THE RECOGNITION AND MANAGEMENT OF VALVULAR HEART DISEASE

Discussion of heart murmurs tends to be associated with specialist ward rounds in teaching hospitals, but a good understanding of this clinical sign provides valuable information.

AUSCULTATING A

When does it occur? •Time the murmur in systole or diastole to the first heart sound and by palpating the upstroke of the carotid artery as systole. • Is the murmur early, mild, late, holosystolic, or diastolic?

How loud is it? •Grade I — very soft, only heard with special effort • Grade II — soft, faint, but heard immediately • Grade III — moderately loud • Grade IV — so loud that a thrill can be felt •Grade V — very loud, heard with only part of the stethoscope on the chest wall J A KER • Grade VI — heard with the stethoscope removed from the chest wall. MB ChB, MMed (Int), MD Professor Where is it maximal?

Department of Internal Medicine • Apex, left parasternal area, aortic area, pulmonary area. School of Medicine Where does it radiate to? University of Pretoria • Neck, axilla, back.

Categories of heart murmurs There are three broad categories of heart murmurs: • systolic (murmur begins with S1 or after S1, ends at S2) • diastolic (murmur begins after S2, ends before S1) • continuous (murmur continues without interruption from systole through S2 into diastole. Typical in patent ductus arteriosus).

Systolic heart murmurs Systolic murmurs are illustrated in Fig. 1 and are classified as: • early systolic • midsystolic •late systolic • holosystolic (pansystolic)

Early systolic murmurs occur in acute severe mitral regurgitation, tricuspid regurgitation (with normal right ventricular (RV) pressures) and ventricular septal defect (VSD).

Midsystolic murmurs occur in aortic sclerosis (often in the elderly), and pulmonary valve stenosis, but can also be a feature of an innocent murmur (common in children, adolescents and young adults).

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Diastolic heart murmurs • Elevated jugular venous Diastolic heart murmurs are neither pressure (JVP). This is a sign of benign nor innocent, and begin with . The patient should be Early systolic or after S2. They are classified as supine at 45°. External jugular follows: pressure can be elevated without S1 A2P2 right heart failure, but internal S 2 Early diastolic murmurs are jugular pressure is more accurate as classic of aortic regurgitation. They a predictor of heart failure. The are best heard with the diaphragm of venous column in the internal is Midsystolic the stethoscope, listening at the left very difficult to palpate, but the parasternal area, with the patient pulsations may readily be seen. S1 • Abdomino-jugular reflux sign. A2P2 sitting upright, expiring completely, This is a useful sign to diagnose S2 and leaning forward. Sometimes, with severe , the early heart failure. A positive sign pulmonary valve can become is defined by an increase in JVP of Late systolic regurgitant (Graham Steell’s murmur). greater than 3 cm, sustained for longer than 15 seconds. During the

S1 Mid-diastolic murmur. There is a examination the patient is supine A2P2 with the head elevated to 45°. The clear interval after S2 before the S2 murmur starts, the best example being bladder of a sphygmonometer is Holosystolic mitral stenosis. inflated, pressing the bulb 1 - 3 times, after which the bulb must be closed and the partially inflated S1 A2P2 FEATURES OF A HIGH-RISK bladder flat laid flat on the upper

S2 HEART MURMUR abdomen. With the open hand the examiner presses the bladder until the mercury column is at 20 mmHg Fig. 1. Systolic murmurs. As a general rule it is necessary to refer a patient with a heart murmur if and keeps it there for 15 seconds any of the features mentioned below while watching the JVP. The patient should breathe normally and not A more sinister cause is mitral are present. The more features hold his/her breath. regurgitation as a result of ischaemic present, the higher the risk. These • Left parasternal heave. A left heart disease. features can be interpreted as being indicative of significant or parasternal heave palpated with the ball of the hand implies RV Late systolic murmurs. The classic pathological heart murmurs. hypertrophy, indicative of cause is mitral valve prolapse, also pulmonary hypertension — a associated with one or more systolic Symptoms complication of valvular disease, clicks. •Effort intolerance, reduced exercise capacity or dyspnoea more than especially mitral valvular disease. Occasionally this left parasternal Holosystolic murmurs are classic grade I (New York Heart heave may be caused by a murmurs of mitral regurgitation and Association). massively enlarged left atrium high-pressure tricuspid regurgitation. •Orthopnoea, or increase in number (especially in mitral valvular A distinctive and diagnostically of pillows used at night, or disease). important feature of tricuspid paroxysmal nocturnal dyspnoea • Loud P . A loud P is indicative of regurgitation is that the murmur (PND). 2 2 pulmonary hypertension. becomes louder with inspiration • Oedema of the ankles. • Cardiomegaly. The normal apex (Carvallo’s sign). The murmur of VSD • ( or angina-like). beat (most lateral, inferior pulsation can also be holosystolic. Systolic • Syncope or pre-syncope could be an on chest) should be in the 5th murmurs that radiate or are associated important symptom of a severe intercostal space (ICS) in the with a palpable thrill are almost aortic valve lesion. midclavicular line. If the always pathological. Clinical signs is more inferolateral, cardiomegaly Features of a benign or • Blood pressure. Low blood must be considered. innocent heart murmur pressure, a very narrow • High-risk signs on : The murmur is soft (grade II or less) and pressure (systolic blood pressure • murmur of grade III or more usually midsystolic. It is heard at the left minus diastolic blood pressure), or a • holosystolic murmur sternal edge, it does not radiate and very wide pulse pressure (> 50 • murmur radiating to the neck there are no other cardiac abnormalities. mmHg) could help in the evaluation (carotids), axilla or back of heart murmurs. • diastolic murmurs • Pulse. If the pulse is irregular, • . consider atrial fibrillation.

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• Signs suggestive of infective bladder and fixate it. Close the tubing (mitral stenosis, mitral regurgitation) . There are numerous system at the bulb, inflate it with 1 - 3 are particularly prone to develop an minor signs of infective endocarditis, pumps and then close the bulb. The increasing severity of pulmonary e.g. anaemia, fever, splinter patient squeezes the rolled-up bladder hypertension. haemorrhages, haematoma. with the non-dominant hand A positive blood culture is a major maximally, while the doctor reads the • Cardiac failure. Valvular heart sign of infective endocarditis. level of the mercury. The patient disease is an important cause of heart • Signs suggestive of embolism. repeats the manoeuvre, but the failure, which will lead to right heart Stroke in a young person, pressure is kept constantly at 50 - 70% failure. haematuria, or absent in a of the maximum, while the doctor patient with a heart murmur are listens to the heart. During this • Infective endocarditis. A heart indicative of embolism. manoeuvre, there will be a depression murmur, especially of valvular origin, • ECG signs: of the vagus tone and an increase in is a high-risk situation as a result of • no P-waves, with irregular R-R sympathetic tone, which will cause which a patient can easily, during intervals (atrial fibrillation) and elevate blood bacteraemias, develop infective • LV or RV hypertrophy pressure and cardiac output. All left- endocarditis. • ST-segment changes or T-wave sided regurgitant murmurs may inversions. decrease. If there is underlying left • Dysrhythmias. A pulse, irregular • Chest X-ray signs: ventricular (LV) dysfunction in in rhythm and volume, is indicative of

• cardiomegaly (cardiac size more ischaemic heart disease, an S4 or S3 atrial fibrillation and is a common than 50% of intrathoracic may be elicited by the manoeuvre. complication of valvular disease, diameter) Mitral regurgitation may be elicited if especially mitral valve disease • valvular calcifications (best seen on there is an underlying ischaemic (stenosis and regurgitation). lateral radiograph) papillary muscle. • any sign of pulmonary eodema • Embolism, usually presenting as (Kerley’s B lines, interstitial lines, etc.) Respiration stroke or peripheral arterial disease. • any sign of pulmonary Inspiration increases venous return, hypertension (large pulmonary which will increase RV volume. MANAGEMENT OF VALVULAR cone or large ). Therefore, right-sided S3 or S4 and the systolic murmur of tricuspid DISEASE BEDSIDE MANOEUVRES regurgitation will increase with inspiration. This also holds true for When a cardiac murmur is heard The accuracy of the cardiac physical the murmur of tricuspid stenosis, during routine auscultation of the heart examination for valvular lesions is pulmonary stenosis and pulmonary the following questions should be enhanced by the use of simple regurgitation. asked: bedside manoeuvres that temporarily • Is it systolic or diastolic? alter cardiac haemodynamics. Postural changes •Are there any features of a high-risk The simplest is to let the patient move patient? Valsalva manoeuvre from a standing to a squatting position • Could it be benign? •Are there any complications? Remove the bulb of a while the examiner listens to the heart sphygmomanometer and place the (the latter usually stays seated while If it is not a benign murmur, a rubber tubing leading to it into the listening to the same area). If the diagnosis is necessary. For this patient’s mouth. The patient blows patient is physically unable to do this, purpose referral to a specialist into this tube until the mercury column he/she remains supine while the physician or cardiologist may be is 40 mmHg and continues blowing to examiner bends the patient’s knees necessary. keep the mercury at 40 mmHg for 15 passively towards the abdomen. Standing produces the same seconds while the doctor listens to the Preventive measures heart. The Valsalva manoeuvre will physiological results as the Valsalva Primary prevention reduce the intensity of all left-sided manoeuvre. With squatting the effects murmurs except in the case of: are the opposite. Squatting causes a Prevention of primary attacks of • hypertrophic obstructive right-sided murmur and aortic and depends on prompt cardiomyopathy mitral regurgitation to increase, but recognition and adequate treatment of group A streptococcal (GAS) • mitral valve prolapse. mitral prolapse and hypertrophic tonsillopharyngitis. Eradication of Caution should be exercised when obstructive cardiomyopathy (HOCM) GAS from the throat is essential with performing the Valsalva manoeuvre in to decrease. the administration of appropriate patients with ischaemic heart disease. GENERIC COMPLICATIONS OF antibiotics, penicillin being the Isometric exercise VALVULAR DISEASE antibiotic of choice. Although it has a narrow spectrum of activity, it has a This is performed with a sustained longstanding proven record and is the • Pulmonary hypertension. isometric handgrip, using a least expensive. sphygmomanometer. Roll up the Patients with mitral valve diseases

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Dosage regimens are the • for penicillin allergy — and often relieve symptoms, especially following: erythromycin 250 mg twice a day during exercise. Benzathine penicillin G per mouth. • 600 000 units once Atrial fibrillation is a common intramuscularly for patients Prevention of infective complication of many valvular < 27 kg. endocarditis diseases; it will lead to deterioration •1.2 million units once Patients with valvular heart disease in cardiac function and it can cause intramuscularly for patients (rheumatic or degenerative in origin) embolism, especially stroke. Early > 27 kg. require additional short-term antibiotic anticoagulation with warfarin to a Penicillin V prophylaxis before certain dental or target International Normalised Ratio • Children: 250 mg 3 times/day surgical procedures to prevent the (INR) of 2 - 3.1 is nearly always per mouth for 10 days. possible development of infective warranted. To control the rate, • Adults: 500 mg 3 times/day per endocarditis. Patients with a history digoxin, beta-blockers or rate-limiting mouth for 10 days. of a previous episode of infective calcium channel blockers (especially Penicillin allergy endocarditis are at particularly high verapamil) are effective. A resting •Erythromycin 40 mg/kg/day risk of again developing endocarditis. pulse rate of below 90/min and (2 - 4 times/day) per mouth for Those with prosthetic valves are also absence of any pulse deficit should be 10 days. in a high-risk category. It is very seen as adequate rate control. The newer macrolides may be used, or important to realise that antibiotic an oral cephalosporin for 10 days. regimens in use to prevent recurrences PRESERVATION OF LV The following antibiotics should not be of rheumatic fever are inadequate to FUNCTION used to treat GAS: tetracyclines, prevent infective endocarditis. trimethoprin-sulfamethoxazole, Whenever a patient with a valvular The use of ACE- inhibitors, as in the chloramphenicol. disease needs to undergo a dental, original heart failure landmark trials, surgical or invasive diagnostic should always be considered and may Secondary prevention procedure the general practitioner preserve ventricular function. After the acute treatment, secondary should seek advice from a specialist prophylaxis should be considered. with regard to the best prophylaxis for Patients who have had a previous infective endocarditis. IN A NUTSHELL attack of rheumatic fever or who have The classification of heart murmurs Medical management rheumatic valvular disease are at high into systole or diastole is the first risk of recurrent attacks of In the vast majority of patients with step in assessing a murmur. endocarditis. These patients should valvular disease, the decision whether Thereafter, certain manoeuvres will therefore receive continuous to replace the valve will be taken by assist in identifying certain antimicrobial prophylaxis. Medical the cardiologist/specialist physician. conditions. Risk assessment is done practitioners should consider each There are, however, patients for whom to try to distinguish between a individual situation when determining medical therapy is the only option. relatively ‘benign’ and relatively the duration of continuous prophylaxis. There are many frail older patients ‘dangerous’ murmur. Prophylactic Rheumatic fever with proven carditis with symptomatic valvular disease measures, mainly antibiotics, should and residual valvular disease should (mainly degenerative) in whom the be considered. receive prophylaxis for at least 10 risks of surgical intervention are years after the last attack of rheumatic prohibitive and for them medical fever, and perhaps until the age of 40 treatment is the only option. It may Further reading Boon NA, Bloomfield P. The medical years, sometimes lifelong. Patients also be possible to influence the management of valvular heart disease. with rheumatic fever with carditis but natural history of valvular disease Heart 2002; 87: 395-400. without valvular disease, should using medical management. Many of Ckinzner MA. The diagnosis of heart receive prophylaxis for 10 years or the landmark trials on the treatment of disease by clinical assessment only. well into adulthood (whichever is heart failure included patients with Curr Probl Cardiol 2001; 26: 290-379. longer). Patients with heumatic fever valvular disease and heart failure. Richardson TR. Bedside cardiac without carditis only need prophylaxis examination: Constancy in a sea of change. Curr Probl Cardiol 2000; 25: 791-825. for 5 years or until the age of 21 years, whichever is longer. Successful What can be done? prophylaxis depends primarily on Symptom control. Diuretics can patient compliance and adherence to relieve the symptoms of congestion but the regimens. must be used cautiously and in low doses so as not to influence the Dosage regimens are the following: haemodynamics of valvular disease • penicillin V 250 mg twice a day negatively. Beta-blockers, cautiously per mouth used in low doses, can by slowing the heart rate improve ventricular filling

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