondary vasoconstriction of pulmonary vasculature—COPD. 3. Pulmonary artery obstruction—pulmonary embolism. 4. Left to right shunts with increased flow across the pulmonary vascular bed—atrial septal defect, ventricular septal defect (Eisenmenger’s syndrome). 5. Idiopathic or Primary Pulmonary Hypertension (PPH) The following description is in general is for pulmonary hypertension, and specifically for PPH.

HISTORY Dyspnea, chest pain, syncope are the important clinical features. 244 Manual of Cardiology

PHYSICAL SIGNS (TABLE 35.1)

TABLE 35.1 Sign Reason

Accentuated pulmonary component High pulmonary pressure increases of S2 (P2 is audible at the apex. the force of pulmonary valve closure Pulmonary ejection click Sudden interruption of opening of pulmonary valve into high pressure artery Pulmonary ejection systolic murmur Turbulent transvalvular pulmonary flow Left parasternal lift High right ventricular pressure Increased jugular “a” waves High right ventricular filling pressure

Chest X-ray 1. Prominent main pulmonary artery trunk. 2. Prominent hilar pulmonary arteries. 3. Pruning of the peripheral vessels.

ECG 1. Right ventricular hypertrophy. 2. Right atrial enlargement.

Echo This test is very useful in identifying the cause of pulmonary hypertension in most cases and also able to noninvasively measure the PA pressure. The PA pressure is estimated noninvasively from the velocity of the tricuspid regurgitation and from the velocity of flow if there is pulmonary regurgitation.

TREATMENT 1. Treatment of the underlying cause such as closure septal defects, etc should take priority. Pulmonary Arterial Hypertension 245

2. Oral anticoagulation: Oral warfarin is used on a low dose so as to maintain INR between 2 and 3. This drug has shown to improve the survival. 3. Diuretics: These drugs appear to be useful in symptomatic relief of patients with right ventricular failure. 4. Digoxin: Patients with right ventricular failure benefit from small dose of digoxin. 5. Oxygen: Patients with resting desaturation or on exercise benefit with supplemental oxygen. 6. Pulmonary vasodilators as given below: 1. Nifedipine: It has been shown that long term administration of high dose nifedipine helps in patients with severe pulmonary hypertension. However, not all patients respond for the same. In order to identify the responders, hemodynamic studies are performed in the cath lab whether the patient will benefit or not.

FIGURE 35.1: Algorithm for treatment 246 Manual of Cardiology

2. Prostacyclin: This drug can be given intravenously as well as by inhalation. 3. Bosentan: This is a orally active endothelin receptor antagonist. Several studies had shown this drug to be useful in the long-term. 4. Sildenafil: This drug increases the activity of endogenous nitric oxide. 7. Lung transplantation or heart lung transplantation. Pulmonary Embolism 247

PULMONARY EMBOLISM 36

Pulmonary embolism usually arises from a venous thrombosis from the legs or from the pelvic veins. Blood clots pass through the venous system and lodge in the pulmonary circulation resulting in pulmonary embolism. The risk factors for the same can be divided into: 1. Hypercoagulable states. 2. Acquired conditions.

HYPERCOAGULABLE STATES 1. Factor V Leiden mutation. 2. Activated protein C resistance. 3. Protein C gene mutation. 4. Protein S deficiency. 5. Anti-thrombin III deficiency (ATIII). 6. Hyper homocysteinemia. 7. Anti-phospholipid antibodies.

ACQUIRED CONDITIONS 1. Surgery. 2. Recent myocardial infarction or stroke. 3. Long duration air travel. 4. Prolonged bed rest. 5. Obesity.

CLINICAL MANIFESTATIONS Sudden onset of unexplained dyspnea is the most important clinical presentation. Depending upon the size of the pulmonary embolism the vary. They are summarized in the Table 36.1. 248 Manual of Cardiology

TABLE 36.1: Syndrome of pulmonary embolism Syndrome Clinical RV dysfunction Massive Breathlessness, syncope, Yes hypotension Moderate Systolic BP normal Yes Small Systolic BP normal No Pulmonary infarction Pleuritic pain, hemoptysis Not present

INVESTIGATIONS 1. Plasma D Dimer ELISA: This is the screening test and suggests that endogenes fibrinolysis is present. 2. Chest CT: This is the initial imaging test of choice. 3. Lung scanning (Perfusion/Ventilation scan): This is now the second choice test and this is done only for patients with renal failure or pregnancy. 4. Echo: This test is useful to identify RV dysfunction which if present suggests massive pulmonary embolism. 5. ECG: Signs of RV ischemia is noted such as S1 Q3 T3 pattern. 6. Venous ultrasound: This test is used to diagnose deep venous thrombosis which may be a marker for pulmonary embolism.

FIGURE 36.1: Diagnostic algorithm Pulmonary Embolism 249

TREATMENT 1. Thrombolysis: This is reserved for high-risk subsets as given below: a. RV dysfunction by Echo. b. Systolic BP low. c. Positive BNP/pro BNP. 2. Pulmonary embolectomy is indicated for massive pulmonary embolism. 3. Anticoagulation: Heparin or Low Molecular Weight Heparin (LMWH) can be used. This forms the corner stone of treatment for pulmonary embolism. Heparin is given in a dose of 80 units/kg as bolus followed by 18 units/kg/hour. The dose of the same is adjusted by APTT test. Warfarin is started along with Heparin on day one itself. By around the fifth day, the prothrombin test will usually show INR value more than 2. At this point in time we can stop the intravenous heparin. LMWH can be substituted for Heparin and this has been shown to be as effective as regular Heparin. Duration of warfarin anticoagulation Underlying cause Duration Idiopathic Life long Secondary to DVT 1 year Following surgery or trauma 6 months 4. IVC filter indications a. Anticoagulation is contraindicated. b. Recurrent pulmonary embolism on anticoagulation.

Section XI INVESTIGATIONS

CHEST X-RAY 37

Chest X-ray for cardiac purposes is taken in the posterior- anterior projection; in this position, the patient faces the X-ray cassette. The X-ray generator is usually 6 feet away from the X-ray film. The right border of the heart consists of the following from above downwards: 1. Superior vena cava. 2. Ascending aorta. 3. Right atrium. The left border of the heart consists of the following from above downwards: 1. Aortic arch. 2. Pulmonary artery. 3. Left atrial appendage (Not usually seen unless enlarged). 4. Left ventricle.

ASSESSING HEART SIZE Cardiac size can reliably be assessed by measuring the cardio- thoracic ratio (CTR). Normally this ratio is less than 50 percent. This ratio is obtained by measuring the two variables mentioned below (Figure 37.1). 1. Measure the maximum transverse diameter of thorax inside of the ribs. 2. Measure the maximum transverse diameter of the heart. 254 Manual of Cardiology

FIGURE 37.1: The above chest X-ray shows a normal CTR. The horizontal lines represent the measurement for the cardiac size and thoracic size. A ratio of the same is indicative of CTR. RA = right atrium. Ao = aortic knuckle. LV = left ventricle

LEFT ATRIUM 1. Double density is the earliest sign. 2. Elevation of left main bronchus, splaying of the carina. 3. Enlargement of left atrial appendage results in straightening of the left heart border (Figure 37.2 on next page).

RIGHT ATRIUM 1. The right heart border extends for more than 5.5 cm from the midline or 3.5 cm from the right sternal border. 2. Right atrium spans over 2½ intercostal spaces in its vertical extent. 3. Increased curvature or radius of curvature of right heart border. 4. Step like angle between RA and SVC. Chest X-ray 255

FIGURE 37.2: The above chest X-ray is taken from a patient with mitral stenosis. The arrow in the left lung field show prominent upper lobe pulmonary veins—Antler sign. The arrow on the right within the cardiac shadow shows the left atrial border seen within the right atrial shadow (shadow in shadow), a feature of left atrial enlargement

FIGURE 37.3: The above figure show sirhg atrial enlargement in a patient with mitral valve disease and pulmonary arterial hypertension. The arrow shows the enlarged right atrium. 256 Manual of Cardiology

LEFT VENTRICLE In PA view LV forms lower portion of left border and apex while in lateral view it forms the posterior aspect of cardiac silhouette below AV ring.

PA View 1. Rounding of apex: This may be due to hypertrophy alone and may be recognized before significant enlargement. 2. Elongation of long axis of LV to the left and downward is earliest feature of dilatation. Apex may be shifted below diaphragm.

Lateral View 1. Extension behind barium filled esophagus. 2. Displacement of postero - inferior border of heart to a point behind IVC at a point 2 cm above diaphragm. (Hoffman- Rigler sign).

RIGHT VENTRICLE Right ventricle is difficult to visualize directly from the PA view and so lateral view is useful. In PA view the enlarged right ventricle displaces the left ventricle so that the apex is tilted up. In the Lateral view, Sternal contact sign is the earliest and most sensitive. This is always to be interpreted in relation to shape of chest, i.e. depressed sternum increases and bowed sternum decreases area of contact. An abnormality of chest is common in congenital heart disease.

PULMONARY BLOOD FLOW Increased Pulmonary Vascularity 1. PA branches are visualized far beyond the usual two-third of the lung fields. Chest X-ray 257

2. The vessels in both the upper and lower lobes appear to be dilated to the same degree. 3. The numbers of end on vessels are five or more in both the lung fields or three or more in one lung field. 4. The transverse diameter of the right descending pulmonary artery is more than the normal. The normal value for males is 15 mm and in females 14 mm.

Pulmonary Arterial Hypertension 1. Peripheral pruning. 2. Marked dilatation of the MPA and the main pulmonary arteries.

Decreased Pulmonary Blood Flow 1. The pulmonary vascular markings are markedly reduced. 2. The pulmonary vessels in the middle one-third and lateral one third are markedly attenuated.

Congestive Cardiac Failure 1. Kerley B lines, distinct, parallel opacities in the lung bases, oriented perpendicular to the pleural surface 2. Kerley A and C lines: These are the radiographic manifestation of more deeply situated lymphatic segments and appear as randomly distributed linear densities found in the mid-lung fields. 3. Cephalization of blood flow: In these cases, the caliber of an upper lobe pulmonary vessel is greater than that of another vessel measured an equal distance from the pulmonary hilus in the lower lobe. 4. In pulmonary edema, the alveolar air is replaced by edema. The confluent, patchy infiltrates of pulmonary edema usually involve the medial two-thirds of the lungs and expand outward from the mediastinum, giving the appearance of bat wings. 258 Manual of Cardiology

5. The most frequent presentation of acute pulmonary edema is that of bilateral, fluffy pulmonary infiltrates. 6. The size of pulmonary vessel in the first intercostals space is more than 3 mm.

Grading of Pulmonary Venous Hypertension1 The grading of PVH is dependent on the distribution of vessels and the right hilar angle. 1. Grade 1: The diameters of the vessels in the upper zone are either equal to or greater than those of the lower zone vessels. Right Hilar angle: this angle which is normally concave gets obliterated. 2. Grade 2: Interstitial pulmonary edema and pleural effusion. The most reliable signs are Kerley B lines. Kerley A lines are seen in acute or advanced degree or grade 2 PVH. In this, the right hilar angle is straightened. 3. Grade 3: Alveolar edema and the right hilar angle is convex.

REFERENCE 1. Grainger and Allison’s. Diagnostic Radiology—A textbook of medical imaging. Churchill Livingstone 2001;874-6. ECG 259

ECG 38

Normally a 12 lead ECG is taken. However in some circum- stances as mentioned below we may need additional leads such as right sided or posterior leads. ECG is to be read in a systematic way and the intervals to be measured. Nowadays most ECG machines have computerized interpretation. This program automatically detects various intervals, heart rate and can also give a possible diagnosis. This however needs to be confirmed by a physician. 1. Rate: At usual speed of 25 mm/sec in the ECG paper, each small square is 0.04 seconds. Each big line is 0.2 secs. Hence to calculate the heart rate, divide 300 by the number of big squares between the R-R interval. 2. Rhythm: One of the most important uses of ECG is to find out the rhythm. In sinus rhythm, a P wave precedes the QRS complex. In atrial fibrillation, there are no P waves. 3. Axis: The normal axis is between –30 degrees and +90 degrees. • If the complexes in lead I and aVF are both positive, then the axis is normal. • If the complex in lead I is positive and aVF is negative, the axis is left ward. • If the complex in lead I is negative and aVF is positive, the axis is right ward. 4. PR interval: This is measured from the beginning of the P wave to the beginning of the QRS. Normal range is 0.12 to 0.20 secs. A prolonged PR indicates that the AV conduction 260 Manual of Cardiology

is abnormal. A short PR implies that there is accessory conduction of electrical impulse from the atria to the ventricles. Example, WPW syndrome. 5. QRS duration: The normal duration is less than 0.12 secs. If the duration is increased beyond this, it suggests ventricular conduction defect. This can be a right bundle branch block or left bundle branch block. Normal Q waves are less than 0.04 secs and less than 2 mm in depth. Pathological Q waves indicates myocardial infarction. 6. QT interval: This is measured from the start of QRS to the end of T wave. QT interval is always corrected to the heart rate. Normal corrected QT interval is 0.38 to 0.42 secs. Long QT intervals can occur due to some syndromes such as Romano—Ward, Jervell-Lange-Nielson syndrome. This can also be secondary to drugs such as amiodarone, quinidine. The importance of long QT syndrome is that it can lead on to polymorphic ventricular tachycardia. 7. ST segment: ST depression suggests ischemia; can also be due to left ventricular hypertrophy or nonspecific changes. ST segment elevation can be due to myocardial infarction, pericarditis. 8. T wave: If T waves are inverted it may suggest ischemia or it may even be nonspecific. Tall peaked T waves suggest hyperkalemia and T waves are flat in hypokalemia.

LEFT ATRIAL ENLARGEMENT 1. The P wave duration is increased in lead II, III, aVF. 2. There is notching in the P wave with inter peak duration more than 0.04 secs. 3. The P wave is inverted in V1 and the negative component is more prominent than the positive component—this is called as P terminal force. ECG 261

FIGURE 38.1: The above ECG shows left atrial enlargement; note the negative P waves in V1 and notched P waves in lead II (shown by arrows)

RIGHT ATRIAL ENLARGEMENT 1. The P waves are peaked more than 3 mm in lead II. 2. The area under the initial positive deflection of P wave in V1 is more than 0.06 mm/sec.

RIGHT VENTRICULAR HYPERTROPHY 1. R in V1 more than S in V1 or S in V5 more than R in that lead also suggests RVH. 2. R in V1 more than 7 mm suggests RVH but the sensitivity is very low. 3. Right axis deviation is also a feature of RVH.

LEFT VENTRICULAR HYPERTROPHY Though there are several criteria one that is commonly used is the voltage criteria, which is given below. 1. S wave in V1 + R in V5 or V6 more than 35 mm suggests LVH. 2. R in aVL more than 11mm also suggests LVH. 262 Manual of Cardiology

FIGURE 38.2: Right ventricular hypertrophy. Note the peaked P waves in lead II, suggestive of right atrial enlargement. In V1 normally the R wave is small. In this patient the R wave is the dominant wave and the S wave is very small. This suggests right ventricular hypertrophy.

FIGURE 38.3: Note the tall R waves in V5, V6 suggestive of left ventricular hypertrophy. In addition the T waves are inverted in V5, V6 suggestive of LV strain pattern. This occurs in patients with left ventricular hypertrophy secondary to pressure overload such as aortic stenosis.

MYOCARDIAL INFARCTION 1. Within hours of infarction T waves become tall and peaked and the ST segment begins to rise. 2. Shortly thereafter the ST elevation is marked with convexity upwards. ECG 263

3. Within 24 hours, T wave starts to invert and the ST segment comes down. 4. Within a few days after MI, pathological Q waves begins to form. Based on the leads in which ST segment elevation is seen, myocardial infarction can be classified into the following types: 1. Anterior: V1 to V6. 2. Anteroseptal: V1 to V4. 3. High lateral: Lead I, aVL. 4. Extensive anterior: Lead I, aVL, V1 to V6. 5. Inferior: Lead II, III, aVF. 6. Posterior: R waves are tall in V1 with ST depression and T is upright. This usually occurs along with inferior wall myocardial infarction. Posterior leads will usually help in the diagnosis. 7. RV Infarction: Leads recorded from the right chest such as V3 R, V4 R shows the ST changes.

FIGURE 38.4: Anterior wall myocardial infarction. The above ECG shows gross ST segment elevation from V1-V6 suggestive of acute anterior wall infarction. 264 Manual of Cardiology

FIGURE 38.5: Inferior wall myocardial infarction. The above ECG shows ST segment elevation in lead II, III, aVF and ST depression in V1-V3 and lead I, aVL. The above features suggestive of acute inferior wall infarction. INDEX

A treatment 101 Acute myocardial infarction 110 Aortic regurgitation 76 complications 120 apex beat 78 diagnosis 110 auscultation 78 management 114 blood pressure 77 pathology 110 etiology 76 post myocardial indications for surgery 83 infarction care 121 medical management 82 right ventricular infarction 120 peripheral signs 80 risk factors 111 pulse 77 thrombolytic drugs 116 surgical management 82 Acute pericarditis 153 Aortic stenosis 70 complications 155 apex beat 71 ECG 154 auscultation 71 physical examination 153 blood pressure 71 tests 155 chest X-ray 72 treatment 155 etiology 70 Acute pulmonary edema, indications for surgery 74 treatment 135 management 74 Alcohol septal ablation 146 pathophysiology 70 Algorithm for cardiac arrest pulse 71 management 227 Apex beat 25, 78, 186 Angina amplitude 26 classification 96 character 27 coronary angiography 101 double apical impulse 29 coronary artery bypass grafting 104 duration 26 drug therapy 102 dyskinetic impulse 28 investigations 97 hyperdynamic apical medical therapy 106 impulse 28 percutaneous coronary hypokinetic impulse 28 intervention 103 normal apical impulse 27 prinzmetal 109 size 26 prognosis 101 sustained apical impulse 27 stable angina 96 Arrhythmias 172, 207 symptoms 97 Arthritis 46 266 Manual of Cardiology

Atrial fibrillation 207 pathogenesis 141 etiology 207 risk factors 145 in Wolff-Parkinson-White treatment 145 syndrome 212 types 142 pathophysiology 207 Cardiomyopathy, restrictive 148 signs 208 Davies disease 149 symptoms 208 differential diagnosis 149 thyroid function test 209 features 148 treatment 210 medical treatment 149 Atrial flutter 214 Carditis 46 Atrial septal defect 170 Chest pain 3, 5, 56, 96 ASD closure 176 angina 6 associated defects 170 causes 6 auscultation 173 PQRST 5 classification 170 Chest X-ray, investigations complications 173 assessing heart size 253 cyanosis in ASD 174 left atrium 254 ECG 175 left ventricle 256 hemodynamic features 171 right atrium 254 physical appearance 171 right ventricle 256 precordial palpation 172 Chorea 47 symptoms 171 Clubbing 10, 168, 194 with mitral regurgitation 176 causes 10 X-ray 174 testing 11 Coarctation of aorta B clinical features 203 investigations 203 Blood pressure 71, 232 treatment 204 Comparison of dilated, hyper C trophic, and restrictive Cardiac arrest cardiomyopathies 138 management 226 Congenital heart disease 166 reasons for 226 atrial septal defect 170 Cardiac tamponade 156 chromosomal Cardiomyopathy, dilated abnormalities 166 chest X-ray 139 coarctation of aorta 203 clinical features 139 cyanotic lesions 168 ECG 140 Eisenmenger’s syndrome 169 treatment 140 Fallot’s physiology 168 Cardiomyopathy, increased pulmonary hypertrophic 141 blood flow 169 clinical features 142 patent ductus arteriosus 186 ECG 143 pulmonary stenosis 168 genetic causes 142 tetralogy of Fallot 191 Index 267

types 167 ST segment 260 ventricular septal defects 179 T wave 260 Congestive cardiac failure 257 Edema 12 Constriction versus restriction 150 causes 13 Cyanosis 168, 193 Examination of cardiovascular system 9 D clubbing 10 cyanosis 9 Differences between jugular venous pulse and carotids 22 dysmorphic features 9 Differential diagnosis of ECG in edema 12 acute pericarditis versus acute STEMI 154 F Dissection of aorta 162 Fatigue 3, 172 chest X-ray 163 Fredrick’s sign 160 CT/MRI/TEE 163 Frog sign 217 signs 162 symptoms 162 G treatment 163 types 162 Guidelines for measuring BP 232 Drugs used for PSVT 217 circumstances 233 Dyspnea 3, 56, 128, 156, 172 equipment 233 etiology 4 posture 232 NYHA classification 3 technique 234 orthopnea 5 Valsalva maneuver 234 paroxysmal nocturnal dyspnea 4 H platypnea 5 Heart block trepopnea 5 complete AV block 223 first degree 223 E second degree 223 ECG treatment 224 axis 259 Heart failure 127 left atrial enlargement 260 brain natriuretic peptide 130 left ventricular hypertrophy cardiac transplantation 134 261 causes 127 myocardial infarction 262 drug therapy 133 PR interval 259 signs 128 QRS duration 260 symptoms 128 QT interval 260 treatment 130 rate 259 Heart sounds rhythm 259 first heart sound 33 right atrial enlargement 261 fourth heart sound 39 right ventricular hypertrophy second heart sound 35 261 third heart sound 38 268 Manual of Cardiology

Hemoptysis 56 K Hill’s sign 19 Katz Wachtel phenomenon 181 Holiday heart syndrome 208 Kussmaul’s sign 160 Hypertension 230, 235 accelerated hypertension 231 L angiogram 237 hypertensive crisis 239 Lutembacher’s syndrome 171 hypertensive target organ damage 236 M inappropriate hypertension Management of PSVT 218 236 Methods to differentiate investigations 236 obstructive versus isolated systolic hypertension nonobstructive HCM 143 230 Mitral regurgitation Korotkoff sounds 231 etiology 64 malignant hypertension 230 methods of measuring 231 heart sounds 66 orthostatic hypotension 230 indications for surgery 69 pseudohypertension 230 management 69 treatment 237, 239 murmurs 66 types 235 precordial motion 65 white coat hypertension 230 symptoms 65 Hypertensive crisis, drugs Mitral stenosis 54 for 239, 240 atrial fibrillation 56 Hypokalemia 260 auscultation 57 chest X-ray 60 I ECG 60 mitral valve replacement 63 Infective end arteritis 84 pathophysiology 54 Infective endocarditis pulmonary hypertension 56 classification 84 Mitral valve replacement 147 Duke’s criteria 85 Monkeberg’s sclerosis 20 antibiotic treatment 87 Morrow procedure 146 clinical findings 85 complications 86 Myocardial infarction 260 investigations 86 prevention 88 O Ischemic heart disease 92 Orthopnea 128 dyslipidemia 93 Ortner’s syndrome 57 management 93 risk factors 92 P Palpitation 3, 8 J Parasternal lift 29 Jugular venous pulse 21 causes 30 measurement 22 Dressler’s grading 31 waveforms 23 examination technique 29 Index 269

Paroxysmal nocturnal dyspnea Pulse, examination 14 128 bisferiens pulse 16 Patent ductus arteriosus 186 dicrotic pulse 17 auscultation 187 hyperkinetic pulse 16 chest X-ray 187 hypokinetic pulse 16 differential diagnosis 189 pulses paradoxus 19 ECG 189 pulsus alternans 18 management 189 18 physical examination 186 pulsus parvus et tardus 16 Pericardial diseases 152 radiofemoral delay 19 acute pericarditis 153 rate 15 constrictive pericarditis 160 rhythm 15 pericardial effusion 156 vessel wall thickness 20 Pericardial effusion 156 chest X-ray 157 R ECG 157 pericardial fluid analysis 158 Rheumatic fever 43 physical examination 156 arthritis 46 treatment 159 carditis 46 Pericardial knock 160 chorea 47 Pericarditis, constrictive 160 diagnosis 44 clinical features 160 in RHD 45 investigations 161 treatment 49 treatment 161 Rheumatic heart disease 44 PND versus bronchial asthma 129 Rib notching 203 Polycythemia 168 Polymorphic ventricular S tachycardia 260 Sestamibi testing 100 Pulmonary arterial Shunt surgery 201 hypertension 243 Sigwart 146 chest X-ray 244 Sone’s method 101 ECG 244 Sudden arrhythmic cardiac etiology 243 death, causes 226 physical signs 244 Syncope 7 treatment 244 differentiating features 8 Pulmonary embolism acquired conditions 247 T clinical manifestations 247 hypercoagulable states 247 Tachycardia investigations 248 differential diagnosis 216 treatment 249 treatment 217 Pulmonary embolism Target for blood pressure syndrome 248 control 238 Pulmonary venous hypertension, Tetralogy of Fallot 191 grading 258 angiogram 198 270 Manual of Cardiology

associated cardiac defects 192 V associated genetic Ventricular septal defect 179 disorders192 auscultation 180 auscultation 195 cardiac catheterization 183 chest X-ray 197 classification 180 clinical features 193 ECG 181 complications 198 indications for surgery 185 differential diagnosis 198 location 179 ECG 196 medical management 185 embryology 191 natural history 184 hemodynamics 192 size 179 symptoms 180 pathology 191 X-ray 181 spell 194 Ventricular tachycardia surgery 200 differential diagnosis 220 Thrills 31 etiology 220 Treadmill testing 98, 145 treatment 220