USMLE STEP 2 CK REVIEW ~ CARDIOVASCULAR

ISCHEMIC HEART DISEASE

. Coronary Artery Disease (CAD) o Accumulation of atheromatous plaques within walls of coronary arteries that supply O2 to myocardium .  Blood flow causes ischemia of myocardial cells due to lack of oxygen  Effects of ischemia reversible if blood flow to heart improved . Complete occlusion of artery causes irreversible cell death called myocardial infarction o Risk Factors: . DM, HTN, tobacco, age >45, hyperlipidemia,  LDL,  HDL, homocysteine .  FHx of premature CAD or MI in 1st-degree relative – men <45yrs & women <55yrs o Clinical Presentation  Asymptomatic, stable angina, unstable angina, MI, sudden cardiac death o Canadian Cardiovascular Society (CCS) Angina Classification: . Class I  Angina w/strenuous or rapid activity – no angina from ordinary physical activity . Class II  Slight limitation of ordinary activity – angina after >2 blocks or >1 flight of stairs . Class III  Marked limitation of ordinary activity – angina after <2 blocks or <1 flight of stairs . Class IV  Any physical activity causes discomfort – angina may be present at rest o Diagnosis: . Resting ECG:  Prior MI  Q waves  UA  ST segment or T-wave abnormalities seen during episode of chest pain . Stress ECG:  Recording ECG before, during & after exercise on treadmill  75% sensitive only if able to exercise sufficiently to reach 85% of predicted maximum HR o Predicted maximum HR = 220 − Patient’s age  Should undergo cardiac catheterization if  results from stress test  Positive findings for CAD: o ST elevation  Transmural infarct o ST depression  Subendocardial ischemia o Failure to exercise >2mins due to symptoms o Hypotension o Ventricular arrhythmia . Stress Echocardiography:  Performed before & immediately after exercise – preferred over stress ECG  More sensitive in detecting ischemia, can assess LV size, EF & diagnose valvular disease  Ischemia evidenced by wall motion abnormalities not seen at rest – akinesis or dyskinesis  Should undergo cardiac catheterization if  test results . Myocardial Perfusion Imaging (MPI):  Stress test preformed after injection w/radioisotope – Thallium-201 or Technetium-99  Nuclear imaging obtained immediately after exercise & again after 4hrs  Viable cells extract radioisotope from blood – no uptake means no perfusion to area  MPI can assess myocardial viability by detecting perfusion, ventricular volume & EF o Reversible ischemia  Impaired perfusion only during stress o Infarction  Impaired perfusion at rest & during stress  MPI has best sensitivity & specificity of exercise stress tests – but more expensive . Pharmacologic Stress:  If unable to exercise – administer IV agents to stimulate myocardial function  Dobutamine   Myocardial O2 demand by  HR,  BP &  contractility  Adenosine & Dipyramidole  Vasodilation actually causes  flow to coronary arteries . Holter Monitoring  Ambulatory ECG useful in detecting silent ischemia . Cardiac Catheterization & Coronary Angiography:  Most accurate way of identifying severity of vessel involvement – indicated in following: o After positive results from any stress test o If revascularization or other surgical intervention being considered o Angina – difficulty diagnosing, refractory to medical therapy, occurring after MI o Severely symptomatic requiring urgent diagnosis & management o Evaluation of valvular disease to determine need for surgical intervention 1 o Treatment: . Risk Factor Modification:  Smoking cessation – 50% reduction in CAD risk 1yr after quitting  HTN control – esp. diabetics  Hyperlipidemia – low-fat diet & HMG-CoA reductase inhibitors  DM – strict glycemic control to reduced vascular disease risk  Exercise & Weight loss – also modifies other risk factors like DM, HTN, hyperlipidemia  Diet –  intake of saturated fat (<7% total calories) &  cholesterol (<200 mg/day) . Pharmacologic Management:  Aspirin   Morbidity &  risk of MI – indicated in all pts. w/CAD o Clopidogrel  If ASA contraindicated or in combination w/ASA  β-blockers   O2 demand via  HR,  contractility,  BP o  Mortality risk – shown to reduce frequency of coronary events o Cardioselective agents preferred – Metoprolol, Atenolol, Carvedilol . Avoids inhibition of peripheral vasodilation & bronchodilation via 2  Nitrates   O2 demand via vasodilation –  preload,  afterload,  coronary perfusion o Symptomatic control of angina only – no impact on survival o Tachyphylaxis – prevent tolerance by maintaining daily nitrate-free intervals  CCBs   O2 demand via coronary vasodilation –  afterload,  coronary perfusion o 2nd line when β-blockers or Nitrates not fully effective o Caution: Verapamil/Diltiazem + -blockers may cause bradycardia or AV block  ACEIs  Not used to treat symptomatic CAD – indicated in CHF, HTN, DM, LV dysfunction . Revascularization:  Percutaneous Coronary Intervention (PCI): o PTCA indicated in moderate one- or two-vessel disease w/normal EF: . Medically refractory angina . NSTEMI/UA w/TIMI risk score ≥3 . Primary PCI for STEMI o Restenosis rates up to 40% within first 6 months – stents significantly  rates  Coronary Artery Bypass Grafting (CABG): o Indicated in severe multi-vessel disease w/ EF or comorbid DM: . Left main artery disease . Triple-vessel disease w/ LV function . Two-vessel disease w/proximal LAD stenosis . Failed or contraindicated PTCA  Thrombolysis  t-PA, Streptokinase, Urokinase, Anistreplase, Alteplase, Reteplase o Indications  STEMI <12hrs after symptom onset or when PCI not viable option . Administer as soon as possible – preferably 3hrs after onset . Not indicated for NSTEMI or UA as artery not occluded o Absolute contraindications: . Active bleeding - Malignant IC neoplasm . Prior intracranial hemorrhage - Prior stroke (≤3 months) . Recent head trauma (≤3 months) - Suspected aortic dissection o Relative contraindications: . HTN – sBP >180 or dBP >110 - Current anticoagulation use . Recent major surgery (≤3wks) - Active peptic ulcer

. Chronic Stable Angina o Symptom complex resulting from imbalance between oxygen supply & demand in myocardium o Often due to fixed stenosis caused by atheroma – O2 demand exceeds available blood supply . Goal of treatment to  myocardial O2 demand &  O2 supply o Pathophysiology: . Factors influencing supply  Luminal diameter (most important), duration of diastole, Hb, SaO2   Myocardial O2 supply – atherosclerosis, vasospasm, hypoxemia, tachycardia, anemia . Factors influencing demand  HR, contractility, wall stress   Myocardial O2 demand – tachycardia, AS, myocardial hypertrophy, hyperthyroidism o Clinical Presentation: . Chest pain  Retrosternal tightness radiating to left ( right) shoulder, arm, neck or jaw 2  Precipitated by the “3 E’s” – 1) Exertion, 2) Emotion, 3) Eating  Brief duration – pain lasts <15mins w/relief after rest or nitrates . Anginal equivalents  Dyspnea, diaphoresis, acute LVF, flash pulmonary edema . Levine’s sign  Clutching fist over sternum when describing chest pain o Diagnosis  Resting ECG – Normal o Treatment  See Pharmacologic Management of CAD

. Unstable Angina (UA) o Acute plaque rupture & thrombosis w/incomplete or transient vessel occlusion O . Oxygen demand unchanged – but  O2 supply 2 to reduced resting coronary flow o UA defined by any of the following: . Angina at rest . New-onset angina . Accelerating pain pattern –  frequency,  duration,  pain intensity o Diagnosis & Treatment  See NSTEMI section below

. Prinzmetal’s (Variant) Angina o Transient coronary vasospasm – 75% ass. w/fixed atherosclerotic lesion o Clinical Presentation  Intermittent angina at rest – pain not provoked by exertion o Diagnosis: . ECG  Hallmark is transient ST elevation during chest pain – represents transmural ischemia . Coronary Angiography  Definitive test – shows coronary vasospasm after IV Ergonovine o Treatment  Vasodilation via Nitrates & CCBs

. Myocardial Infarction (MI) o Due to coronary atherosclerosis w/superimposed thrombus on ruptured plaque o Classification: . 1) Non-ST Elevation Myocardial Infarction (NSTEMI):  Acute plaque rupture & thrombosis w/incomplete or transient vessel occlusion O  Oxygen demand unchanged – but  O2 supply 2 to reduced resting coronary flow  Defined by presence of 2/3 criteria: o Symptoms of angina or ischemia o Rise & fall of serum MI markers – absent enzyme markers in UA o Ischemic ECG changes – without ST elevation or new LBBB  NSTEMI & UA categorized together due to similar etiology & presentation . 2) ST Elevation Myocardial Infarction (STEMI):  Acute plaque rupture & thrombosis w/total vessel occlusion causing myocardial necrosis  Defined by new ischemic ECG changes + 1 or both of ischemic Sx. &  cardiac enzymes o ECG  ST elevation in 2 contiguous leads or new BBB – either LBBB or RBBB o Clinical Presentation: . Chest pain, dyspnea, diaphoresis, weakness, fatigue, N/V  Pain similar to angina but more intense & longer duration  Up to 1/3 asymptomatic – painless infarcts o Diagnosis: . ECG  Markers for ischemia/infarction include:  Peaked T-waves – occur very early & may be missed  ST elevation – transmural injury of entire wall thickness (can be Dx. of acute infarct)  ST depression – subendocardial injury involving inner half of wall  Q-waves – seen later as specific evidence of necrosis (not seen w/NSTEMI)  T-wave inversion – sensitive but not specific . Cardiac Enzymes  Diagnostic gold standard for myocardial injury  Creatine Kinase-MB (CK-MB): o  CK-MB within 4-8hrs w/peak at 24hrs – returns to normal after 48-72hrs o Sensitivity & specificity >95% if measured within 24-36hrs of onset of chest pain o CK & CK-MB should be measured on admission & every 8hrs over 24hr period  Troponin I & T: o  Troponin within 3-5hr w/peak at 24-48hrs – returns to normal after 5-14 days o Greater sensitivity & specificity than CK-MB for myocardial injury 3 . Troponin I can be falsely elevated in renal failure o Troponin should be measured on admission & every 8hrs over 24hr period  Creatinine Phosphokinase (CPK)  Elevated within 4-8hrs – but nonspecific  Myoglobin  Elevated within 1hr – but nonspecific o TIMI Risk Score: . 1 point for each criteria met:  Age >65 yrs  At least 2 anginal episodes in last 24hrs   Serum cardiac biomarkers  ASA use in prior 7 days  Presence of 3 or more CAD risk factors  Prior coronary stenosis ≥ 50%  ST segment deviation on admission ECG . Risk of all cause mortality at 14 days:  0-1 points = 4.7%  4 points = 19.9%  2 points = 8.3%  5 points = 26.2%  3 points = 13.2 %  6-7 points = 40.9% o Treatment: . Management of NSTEMI:  Initial  O2 + NTG + -blockers + ASA  Morphine  Clopidogrel  Initiate w/ASA – 300mg loading dose & 75mg daily o Avoid use if emergency CABG likely – discontinue 5 days before CABG  Heparin  For all pts. w/MI – does not dissolve clots only prevents future formation o LMWH preferred – except in renal failure or if CABG planned within 24hrs  GP IIb/IIIa inhibitors  Abciximab, Eptifibatide, Tirofiban o If undergoing PCI or at high-risk – ex.  Troponin, TIMI >4, DM  Invasive  Early coronary angiography & revascularization o Indicated w/following high-risk indicators: . Recurrent or persistent pain refractory to medical therapy . LV dysfunction – EF <40% . TIMI risk score ≥3 . Sustained VT or dynamic ECG changes . High-risk findings on non-invasive stress testing . PCI within previous 6 months o Thrombolysis is NOT indicated for NSTEMI or UA! . Management of STEMI:  Initial  O2 + NTG + -blockers + ASA  Morphine  Invasive  Emergency reperfusion indicated if <12hrs from symptom onset o PCI  Preform <90mins (EMS-to-balloon) after first medical encounter . Advantages over thrombolysis:   Mortality rate &  rate of MI reoccurrence   Risk of intracranial hemorrhage  90% success rate – vs. 60% w/thrombolytics o Thrombolysis  Preform <30mins (EMS-to-needle) after first medical encounter . Generally preferred if given <3hrs after symptom onset . Indicated if PCI contraindicated or unable to preform PCI within 90mins o CABG  Indicated if >12hrs after symptom onset or failed PCI  Adjunctive medical therapy: o Clopidogrel  300-600mg loading dose & 75mg daily . Avoid use if emergency CABG likely – discontinue 5 days before CABG o Heparin  Initial bolus of UF advised while in transit to catheterization lab . LMWH preferred – except renal failure or if CABG planned within 24hr o GP IIb/IIIa inhibitors  If undergoing primary PCI . Avoid use if initiating thrombolytic therapy due to  risk of bleeding . Outpatient Management:  Aspirin  81-162mg QD  -blockers  Metoprolol 25-50mg BID or Atenolol 50-100mg QD o CCBs  If -blockers contraindicated in absence of severe LV dysfunction . Use non-dihydropyridine CCBs – Diltiazem or Verapamil  Clopidogrel  75mg QD x 1-12 months – at least 12 months if stent placed  Nitrates  Alleviates pain – caution in right-sided MI pts. who are preload dependent 4  Warfarin  If high risk of systemic thromboembolism – A-Fib, CHF, LV thrombus  ACEIs  Prevents adverse ventricular remodeling – CHF,  LVEF <40%, anterior MI o ARBs – if contraindicated or intolerant to ACEIs  Statins  Early & intensive therapy o Complications: . CHF  MCC of in-hospital mortality – if severe may lead to cardiogenic shock . Myocardial ruptures:  Free wall rupture  90% occur within 2wks – MC after 1-4 days w/90% mortality rate o Often leads to hemopericardium & cardiac tamponade o Treat w/immediate pericardiocentesis & surgical repair  Interventricular septum rupture  Occurs within 10 days after MI o Emergent surgery indicated – likelihood of survival correlates w/size of defect  Papillary muscle rupture  Leads to MR – if suspected obtain ECHO immediately o Emergent surgery & mitral valve replacement usually necessary o Reduce afterload via Sodium nitroprusside or intra-aortic balloon pump (IABP)  Ventricular pseudoaneurysm  Incomplete free wall rupture – contained by pericardium o Surgical emergency – likely to progress to free-wall rupture  Ventricular aneurysm  Rarely rupture – in contrast to pseudoaneurysms o Associated w/ incidence of ventricular tachyarrhythmias o Medical management may be protective or surgery if aneurysm severe . Arrhythmias:  VT  Within 48hrs of MI – usually due to myocardium reperfusion  Bradycardia  Usually from inferior wall MI – seen in early stages o May be protective mechanism causing  O2 demand – treat w/Atropine  AV block  Due to ischemia of conduction tracts o Inferior wall MI  1st degree or 2nd degree (type I) block – no treatment o Anterior wall MI  2nd degree (type II) or 3rd degree block – requires pacemaker . Recurrent infarction  Extension of existing infarction or re-infarction of new area  Suspect if repeat ST elevation on ECG within first 24hrs after infarction . Acute pericarditis  Treat w/Aspirin . Dressler's syndrome  Autoimmune inflammatory reaction occurring weeks to months after MI  Consists of fever, malaise, pericarditis, leukocytosis & pleuritis – treat w/NSAIDs

HEART FAILURE

. Congestive Heart Failure (CHF) o Failure of heart to pump blood effectively to tissues – heart can not meet demand of body o Pathophysiology: . Systolic dysfunction  Impaired ventricular ejection –  LVEF +  SV =  CO  Findings – apex beat displaced, S3, cardiomegaly,  LVEF, LV dilatation  Due to impaired myocardial contractility – MI, HTN, DM, alcohol, myocarditis, DCM . Diastolic dysfunction  Impaired ventricular filling during diastole w/normal EF – MC in elderly   LV filling pressures produce upstream pulmonary & systemic venous congestion  Findings – HTN, apex beat sustained, S4, normal-size heart, LVH, normal LVEF  Due to decreased compliance: o Transient  Ischemia – relaxation of myocardium is active & requires ATP o Permanent  Hypertrophy (HTN, AS, HCM), RCM, MI . High-Output HF   CO demand –  thiamine, hyperthyroid, A-V fistula, L-R shunt, Paget’s, CRF o Etiology: . Most common  CAD, HTN, valvular (AS, AR, MR), EtOH (DCM) . Less common:  Toxic – anthracyclines, radiation, uremia, catecholamines  Infectious – Chagas, Coxsackie, HIV  Endocrine – Hyperthyroidism, DM, acromegaly  Infiltrative – sarcoidosis, amyloidosis, hemochromatosis  Genetic – HCM, Friedreich’s ataxia, muscular dystrophy, congenital heart disease  Metabolic – thiamine or selenium deficiency 5 o Clinical Presentation: . LHF  Pulmonary venous congestion  Cool extremities, slow capillary refill, peripheral cyanosis  Syncope, DOE, systemic hypotension, tachycardia, pulsus alternans  Orthopnea – SOB while laying flat & relived by elevation of head w/pillows  Paroxysmal nocturnal dyspnea (PND) – causes night time awakenings  Auscultation: o Displaced PMI – usually to left due to cardiomegaly o S3 ventricular gallop – due to rapid filling into noncompliant LV . May be normal finding in children o Crackles/rales at lung bases – indicates pulmonary edema o Dullness to percussion &  tactile fremitus of lower lung – due to pleural effusion . RHF  Systemic venous congestion  MCC of RHF is LHF – can mimic LHF if  RV output leads to LV under-filling  Cyanosis, peripheral edema, JVD, Kussmaul’s sign  Nocturia – due to  venous return w/elevation of legs  Hepatomegaly  hepatojugular reflex – RUQ pain due to hepatic congestion  Auscultation  S3 (right-sided), right ventricular heave, tricuspid regurgitation  RHF can present similar to cirrhosis – but w/cirrhosis no JVD or trouble lying flat o New York Heart Association (NYHA) Classification: . Class I  Nearly asymptomatic – symptoms only w/vigorous activities . Class II  Slight limitation of activities – symptoms w/moderate exertion (ex. climbing stairs) . Class III  Markedly limiting – symptoms w/normal activities of daily living (ex. walking) . Class IV  Incapacitating – symptoms occur at rest o Diagnosis: . CXR  Cardiomegaly, pleural effusion, Kerley B-lines, bronchiolar-alveolar cuffing . ECHO  Assess LV function via LVEF – helps distinguish systolic from diastolic dysfunction  Initial test of choice – preform when CHF suspected based on PE findings or CXR . ECG  Nonspecific – may show chamber enlargement, arrhythmia, ischemia/infarction . Radionuclide Angiography (MUGA)  Precise measurement of left & right ventricular EF  RBCs tagged w/radioisotope & imaged during exercise & rest  Useful when ECHO suboptimal or more precise assessment of LVEF needed  Can also assess wall motion abnormalities in ischemic heart disease .  BNP  Secreted by ventricles due to LV stretch & wall tension  Basic natriuretic peptide precursor cleaved into proBNP & secreted into ventricles  proBNP cleaved into active C-terminal portion & inactive NT-proBNP portion o HF suggested if NT-proBNP >450pg/mL o Treatment: . Diet  Sodium restriction – initially <4g/day . Diuretics  No effect on prognosis or mortality – Furosemide is most potent diuretic . ACEIs   Mortality & prolongs survival – venous & arterial dilation =  preload &  afterload  Diuretics + ACEI is initial treatment in most symptomatic cases  ACEIs given to all pts. w/systolic dysfunction – even if asymptomatic  Start w/low dose to prevent hypotension – monitor BP, K+, BUN, Cr . β-Blockers   Mortality in pts. w/post-MI heart failure  Slows progression of heart failure by slowing down tissue remodeling  Use cautiously & titrate slowly as may initially worsen CHF . ARBs  2nd line only if unable to tolerate ACEIs . Hydralazine & Isosorbide Dinitrates  2nd line only if unable to tolerate ACEIs  Less effective on reducing mortality compared to ACEIs . Digitalis  No effect on mortality – only symptomatic relief  Consider use if EF <30%, severe CHF, or severe A-fib o Management Guidelines: . Mild (NYHA Class I to II)  Na+ restriction + ACEI  Loop diuretic . Moderate (NYHA Class II to III)  Loop diuretic + ACEI  -blocker . Severe (NYHA Class III to IV)  Loop diuretic + ACEI + Digoxin  Add Spironolactone – if class IV & still symptomatic despite above treatment 6 o Complications: . Acute Pulmonary Edema (APE)  Caused by rapid decompensation of LV function  Precipitants – dysrhythmias, MI, medication noncompliance,  Na+ load,  inotropy  Treatment – NOMAD: NTG, Oxygen, Morphine, Aspirin, Diuretic . Paroxysmal Nocturnal Dyspnea (PND)  Brief SOB that awakens pt. from sleep – treat w/NOMAD  Due to  volume load when lying horizontal or sudden  in myocardial contractility o Results in pulmonary edema causing impaired exchange of oxygen  Improvement w/walking & no response to bronchodilators – differentiates from asthma

MYOCARDIAL DISEASE

. Myocarditis o Etiology: . Viral  Coxsackie B (MCC), Echovirus, HIV, CMV, Influenza, EBV, HBV, Adenovirus . Bacterial  S. pyogenes, C. diphtheriae, Meningococcus, B. burgdorferi, M. pneumoniae . Parasitic  T. cruzi (Chagas), Toxoplasma, Trichinella, Echinococcus . Systemic  Kawasaki’s, SLE, sarcoidosis, acute RF . Toxic  Catecholamines, chemotherapy, cocaine . Hypersensitivity  Antibiotics, diuretics, lithium, clozapine, insect/snake bites o Clinical Presentation: . Varies from asymptomatic to fulminant cardiac failure & death  Acute CHF – dyspnea, rales, peripheral edema, JVD  Chest pain – due to pericarditis or cardiac ischemia  Fever,  preceding URI, palpitations, arrhythmias, syncope . Auscultation – S3/S4, MR, TR,  friction rub if pericardium involved o Diagnosis: . Labs  Leukocytosis,  ESR,  CK,  Troponin I – check cultures, viral titers & cold agglutinins . ECG  ST changes, dysrhythmias,  conduction disturbances . CXR  Often normal  cardiomegaly or pulmonary venous congestion . ECHO  Dilated chambers, hypokinetic wall movements, pericardial effusion . Myocardial Biopsy o Treatment: . CHF  ACEIs  necrosis & inflammation – also address any underlying causes . Dysrhythmias  Digoxin – use cautiously as effects may be exaggerated by inflamed myocardium . IVIG  May be of benefit . Contraindicated  Immunosuppressives (ex. steroids, cyclosporine, NSAIDs)

. Dilated Cardiomyopathy (DCM) o Dilation & impaired systolic function of one or both ventricles – MC type of cardiomyopathy o Etiology: . MCC  CAD w/prior MI . Infectious  Coxsackie B, HIV, chagas, lyme disease, rickettsial, acute RF, toxoplasmosis . Toxic  Alcohol, cocaine, doxorubicin, chloroquine, clozapine, heavy metals (Pb, Hg, Cb) . Endocrine  DM, hyper/hypothyroidism, pheochromocytoma, acromegaly . Metabolic  Uremia, hypocalcemia, hypophosphatemia,  thiamine (wet-beriberi),  selenium . Neuromuscular  Duchenne’s, myotonic dystrophy, Friedreich’s ataxia . Collagen  SLE, PAN, dermatomyositis, progressive systemic sclerosis . Other  Uncontrolled tachycardia (persistent A-Fib), peripartum cardiomyopathy,  FHx in 20% o Clinical Presentation: . CHF – dyspnea, rales, peripheral edema, JVD . Arrhythmias – can be fatal causing sudden death . Angina – due to  O2 demands of enlarged ventricles . Emboli – systemic or pulmonary & may present w/neurologic deficits . Auscultation – S3/S4 gallop (stiffened ventricular walls), regurgitant valves (MR, TR), rales o Diagnosis: + . Labs   BNP,  Cr,  LFTs,  HCO3,  Na . ECG  ST & T-wave abnormalities, conduction defects (BBB), arrhythmias (VT, A-Fib) 7 . CXR  Global cardiomegaly & pleural effusion . ECHO  Enlarged chambers, global hypokinesis,  LVEF, MR, TR, mural thrombi . Angiography  To exclude ischemic heart disease in certain cases o Treatment: . Initial  Address underlying cause & treat CHF accordingly . Warfarin  Thromboembolism prophylaxis indications:  A-Fib, previous thromboembolism, documented thrombus, LVEF <30% (controversial) . ICD  Consider if life-threatening dysrhythmias . Surgical  Consider in severe, refractory cases – ex. LVAD, volume reduction, heart transplant . Vaccination  Influenza & S. pneumoniae o Prognosis  20% mortality in 1st year & 10% per year after – often due to CHF, arrhythmias or emboli

. Restrictive Cardiomyopathy (RCM) o Impaired ventricular filling – 2O to myocardial stiffening, fibrosis or  compliance . Usually intact systolic function w/non-dilated & non-hypertrophied ventricle o Etiology: . Cardiac  Endomyocardial fibrosis, Loeffler’s endocarditis, radiation heart disease . Other  Carcinoid syndrome, amyloidosis, sarcoidosis, hemochromatosis, Gaucher’s, Hurler’s o Clinical Presentation: . CHF – commonly present w/dyspnea & exercise intolerance (RHF usually predominates)  Arrhythmias,  JVP, Kussmaul’s sign, thromboembolic events . Auscultation – S3, S4, MR, TR o Diagnosis: . ECG  Low voltage, non-specific ST/T-wave changes, conduction abnormalities (left BBB) . CXR   Enlarged atria & pulmonary venous congestion . ECHO  LAE, RAE, normal sized ventricles, thicken myocardium, MR or TR  May see speckled appearance if amyloid cause . Cardiac Catheterization  Reveals  end-diastolic ventricular pressures . Endomyocardial Biopsy  May detect eosinophilic infiltration or myocardial fibrosis  Can also help distinguish RCM from constrictive pericarditis o Treatment: . Initial  Address underlying cause & treat CHF accordingly . A-Fib  Anticoagulation + HR control . Permanent pacemaker  If complete heart block . Heart transplant  Consider in refractory cases

. Hypertrophic Cardiomyopathy (HCM) o Unexplained ventricular hypertrophy – not caused by systemic HTN or AS . Often involve asymmetric pattern of hypertrophy – MC is septal hypertrophy o Etiology  50% inherited defect in cardiac sarcomeric proteins – auto. dominant w/variable penetrance o Pathophysiology: . Hypertrophic Obstructive Cardiomyopathy (HOCM)  Dynamic LVOT obstruction  Systolic flow draws anterior leaflet of mitral valve into tract via Bernoulli effect o Causes dynamic left ventricular outflow tract obstruction  Outflow obstruction can cause – left atrial dilatation, A-Fib, CHF, RHF   LVEDV =  Obstruction   Blood volume, negative inotropes,  peripheral resistance . Diastolic dysfunction  Impaired ventricular filling – 2O to LVH which reduces compliance o Clinical Presentation: . Asymptomatic  Screening important . Angina  At rest or during exercise – usually unresponsive to NTG  May respond to recumbent position – pathognomonic (but rare) . Syncope  After exertion or Valsalva maneuver . CHF  Dyspnea, rales, peripheral edema, JVD . Arrhythmias  A-Fib or VT – sudden death in HCM usually due to arrhythmia . Auscultation:  Harsh systolic ejection murmur at LLSB w/S4  2O to LVOT obstruction o Enhanced by  LVEDV  Standing position or Valsalva maneuver

8 . HCM is one of few murmurs that diminishes w/squatting ( LVEDV)  Paradoxical splitting of S2  Pulmonic valve closes before aortic valve o Inspiration will delay pulmonic closure & cause paradoxical narrowing of split   Pansystolic murmur due to MR . Pulses  Rapid upstroke & bifid carotid pulse . Precordial palpation  PMI localized & triple apical impulse o Diagnosis: . ECG  LVH, PVCs, A-Fib, prominent Q-waves, high voltages across precordium . ECHO  Septal hypertrophy, LVH, systolic anterior motion of mitral valve, MR . Cardiac Catheterization  Preform if considering invasive therapy o Treatment: . Preventative  Avoid factors that  obstruction – esp. volume depletion & strenuous exertion . Pharmacologic management:  -blockers  Disopyramide   HR,  LV filling time,  inotropy  CCBs (Verapamil)  2nd line – only in pts. without resting or provocable obstruction  Avoid  ACEI, Nitrates, Diuretics –  preload will worsen obstruction & symptoms . Refractory cases  Septal myectomy or Septal ethanol ablation or Dual chamber pacing . Ventricular arrhythmias  Amiodarone or ICD . Screening  1st degree relatives of HCM pts. screened via physical, ECG & 2D-ECHO  Screen annually during adolescence – then serially every 5yrs o Complications: . MCC of sudden cardiac death (SCD) in young athletes  Factors ass. w/ risk of SCD: o Syncope or non-sustained VT o Marked ventricular hypertrophy – max. wall thickness ≥30mm o Abnormal BP response to exercise in young pts.

PERICARDIAL DISEASE

. Pericarditis o Etiology: . Idiopathic  Usually presumed to be viral – MCC is Coxsackie virus . Infectious  Coxsackie, Echovirus, S. pneumoniae, S. aureus, TB, Histoplasmosis, Blastomycosis . Acute post-MI  Direct extension of myocardial inflammation seen after 1-7 days . Dressler’s syndrome  Autoimmune response to infarcted myocardium seen after 2-8wks . Trauma  Post-cardiac surgery (ex. CABG) . Metabolic  Uremia (common), hypothyroidism . Malignancy  Hodgkin’s, breast, lung, renal cell carcinoma, melanoma . Collagen  SLE, polyarteritis, RA, scleroderma . Drugs  Hydralazine, Procainamide, Isoniazid . Other  Radiation, dissecting aneurysm o Clinical Presentation: . Recent viral URI  fever . Pleuritic chest pain – alleviated by sitting up & leaning forward  Does not respond to NTG – pain aggravated by lying supine, coughing, swallowing . Auscultation – pericardial friction rub on expiration is pathognomonic (but variably present) o Diagnosis: . ECG  Diffuse ST elevations, PR depressions (specific), low voltage . CXR  Normal sized heart & pulmonary infiltrates . ECHO  To assess for pericardial effusion . Diagnostic triad  1) Chest pain, 2) Friction rub, 3) ECG changes o Treatment: . Self-limited  Most cases resolve in 2-6wks . NSAIDs  Mainstay of therapy – to relieve pain &  inflammation . Steroids  For intractable cases (ex. Dressler’s) o Complications  Recurrence, atrial arrhythmia, pericardial effusion, tamponade, constrictive pericarditis

9

. Pericardial Effusion o Etiology: . Transudative (serous)  CHF, hypoalbuminemia/hypoproteinemia, hypothyroidism . Exudative (serosanguinous or bloody)  Causes similar to acute pericarditis  May develop acute effusion 2O to hemopericardium – trauma, post-MI rupture, dissection o Clinical Presentation: .  Asymptomatic – can also present similar to acute pericarditis . Dyspnea, cough,  JVP w/dominant ‘x’ descent,  pulse pressure .  Esophageal/recurrent laryngeal/tracheo-bronchial/phrenic nerve irritation . Auscultation – distant heart sounds  rub o Diagnosis: . ECHO  Procedure of choice – shows fluid in pericardial sac . ECG  Low voltage & flat T-waves . CXR  Cardiomegaly, rounded cardiac contour (“water bottle” appearance) . Pericardiocentesis  Identify transudate vs. exudate, infectious agents, neoplastic involvement o Treatment: . Mild effusion  Frequent observation w/serial ECHOs + Anti-inflammatory agents . Severe effusion  Pericardiocentesis – may develop cardiac tamponade

. Pericardial Tamponade o Due to rapid accumulation of fluid in pericardial sac – impairs cardiac filling leading to  CO o Etiology  Pericarditis, trauma, post-MI rupture, aortic dissection w/rupture, malignancy, uremia o Clinical Presentation: . Beck’s Triad – 1) Hypotension, 2) Muffled heart sounds, 3) JVD . Other – dyspnea, tachypnea, tachycardia, peripheral edema . Pulsus Paradoxus   sBP by >10 mmHg w/inspiration – due to  stroke volume  Transiently enlarged RA bulges leftward causing  LV volume & output . Auscultation – distant heart sounds o Diagnosis: . ECG  Electrical alternans – pathognomonic variation in R-wave amplitude . ECHO  Pericardial effusion & compression of cardiac chambers (RA & RV) in diastole o Treatment: . Pericardiocentesis  Preform immediately if unstable .  Fluids  To expand volume &  CO – avoid diuretics & vasodilators . Pericardiotomy  Pericardial window created to allow drainage

. Constrictive Pericarditis o Chronic pericarditis resulting in fibrosed, thickened, adherent or calcified pericardium o Etiology  Any cause of acute pericarditis may result in chronic pericarditis . Idiopathic, viral, TB, radiation (worst prognosis), post-cardiac surgery, uremia, MI o Clinical Presentation: . Dyspnea, tachycardia, palpitations, JVD, normal BP, absent pulsus paradoxus . Kussmaul’s sign – failure of jugular venous pressure to fall during inspiration . May mimic CHF (esp. RHF) – ascites, hepatosplenomegaly, edema . Auscultation – distant heart sounds . Precordial –  pericardial knock (early diastolic sound) o Diagnosis: . ECG  Low voltage, T-wave flattening or inversion, notched P-waves . CXR  Pericardial calcification & effusions . ECHO/CT/MRI  Pericardial thickening . Cardiac Catheterization  Equalization of end-diastolic chamber pressures – diagnostic o Treatment  Diuretics + Na+ restriction – if unresponsive consider Pericardiectomy

10 VALVULAR HEART DISEASE (VHD)

. Mitral Stenosis (MS) o Etiology  MCC is RHD – MC in females & congenital MS rare cause o Clinical Presentation: . DOE, orthopnea, fatigue, palpitations, PND . Pulmonary HTN – peripheral edema, cough,  hemoptysis . Dysphagia & hoarseness – enlarged LA impinging on esophagus & recurrent laryngeal nerve . A-Fib – dilation of left atrium major cause of A-fib . Systemic emboli – due to blood stagnation in enlarged LA . Absent “a” wave on JVP, sternal lift due to RVH,  pulse pressure, loud . Auscultation  OS after S2 w/mid-diastolic rumble at apex – best heard over LSB  Murmur enhanced by  LA return – esp. expiration  Long murmur & short A2-OS interval correlate w/severe MS o Diagnosis: . ECG  LAE, A-Fib, RVH, RAD . ECHO  Thickening of MV leaflets . CXR  LAE w/straight left heart border, kerley B-lines (pulmonary effusion), large PA o Treatment: . Diuretics & Na+ restriction . Digitalis  To control ventricular rate in A-Fib . Anticoagulation  If atrial thrombus or A-Fib present . Balloon valvuloplasty  Standard of care – indicated in symptomatic pts. w/orifice ≤1.2cm2  Alternatives – Mitral commissurotomy or Valve replacement

. Mitral Regurgitation (MR) o MR   CO   LV/LA pressure  LV/LA dilatation  CHF + Pulmonary HTN o Etiology: . MCC is mitral valve prolapse (MVP) . Endocarditis, rheumatic fever, LV dilatation/aneurysm, Marfan’s, HOCM . MV annulus calcification or chordae/papillary muscle rupture o Clinical Presentation: . Dyspnea, PND, orthopnea, palpitations, peripheral edema, cough . Auscultation – loud holosystolic murmur at apex w/radiation to axilla &  S3  Murmur enhanced by expiration or  TPR o Diagnosis: . ECG  LAE,  LVH . ECHO  Shows diseased or prolapsed valve . Swan-Ganz Catheterization  Prominent ‘V’ wave due to systolic overload on LA o Treatment: . Asymptomatic  Serial ECHOs . Diuretics  To  preload & improve CO . Vasodilators  To  afterload which favors aortic exit – esp. ACEIs . Surgery  Indicated in acute MR w/CHF or if signs of LV dysfunction  Valve repair  Annuloplasty rings, leaflet repair, chordae transfers/shorten/replacement o Advantages – low rate of endocarditis, no anticoagulation & less reoperation  Valve replacement  If failed repair or heavily calcified annulus

. Mitral Valve Prolapse (MVP) o MV displaced into LA during systole – MC valvular disorder w/90% of cases in women o Pathophysiology: . Myxomatous degeneration of chordae – due to defect in connective tissue proteins  Due to Marfan’s, Ehlers-Danlos, RHD, pectus excavatum o Clinical Presentation: . Asymptomatic  Stabbing chest pain, dyspnea, palpitations, presyncope, systemic emboli . Auscultation  Mid-systolic click followed by late-systolic high-pitched murmur at apex  Murmur enhanced by  venous return – valsalva or squat-to-stand maneuver  Click due to tensing of mitral leaflet -  preload will move click closer to S1 11  Other findings   Wide splitting of S2 or  S3 o Diagnosis: . ECG  Non-specific ST & T-wave changes, paroxysmal SVT, ventricular ectopy . ECHO  Systolic displacement of thickened MV leaflets into LA o Treatment: . Asymptomatic  No treatment . Symptomatic  -blockers & avoidance of stimulants – ex. caffeine . Anticoagulation  If systemic emboli . Endocarditis prophylaxis  If murmur audible or if myxomatous leaflet . Surgical  MV repair favored over replacement if significant MR

. Aortic Stenosis (AS) o AS  Outflow obstruction   EDP  Concentric LVH  LVF o Normal AV area = 3–4cm2  Severe AS = <1cm2 & critical AS = <0.5cm2 o Etiology: . Degenerative calcification – idiopathic & seen in older population . Bicuspid AV – MC congenital valve abnormality & ass. w/early calcific AS by age 60-70yrs . Rheumatic heart disease o Clinical Presentation: . Dyspnea, PND, orthopnea, peripheral edema . Exertional angina & syncope – CO cannot maintain BP causing syncope & angina . Parvus et Tardus – pulses weak compared to heart sounds . Narrow pulse pressure, brachial-radial delay & sustained PMI . Auscultation:  Crescendo-decrescendo SEM radiating to carotid arteries – best over right 2nd interspace o Gallavardin phenomenon – musical quality at apex  S4, late S3 & paradoxical splitting of S2 – delayed AV opening causes closure after PV o Diagnosis: . ECG  LVH & strain, LBBB, LAE . ECHO  Reduced valve area, pressure gradient, LVH,  LV function . CXR  Calcified valve & post-stenotic aortic root dilatation o Treatment: . Asymptomatic  Serial ECHOs – avoid exertion, nitrates, arterial dilators & ACEIs . Surgery  If severe AS or LV dysfunction  Valve replacement – definitive therapy  Valvuloplasty – temporary improvement as rate of restenosis very high

. Aortic Regurgitation (AR) o AR  LV dilatation  SV + sBP + dBP   Wall tension  Pressure overload  LVH o Etiology: . Aortic root  Marfan’s, Ehlers–Danlos, dissecting aneurysm, syphilis, CT disease, HTN  Associated w/cystic medial necrosis . Valvular  Congenital bicuspid AV, Turner’s syndrome, large VSD, RHD, endocarditis . Acute AR  IE, aortic dissection, trauma, failed prosthetic valve o Clinical Presentation: . Asymptomatic  Until late LVF develops – dyspnea, orthopnea, PND . Angina  Low pressure in aortic root causes  diastolic coronary blood flow . Pulses  Wide pulse pressure & bounding/waterhammer pulse  Pulsus Bisferiens – dicrotic pulse w/2 palpable waves in systole  Duroziez sign – diastolic femoral bruit when femoral artery compressed  Hill’s sign – sBP in legs >20 mmHg higher than arms  Quincke’s sign – alternating blushing & blanching of fingernails when pressure applied  De Musset’s sign – bobbing of head w/heartbeat . Precordial  Hyperdynamic apex & laterally displaced PMI due to LVE . Auscultation  Early decrescendo diastolic murmur at LLSB (cusp) or RLSB (aortic root)  Best heard sitting, leaning forward & on full expiration  Soft S1, absent S2 & late S3

12  Austin Flint murmur – low-pitched diastolic rumble heard in severe regurgitation o Due to regurgitated blood striking anterior mitral leaflet – similar sound to MR o Diagnosis: . ECG  LVH & LAE . ECHO  To identify leaflet or aortic root anomalies . CXR  LVH, aortic root dilation . Stress Test  Hypotension w/exercise o Treatment: . Asymptomatic   Afterload w/ACEIs, Nifedipine, Hydralazine – avoid exertion . Surgery  Indicated in severe cases w/LVEF <50%  Valve replacement – only definitive treatment  Bentall procedure – aortic root replacement

. Tricuspid Stenosis (TS) o Etiology  RHD, congenital, carcinoid – rare valvular defect o Clinical Presentation: . Peripheral edema, JVD, palpitations, Kussmaul’s sign,  abdominojugular reflex (AJR) . Auscultation  Rumbling diastolic murmur – best heard over LSB between 4th–5th interspace o Diagnosis: . ECHO  Diagnostic . CXR  RA dilation without pulmonary artery enlargement o Treatment  Diuretics ( preload) or Valve replacement (if severe TS)

. Tricuspid Regurgitation (TR) o Right ventricle dilatation  TR  Further RV dilatation  RHF o Etiology  RHD, IE (IV drugs), RV dilation,  PA pressure (LHF, MS, MR), Ebstein anomaly o Clinical Presentation: . Peripheral edema, JVD, palpitations, Kussmaul’s sign,  AJR . Auscultation  Holosystolic murmur heard best at LLSB – accentuated by inspiration o Diagnosis  ECHO is diagnostic – ECG shows RAE, RVH, A-Fib o Treatment  Diuretics ( preload) + Annuloplasty (repair) + Endocarditis prophylaxis

. Pulmonic Stenosis (PS) o Etiology  Congenital (MCC) or RHD (rare) o Clinical Presentation: . Chest pain, syncope, fatigue, peripheral edema, right-sided S4 . Auscultation  Systolic murmur w/pulmonary ejection click  Best heard at 2nd LICS & accentuated by inspiration o Diagnosis  ECHO is diagnostic – ECG shows RVH o Treatment  Balloon valvuloplasty – if severe symptoms

. Pulmonic Regurgitation (PR) o Etiology  Pulmonary HTN, IE, RHD, Tetrology of Fallot (post-repair) o Clinical Presentation: . Chest pain, syncope, fatigue, peripheral edema . Auscultation  Graham Steell diastolic murmur at 2nd–3rd LICS – accentuated by inspiration o Diagnosis  ECHO is diagnostic – ECG shows RVH o Treatment  Rarely requires treatment – valve replacement if severe

. Infective Endocarditis o Infection of cardiac endothelium, characterized by vegetations involving valve leaflets or walls . Leaflet vegetation  Platelet-fibrin thrombi, WBCs & bacteria . Valve involvement  MV >> AV > TV > PV o Classification: . Acute Bacterial Endocarditis (ABE):  Infection of healthy valves – MCC is S. aureus  Produces metastatic foci & usually fatal if not treated within 6wks . Subacute Bacterial Endocarditis (SBE): 13  Seeding of previously damaged valves (RHD, CHD) – MCC is S. viridans  Does not produce metastatic foci o Etiology: . ABE  S. aureus, GNs . SBE  S. viridans, oral flora, GAS, Enterococci, S. epidermidis . IVDU  S. aureus, Streptococci, Enterococci, Candida  Don’t TRI drugs – ABE often affects tricuspid valve in IV drug users . Prosthetic valves (<20%)  S. aureus, S. epidermidis, GN bacilli, fungi . Nosocomial  Indwelling venous catheters, hemodialysis, CT surgery o Clinical Presentation: . Systemic  Fever, chills, rigors, night sweats, anorexia, weight loss . Cardiac  New-onset regurgitant murmur, dyspnea, chest pain . Vascular:  Petechiae  Multiple nonblanching red macules on upper chest & mucous membranes  Janeway lesions  Multiple hemorrhagic nontender macules on palms & soles  Splinter hemorrhages  Fine linear hemorrhages in middle of nailbed  CNS emboli  Focal neurological signs  Renal emboli  Microscopic hematuria, flank pain  Septic pulmonary emboli . Immune-complex:  Osler’s nodes  Tender raised subcutaneous nodules on fingers & toes  Roth’s spots  Retinal hemorrhages w/pale center – seen on fundoscopy  Glomerulonephritis & arthritis . SBE specific  Splenomegaly & clubbing o Diagnosis: . Duke’s Criteria  2 major or 1 major + 3 minor or 5 minor criteria  Major criteria: o  Blood Cx.  2  cultures 12hrs apart, or 3  taken at least 1hr apart o ECHO  Vegetations pathognomonic – but absence doesn’t R/O endocarditis . Transesophageal ECHO more sensitive  Minor criteria: o Predisposing valve lesion or IVDU o Fever >38°C o Vascular  Arterial emboli, septic pulmonary infarcts, Janeway lesions o Immunologic  Osler’s nodes, Roth’s spots o  Blood cultures – but not meeting major criteria o ECHO suspicious for endocarditis – but not meeting major criteria o Treatment: . Antibiotics  1st line = Cloxacillin + Gentamicin – minimum 4wks   Ampicillin – add if native valve & non-IVDU   Rifampin – add If prosthetic valve . Prophylaxis  For high-risk pts. – ex. VHD or previous H/O endocarditis  Dental/respiratory procedures  Amoxicillin 30-60min prior o Clindamycin – if penicillin-allergic

. Rheumatic Fever o Inflammatory disease due to antibody cross-reactivity following GAS infection o Diagnosis  2 major criteria or 1 major + 2 minor criteria w/recent strep infection . Major criteria:  Arthritis  Migratory, multiple joints, very warm, tender  Pancarditis  Involving pericardium, myocardium, endocardium  Erythema marginatum  Pink macules w/central blanching & nonpruritic  Subcutaneous nodules  Pea-sized, firm, non-tender nodules (often extensor surfaces)  Sydenham’s chorea  Clumsiness, difficulty w/handwriting . Minor criteria:  H/O RF or RHD  ESR or CRP or Leukocytosis  Fever  Prolonged PR interval 14  Polyarthralgia  Recent streptococcal pharyngitis o Treatment: . Penicillin or Erythromycin  For 10 days to eradicate throat carriage of GAS . ASA  For arthritis . Prednisone  If severe carditis . 2O Prophylaxis  Daily Penicillin or Erythromycin  No carditis – 5yrs or until 21yrs old (whichever is longer)  Carditis but no RHD – 10yrs or longer  Carditis w/RHD – minimum 10yrs since last episode (sometimes life long) o Complications: . Acute  Myocarditis, A-Fib, acute MR, pericarditis . Chronic  Rheumatic heart disease (RHD)  Valvular abnormalities due to IC deposition in valve leaflets o Fibrous thickening, adhesion & calcification resulting in stenosis or regurgitation o Valve involvement – MV > AV > TV  Onset usually after 10-20yr latency from acute carditis of rheumatic fever   Risk of IE & thromboembolism

ARRHYTHMIAS

. Alterations in Impulse Formation o Abnormal Automaticity: . Normally only specialized cells of conduction system exhibit automacity & are pacemaking cells  Cells found in SA node, AV node & ventricular conduction system . Cells in myocardium outside conduction system may inappropriately acquire automaticity  Often seen in diseased cells – ex. Post-MI ventricular ischemia . Ectopic generators assume pacemaking control if they depolarize at rate greater than SA node . Automaticity can be influenced by:  Sympathetic & parasympathetic stimulation  Abnormal metabolic conditions – hypoxia, acidosis, hypothermia  Electrolyte abnormalities or drugs (ex. digitalis)  Local ischemia/infarction o Early After-depolarizations: . Due to membrane potential becoming more positive during repolarization . Tachyarrhythmia results from self-maintained depolarizing oscillations of action potential . Basis for degeneration of QT prolongation into Torsades de Pointes o Delayed After-depolarizations: . Occurs after AP has fully repolarized, but before next normal AP – delayed after-depolarization . Seen w/ intracellular Ca2+ (digitalis toxicity, ischemia) or w/catecholamine stimulation

. Alterations in Impulse Conduction o Re-entry Circuits: . Self-sustaining re-entry circuit causes rapid repeated depolarizations in region of myocardium . Infarcted myocardium w/ischemia will consist of non-excitable & partially excitable zones  These zones promote formation of re-entry circuits o Conduction Block: . Ischemia, fibrosis, trauma & drugs can cause transient, permanent, uni- or bi-directional block . MCC is refractory myocardium – cells in refractory period or unexcitable due to fibrosis . If block occurs along conduction system, distal conduction zones can assume pacemaking control . Can lead to bradycardia – but also tachycardia if impaired conduction leads to re-entry o Bypass Tracts: . Normally AV node only conducting tract from atria to ventricles . Development of additional or accessory conducting tracts can bypass AVN  Facilitates premature ventricular activation before normal AVN conduction . Sinus Bradycardia o HR <60 bpm w/normal P-waves & PR intervals o Precipitants   Vagal tone, anti-arrhythmics, vomiting, inferior MI,  ICP, hypothyroidism, hypothermia

15 o Normal variant  Bradycardia may be seen in normal adults – esp. athletes & elderly o Asymptomatic  May have light-headedness or possible syncope o Treatment  Atropine  pacing or pressors for hypotension – only if symptomatic

. Sinus Block, Pause & Arrest o 3 disorders involving SA node – sinus pacemaker fires but impulse fails to depolarize atrial myocardium . Results in no initial P wave & consequently no QRS complex, ST segment or T wave o Sinus (SA) Block  Complete block or failure of sinus node to depolarize atria . Block can last one or more cardiac cycles & is multiple of normal P-P interval o Sinus Pause  Delay in formation of sinus impulse in SA node – causes temporary pause (usually >3 sec) . If pause >3x the normal P-P interval it may be called sinus arrest o Sinus Arrest  Longer delay in formation of sinus impulse in SA node . P-P prolongation not gradual (unlike sinus arrhythmia) & not multiple of P-P (unlike SA block) o Escape beats or rhythm may occur: . Atrial escape  P-waves w/abnormal morphology . Junctional escape  P-waves not seen or follow QRS (retrograde P) – rate 40-60 bpm . Ventricular escape  No P-wave & wide abnormal QRS – slow rate 20-40 bpm

. Sick Sinus Syndrome (SSS) o Sinus node dysfunction  Marked bradycardia, sinus pause/arrest, sinoatrial block o Frequently ass. w/episodes of atrial tachyarrhythmias – “tachy-brady syndrome” o Treatment  Electronic pacemaker – indicated if symptomatic bradycardia . -blocker, CCB, Digoxin  Often initiated after pacemaker insertion for tachycardia

. AV Conduction Blocks o Precipitants  Inferior wall MI, Digitalis toxicity,  vagal tone, conduction system disease o 1st Degree AV Block  Prolonged PR interval >0.20s – benign & no treatment required o 2nd Degree AV Block: . Mobitz Type I (Wenckebach):  Progressive PR prolongation followed by failure of P-wave conduction  AV node is MC site of block – benign & no treatment required . Mobitz Type II:  P-wave suddenly fails to conduct without prior PR prolongation – QRS drops suddenly  Site of block within His-Purkinje system – requires permanent pacemaker o Treat quickly as it can progresses to 3rd degree complete heart block o 3rd Degree AV Block (Complete Heart Block): . Failure of atrial impulse conduction to ventricles – independent atrial & ventricular activity . No relationship between P-waves & QRS complexes – always requires pacemaker implantation

. Premature Complexes o Premature Atrial Complexes (PACs): . Ectopic supraventricular beat originating in atria . ECG  Early P-waves that differ in morphology from normal sinus P-waves  Difference is because PACs originate within atria & not sinus node  Normal QRS complex because conduction below atria is normal . Asymptomatic – but may cause palpitations or give rise to PSVTs . PACs found in >50% of normal adults – Tx. w/β-blockers if symptomatic o Premature Ventricular Complexes (PVCs): . Early beat fires independently from focus in ventricle & spreads to other ventricle . Conduction not through normal pathway – but through slower ventricular muscle  Slower conduction causes wide QRS complexes >120msec w/bizarre morphology  No preceding P-wave seen – it’s hidden within wide QRS complex  Couplet  2 successive PVCs  Bigeminy  Sinus beat followed by PVC  Trigeminy  Sinus beat followed by 2 PVCs . PVCs seen in >50% of men w/Holter monitoring – usually asymptomatic & benign . PVC significant in following situations:  Consecutive (≥3 = VT) or Multiform (varied origin) 16  “R on T phenomenon”  PVC falling on T-wave of previous beat o May precipitate VT or V-Fib – can potentially cause sudden death . β-blockers  May benefit if symptomatic – also order EP study to assess benefit of ICD . Studies shown  risk of death if antiarrhythmic drugs used to suppress PVCs after MI

. Atrial Fibrillation (A-Fib) o Disorganized electrical activity of atrial myocardium causing atrial quiver & rapid ventricular rate o Pathophysiology: . Initiation  Single circuit re-entry or ectopic foci produce atrial tachycardia – 350-600 bpm  Impulses then conduct irregularly across atrial myocardium to give rise to fibrillation . Maintenance  Tachycardia causes atrial structural & EP changes – which further promotes A-Fib  Longer pt. remains in AF the more difficult to convert back to sinus rhythm . AV node blocks most incoming atrial impulses producing ventricular rate <200 bpm  Tachycardia leads to suboptimal cardiac output o Etiology: . CAD, MI, HTN, RHD, pericarditis, pulmonary disease, PE, post-op stress . Sepsis, hyper-/hypothyroidism, pheochromocytoma, sick sinus syndrome . Excessive alcohol – ex. "holiday heart syndrome" o Clinical Presentation: . Fatigue, exertional dyspnea, palpitations, dizziness . Irregularly irregular pulse,  angina,  syncope . Stroke or TIA – 2O to embolizing thrombi due to atrial stasis o Diagnosis: . ECG:  No identifiable P-waves due to tachycardia – chaotic fibrillatory baseline  Irregular RR intervals due to irregular impulse conduction to ventricles – 100-180 bpm  Narrow QRS complexes – unless aberrancy or previous BBB   Ashman phenomenon – wide QRS complexes following long-short RR interval . ECHO  Can identify presence of clot in left atrium . Other  Absent S4 on auscultation & absent “a” wave in JVP due to loss of atrial contraction o Treatment: . Rate control  Nondihydropyridine CCBs or β-blockers – target pulse rate 60-100 bpm  Consider using Digoxin or Amiodarone in pts. w/HF . Cardioversion  Convert back to sinus rhythm – after rate control achieved  Electrical cardioversion indications: o Hemodynamically unstable  Immediate electrical cardioversion o A-Fib <48hr  Usually can cardiovert without anticoagulation o A-Fib >48hr  Risk of embolization during cardioversion rises to 2-5% . Anticoagulate for 3wks before & 4wks after electrical cardioversion  Pharmacologic cardioversion  Only used if electrical conversion fails or unfeasible o Agents – Ibutilide, Procainamide, Flecainide, Sotalol, Amiodarone . Anticoagulation  To prevent embolic CVA – target INR of 2-3  A-Fib >48hrs  Obtain Transesophageal ECHO (TEE) to R/O thrombus in LA o If no thrombus seen  Start IV Heparin & perform cardioversion within 24hrs . TEE avoids need for 3wk wait prior to starting anticoagulation o Anticoagulation for 4wks after cardioversion still required . Chronic A-Fib  CCB or β-blocker (rate control) + Warfarin (chronic anticoagulation) . "Lone" A-Fib  Pts. < 60yrs w/A-Fib in absence of underlying CV risk factors  Do not require anticoagulation due to low risk for embolization

. Atrial Flutter o Rapid, regular atrial depolarization from macro re-entry circuit within atrium – MC site is RA o Pathophysiology: . Irritable automaticity focus in atria fires giving rise to regular atrial contractions – 250-350 bpm . Ventricular rate 30-50% of atrial rate – difference due to long refractory period in AV node  AV node allows only 1 out of every 2 or 3 flutter waves to conduct to ventricles  AV block may be fixed (2:1, 3:1, 4:1) or variable o Etiology  COPD, CAD, MV disease, ASD, CHF, cardiac surgery, PE, pericarditis, thyrotoxicosis 17 o Diagnosis: . ECG  Sawtooth flutter baseline in inferior leads (II, III, aVF)  Narrow QRS after every second or third “tooth” P-wave o Treatment: . Rate control  -blocker, CCB, Digoxin . Cardioversion  Preform immediate electrical cardioversion if unstable  Chemical cardioversion – Sotalol, Amiodarone, Type I antiarrhythmics . Anticoagulation  Same guidelines as A-Fib . Long-term   Catheter radiofrequency (RF) ablation – success rate dependent on site of origin

. Multifocal Atrial Tachycardia (MAT) o Irregular rhythm caused by 3 or more atrial foci w/atrial rate 100-200 bpm – may mimic AF o Etiology  COPD (MCC), hypokalemia, sepsis, theophylline or digitalis toxicity o Diagnosis  ECG – at least 3 distinct P-wave morphologies w/variable PR & RR intervals o Treatment: . Normal LV function  CCBs, β-blockers, Digoxin, Amiodarone, IV Flecainide, IV Propafenone  -blockers may be contraindicated in severe pulmonary disease .  LV function  Digoxin, Diltiazem, Amiodarone . Electrical cardioversion  Ineffective & should not be used

. Paroxysmal Supraventricular Tachycardia (PSVT) o Etiology: . Ischemic heart disease or excessive caffeine/alcohol consumption . Digoxin toxicity – MC arrhythmia is paroxysmal atrial tachycardia w/2:1 block . AV node reentry or AV reciprocating tachycardia (accessory pathway) . Atrial flutter w/rapid ventricular response o Classification: . AV Nodal Reentrant Tachycardia (AVNRT):  Accounts for 60-70% of all PSVTs & often found in absence of structural heart disease  Re-entrant circuit using dual pathways within AV node – fast -fibers & slow -fibers  Usually initiated by premature atrial or ventricular contractions  Sudden onset & offset, fast regular rhythm w/rate between 150-250 bpm  ECG  No discernible P-waves – buried within narrow QRS complexes o Rapid conduction causes impulses to activate atria & ventricles simultaneously . AV Reentrant Tachycardia (AVRT):  Re-entrant loop via accessory pathway between atria & ventricle – conducts retrogradely o Initiated by a premature atrial or ventricular complex  Orthodromic AVRT  Stimulus travels up bypass tract (V to A) & down AV node (A to V) o Accounts for 95% of reentrant tachycardia – associated w/WPW syndrome o ECG – narrow QRS – no delta wave  Antidromic AVRT  Stimulus goes up AV node (V to A) & down bypass tract (A to V) o ECG – wide & abnormal QRS due to ventricular activation via bypass tract o Treatment: . Vagal stimulation  Delays AV conduction & blocks reentry mechanism  Valsalva, carotid sinus massage, breath holding, head immersion in cold water/ice . IV Adenosine  1st line if no response to vagal maneuvers  Works by  SA & AV nodal activity w/short duration of action . CCB, β-blocker, Digoxin  Alternatives if LV function preserved . Electrical cardioversion  If drugs not effective or if unstable – almost always successful . Long-term prevention:  1st line  Digoxin (preferred), -blocker, CCB  2nd line  Anti-arrhythmics – Flecainide or Propafenone  3rd line  Radiofrequency catheter ablation of either AV node or accessory tract

. Wolff-Parkinson-White Syndrome (WPW) o Accessory conduction tract from atria to ventricles causing premature ventricular excitation 18 . Bundle of Kent – abnormal fast conducting fibers that bypass AV node – found in either LA or RA o Etiology  Congenital defect seen in 1.5/1000 of general population o Can lead to paroxysmal tachycardia via 2 possible mechanisms: . Orthodromic AVRT:  Impulse travels through AV node (anterograde) & depolarizes ventricles  Then travels back through accessory pathway (retrograde) & re-depolarizes atria  Absent delta waves because conduction occurs retrograde over accessory pathway . SVT (A-Fib or Atrial flutter):  Accessory pathway allows all or most impulses to pass causing  ventricular rate o Normally, AV node allows only certain impulses to ventricles o Diagnosis: . ECG  Wide QRS complex w/slurred upstroke (delta wave) & PR interval <120 msec   Tachyarrhythmias – most often AVRT or AF o Treatment: . Electrical cardioversion  If A-Fib w/rapid ventricular response . Surgical  Radiofrequency catheter ablation of bypass tract – effective treatment . Pharmacologic  Amiodarone, Flecanide, Procainamide, Propafenone, Sotalol . -blockers, CCBs, Digoxin  Contraindicated due to conduction blockage at AV node  Allows unopposed conduction down accessory bypass tract

. Torsades de Pointes o Variant of polymorphic VT occurring in pts. w/baseline QT prolongation – ventricular rate 150-300 bpm o Etiology: . Congenital long QT syndromes . Drugs  Class IA (Quinidine), Class III (Sotalol), Phenothiazines (TCA), Erythromycin, Quinolones . Electrolytes  Hypokalemia or hypomagnesemia o Diagnosis  ECG – QRS complexes “rotate around baseline” changing their axis & amplitude o Treatment  IV Magnesium, temporary pacing, Isoproterenol & correct underlying cause of prolonged QT

. Ventricular Tachycardia (VT) o Rapid & repetitive firing of 3 or more consecutive PVCs – originates below the bundle of His o Etiology: . CAD w/prior MI (MCC), active ischemia, hypotension, cardiomyopathies . Prolonged QT syndrome, drug toxicity, congenital defects o Classification: . Sustained VT  Lasts >30secs – almost always symptomatic  Life-threatening arrhythmia – can progress to V-fib if untreated  Often ass. w/hemodynamic compromise or development of myocardial ischemia . Nonsustained VT  Brief, self-limited runs of VT – usually asymptomatic   Risk for sudden death if CAD & LV dysfunction present o Clinical Presentation: . Palpitations, dyspnea, light-headedness, angina, impaired consciousness,  syncope o Diagnosis: . ECG  Wide QRS tachycardia – may be monomorphic or polymorphic  Monomorphic VT  MC type – identical complexes w/uniform morphology  Polymorphic VT  Constantly changing morphology, amplitude & polarity  Other  AV dissociation – sinus P-waves continue their cycle unaffected by tachycardia o Treatment: . Sustained VT:  Hemodynamically stable  IV Amiodarone, IV Procainamide, IV Sotalol  Hemodynamic compromise  Immediate electrical cardioversion o Follow w/IV Amiodarone to maintain sinus rhythm . Nonsustained VT  Do not treat if asymptomatic w/no underlying heart disease

. Ventricular Fibrillation (V-Fib) o Multiple foci in ventricles fire rapidly – leads to chaotic quivering of ventricles & no cardiac output 19 . Most episodes begin w/VT – except in setting of acute ischemia/infarction o Etiology: . MCC – Ischemic heart disease . Prolonged QT – torsades de pointes, anti-arrhythmic drugs . WPW w/A-Fib + rapid ventricular response o Clinical Presentation: . Loss of consciousness if V-Fib >5-6 secs – if untreated can lead to sudden cardiac death . BP immeasurable, absent heart sounds & pulse o Diagnosis  ECG – QRS & P-waves both unidentifiable o Treatment: . Medical emergency  Immediate defibrillation & CPR  Unsynchronized DC cardioversion – up to 3 sequential shocks to establish rhythm . If V-Fib persists  IV Epinephrine – 1mg bolus initially & then every 3-5 mins  Causes  myocardial & cerebral blood flow – also reduces defibrillation threshold  Attempt to defibrillate again 30-60secs after first epinephrine dose . Refractory V-Fib  IV Amiodarone followed by shock  2nd line – Lidocaine, Bretylium, Magnesium, Procainamide . Chronic therapy  ICD is mainstay – alternative is long-term Amiodarone o Prognosis: . V-Fib not ass. w/acute MI   Recurrence rate – up to 30% within first year  Require chronic therapy – ICD or prophylactic Amiodarone . V-Fib within 48hrs of acute MI  Low recurrence rate – 2% within first year

VASCULAR DISEASE

. Hypertension (HTN) o Classification: . HTN  Pre-HTN: ≥120/89, Stage 1: ≥140/90, Stage 2: ≥160/100 . Isolated Systolic HTN  sBP ≥140 & dBP <90 – often develops in 5th decade  Associated w/progressive reduction in vascular compliance . Hypertensive Urgency  sBP >210 or dBP >120 w/absent end-organ damage . Hypertensive Emergency  sBP >220 or dBP >120 + acute end-organ damage  Papilledema, retinal hemorrhages, bulging discs, mental status changes,  Cr o Etiology: . 1O (Essential) HTN  MCC of HTN (>90%) – unknown cause . 2O HTN:  Renal – renal failure, glomerulonephritis, renal artery stenosis  Endocrine – cushing’s syndrome, 1O hyperaldosteronism, pheochromocytoma  Other – coarctation of the aorta or drug-induced (cocaine, amphetamines) o Risk Factors   FHx, obesity, smoking, DM, high Na+/fat diet, alcohol, black race, male gender o Clinical Presentation  Asymptomatic  light-headed, occipital headache, epistaxis, hematuria, vision o Diagnosis: . Fundoscopic  Check for AV nicking, hemorrhage or papilledema . Auscultation  Check for renal artery bruits . ECG  May show LVH or left ventricular strain o Treatment: . Lifestyle modification  Na+ restriction, low-fat diet, weight loss, exercise, smoking cessation . Pharmacologic  Thiazides (preferred), ACEIs, CCBs, -blockers . Hypertensive Emergency   MAP by 25% in 1-2hrs – gradually reduced BP back to normal  Severe HTN  IV agents – Nitroprusside, Labetalol, Nitroglycerin  No immediate danger  Oral agents – Captopril, Clonidine, Labetalol, Diazoxide . Hypertensive Urgency   BP within 24hrs via oral agents

. Aortic Dissection o Tear in aortic intima allowing blood to dissect into aortic media . Acute dissection < 2wks – initial mortality 1% per hour 20 . Chronic dissection >2wks – mortality levels up to 75-80% o Etiology: . Cardiac  HTN (longstanding in 70% of cases), bicuspid AV, coarctation of the aorta, PDA . Other  Marfan’s, Ehlers–Danlos, syphilis, trauma, 3rd trimester pregnancy, cocaine o Classification: . DeBakey:  Type I  Ascending & descending aorta – MC type – 50%  Type II  Ascending aorta only – stops at innominate artery  Type IIIA  Descending thoracic aorta only – proximal to diaphragm  Type IIIB  Type IIIA + abdominal aorta . Stanford:  Type A (Proximal)  Ascending aorta  aortic arch – requires emergent surgery o Mortality rate w/o surgery is 3% per hour for first 24hrs & 80% at 2wks  Type B (Distal)  Descending aorta only – distal to subclavian artery o Clinical Presentation: . HTN (75%)  syncope . Chest pain – sudden onset, tearing or stabbing pain w/radiation to back  Anterior chest pain more common w/proximal dissection (type A)  Interscapular back pain more common w/distal dissection (type B) . Asymmetric BP & pulses between arms – poor prognosis if >30 mmHg difference . Aortic regurgitation w/new diastolic murmur – esp. w/proximal dissection o Diagnosis: . CXR  Widened mediastinum & pleural capping (effusion in lung apices) . TEE  Can visualize AV & thoracic aorta – but not abdominal aorta  Preferred test in acute setting alongside CT due to high sensitivity & specificity  Ideal test for unstable pts – noninvasive & preformed at bedside . CT/MRI  Both highly accurate – MRI not ideal in acute setting due to longer time . Angiography  Invasive – but best test for determining extent of dissection for surgery o Treatment: . Immediate BP control:  IV -blockers  To  HR & diminish force of LV ejection  IV Sodium Nitroprusside  To  sBP below 120mmHg . Surgical  Resection of intimal tear & replacement of affected aorta w/prosthetic graft  High morality rate due to operative or post-op complications . Type A  Requires emergent surgery w/cardiopulmonary bypass   Hypothermic circulation for transverse arch dissections   Valve replacement & coronary re-implantation for aortic root involvement . Type B  Managed medically – 10-20% may require urgent surgery for complications o Complications: . Ischemic syndromes  Due to dissection or occlusion of aortic branches  Coronary (MI), Carotids (stroke, hemiplegia, horner’s), Splanchnic (ischemic gut) . AR  Due to dissection through aortic root w/unseating of AV cusps . Cardiac Tamponade  Due to dissection into pericardium . Rupture  Pleural (dyspnea, hemoptysis) or Peritoneum (hypotension, shock)

. Aortic Aneurysm o Localized dilatation aortic segment w/diameter at least 1.5x that of normal o True aneurysm  Involves all vessel wall layers – intima, media, adventitia o False aneurysm  Disruption of aortic wall or anastomotic site between vessel & graft . Containment of blood by fibrous capsule made of surrounding tissue o Classification: . Thoracic (TAA) – ascending, transverse arch, descending . Thoracoabdominal . Abdominal (AAA) – 90% infrarenal & MC between renal arteries & iliac bifurcation

o Etiology: . Degenerative, atherosclerotic, trauma, vasculitis, bicuspid AV . Marfan’s, Ehlers–Danlos, infectious (syphilis, fungal), mycotic (Salmonella, Staph) 21 o Risk Factors  Smoking, HTN, age >65,  FHx, M>W, CAD o Clinical Presentation: . Asymptomatic (75%) – discovered incidentally due to pain in chest, abdomen, flank or back   Syncope, hypotension, palpable pulsatile abdominal  Airway or esophageal obstruction – hoarseness, hemoptysis, hematemesis . Uncommon presentation:  Partial bowel obstruction or ureteric obstruction w/hydronephrosis  GI bleed – duodenal mucosal hemorrhage, aortoduodenal fistula  Aortocaval fistula or distal embolization (blue toe) o Diagnosis: . U/S  Test of choice to evaluate size & location of aneurysm – 100% sensitive . CT  100% sensitive – but longer time to preform than U/S . AXR  May show calcifications of dilated segment allowing measurement of aortic diameter  But not used to R/O aneurysm – only for quick assessment o Treatment: . Conservative management:   Risk factors  Smoking cessation, HTN/DM/hyperlipidemia control, exercise  U/S  Every 6 months to 3yrs – depending on size & location . Surgical indications:  Risk of rupture ≥ Risk of surgery – size >5.5cm or >2x normal lumen size  Symptomatic, ruptured or mycotic aneurysm  Acute type A dissection or complicated type B dissection  Ascending thoracic aortic aneurysms . Surgical procedures:  Laparotomy w/graft replacement  Endoluminal graft placement under image guidance o Newer procedure w/ mortality,  procedure time &  recovery time o Disadvantages – endoleak rates up to 20-30% & high-cost for device o Complications: . Rupture of AAA  Emergent laparotomy indicated – crossmatch 10 units PRBCs  Triad of 1) Abdominal pain, 2) Hypotension, 3) Palpable pulsatile abdominal mass o No further diagnostic testing needed w/triad of symptoms  CV collapse & syncope – 2O to sudden hemorrhage  Signs suggesting expansion & impending rupture: o Sudden onset of severe pain in back or abdomen & radiating to groin/legs o Grey Turner's sign  Ecchymoses on back & flanks o Cullen's sign  Ecchymoses around umbilicus

. Peripheral Vascular Disease (PVD)  Chronic Arterial Insufficiency o Occlusive atherosclerotic disease of LE w/symptoms related to site of obstruction . MC site of occlusion is superficial femoral artery in Hunter's canal – also popliteal & aortoiliac o Risk Factors  DM, smoking, CAD, hyperlipidemia, HTN, hyperhomocystinemia o Clinical Presentation: . Intermittent claudication  Leg pain reproduced in same location by same walking distance  Pain completely relieved by rest  Femoral or popliteal occlusion causes calf claudication  Aortoiliac occlusion causes buttock, hip & calf claudication . Rest pain  Continuous & typically felt over distal metatarsals  Often prominent at night – awakens pt. from sleep  Pain relieved by hanging foot over side of bed or standing –  perfusion due to gravity  Rest pain suggests severe ischemia & possible progression to gangrene . Pulses  May be diminished or absent . Signs of  perfusion:  Muscular atrophy,  hair growth, thick toenails,  capillary refill,  skin temp.  Pallor of elevation & rubor of dependency – skin color change w/leg elevation  Skin ulceration – MC in toes & 2O to local trauma that does not heal  End-stage disease – tissue infarction & gangrene 22 o Diagnosis: . ABI  Ratio of sBP at ankle to sBP at arm is normally ≥1.0  Claudication = ABI <0.7 & Rest pain = ABI <0.4  False ABI seen w/calcified arteries as vessels not compressible – esp. DM . Pulse volume recordings  Noninvasive assessment of segmental limb perfusion  Pulse wave forms represent volume of blood per heart beat at sequential sites down leg  Large wave form indicates good collateral blood flow . Arteriography  Gold standard for diagnosing & locating site of occlusion  Invasive & only needed if considering surgery/revascularization o Treatment: . Conservative management:  Smoking cessation  Smoking causes vasoconstriction & progression of atherosclerosis  Exercise  Develops collateral circulation & improves exercise tolerance  Atherosclerotic risk factor reduction  Control of hyperlipidemia, HTN, DM, weight  Avoid extremes of temperature – esp. extreme cold  Foot care – esp. in diabetics . Pharmacologic management:  Anti-platelets  ECASA, Clopidogrel – rarely Ticlopidine  Cilostazol  cAMP-phosphodiesterase inhibitor w/anti-platelet & vasodilatory effects  Pain relief  Morphine sulphate – supplemented w/NSAIDs o If opiate analgesia inadequate – consider lumbar sympathectomy . Surgical indications  Claudication interfering w/lifestyle, rest pain, pre-gangrene, gangrene . Surgical procedures:  Endovascular stenting/angioplasty  Arterial bypass grafts – reversed vein graft or synthetic graft (Gor-Tex, Dacron) o Bypass graft sites – aortofemoral, axillofemoral, femoropopliteal, distal arterial  Amputation – if not suitable for revascularization and/or gangrene

. Acute Arterial Occlusion o Acute occlusion/rupture of peripheral artery  MC in LE – femoropopliteal > aortoiliac o Urgent management required  Irreversible ischemia & myonecrosis results >6hrs o Etiology: . Cardiac emboli (MCC – 80%)  A-Fib, post-MI, VHD, endocarditis, atrial myxoma . Arterial emboli  Proximal arterial aneurysm, atheroembolism . Thrombi  Atherosclerotic, trauma, hematological disorders & stasis o Clinical Presentation: . “6 P’s” – but all may not be present:  Pain in LE – progressing within hrs to feeling of cold, numbness & loss of function o Pain absent in 20% of cases due to prompt onset of anesthesia & paralysis  Pallor – within few hrs becomes mottled cyanosis  Paresthesia – light touch (small fibers) lost first, then sensory (large fibers)  Paralysis – loss of power most important because it indicates impending gangrene  Polar – cold  Pulselessness – not reliable o Diagnosis: . Arteriogram  To define site of occlusion . ECG  Check for A-Fib or MI . ECHO  For evaluation of valves, clot or MI o Treatment: . IV Heparin  Immediate bolus w/continuous infusion to maintain PTT >60secs . Embolectomy via Fogarty balloon catheter . Thrombectomy  graft  bypass . Amputation  If irreversible ischemia

. Deep Venous Thrombosis (DVT) o Thrombus formation & subsequent inflammatory response in superficial or deep vein o Thrombi propagate in direction of blood flow – MC in LE originating in calf veins

23 o Most important complications are PE (50% risk w/proximal DVT) & chronic venous insufficiency o Virchow’s Triad: . 1) Endothelial damage  Leads to  inhibition of coagulation & local fibrinolysis  Trauma, major surgery, pelvic procedures . 2) Venous stasis  Immobilization inhibits clearance & dilution of coagulation factors  Prolonged bed rest, long flights, orthopedic casts, CHF . 3) Hypercoagulability  Hereditary states, OCP, malignancy o Clinical Presentation: . Asymptomatic – esp. if superficial venous system patent allowing blood drainage . Classic findings  Seen in 50% – but all have low sensitivity & specificity:  Unilateral LE pain, edema & erythema – improves w/rest or elevation  Homan’s sign – pain w/foot dorsiflexion  Palpable cord – due to thrombosed vein . Phlegmasia alba dolens  White leg w/no ischemia . Phlegmasia cerulea dolens  Cyanotic leg w/severe pain & ischemia . Post-phlebitic (post-thrombotic) syndrome  Pain, edema, hyperpigmentation & skin ulceration  MC complication in up to 2/3 of DVT cases – due to small obstructions still remaining o Diagnosis: . vDuplex U/S  Initial test for DVT – high sensitivity & specificity  Only for detecting proximal thrombi (popliteal, femoral) – not distal (calf vein) thrombi   vDuplex  leg symptoms  Rules in proximal DVT   vDuplex w/o leg symptoms  Does not R/O DVT! . Venography  Best test for diagnosis of distal calf vein thrombi – but invasive & rarely used  Visualize deep & superficial venous systems – assesses patency & valvular competence . Impedance Plethysmography  Noninvasive alternative to doppler U/S  High sensitivity for proximal DVT only – but poor specificity due to  false positive rate . D-dimer  Low specificity – may be elevated in MI, CHF, pneumonia, post-operative state  Must be combined w/Doppler to R/O DVT o Treatment: . Acute anticoagulation  Unfractionated Heparin or LMWH  IV Heparin  Bolus w/continuous infusion for 5-10d – goal is aPTT of 1.5–2.5x control  LMWH (Dalteparin, Enoxaparin)  No lab monitoring, better bioavailability & as effective . Chronic anticoagulation  Warfarin – continue for 3-6 months or more based on risk factors  Start w/Heparin & overlap for 5+ days until therapeutic INR of 2-3 reached . Thrombolytics  Streptokinase, Urokinase, TPA – indicated in massive PE or RHF  Speeds up lysis of clots – but no improvement in survival . IVC Filter  Indications for Greenfield filter placement:  Contraindication to anticoagulation in documented DVT or PE  Failure of adequate anticoagulation as reflected by recurrent DVT or PE  Low pulmonary reserve w/high risk of death from PE . Preventative  Leg elevation, pneumatic compression boots, compression stockings

. Chronic Venous Insufficiency (CVI)  Post-Phlebitic Syndrome o Pathophysiology: . Superficial & deep venous systems connected by perforating veins  Valves allow flow from superficial to deep veins – but not vice versa . Prior DVT is underlying cause in many cases w/2 major effects:  Destruction of valves in deep venous system causing valvular incompetence  Valves in perforator veins also damaged 2O to chronically elevated deep venous pressure o Inhibits normal transmission of blood from superficial to deep system . Ambulatory venous HTN  Interstitial fluid accumulation resulting in edema  Extravasation of plasma proteins & RBCs into subcutaneous tissues o Results in brawny induration & dark pigmentation due to hemosiderin deposits . Eventual  capillary blood flow w/tissue hypoxia – mild trauma may cause ulcer formation  Venous ulcers MC at medial malleolus – overlying incompetent perforator vein o Ulcer formation directly proportional to amount of swelling present o Clinical Presentation: 24 . Pain  Aching or tightness of involved leg – often worse at end of day . Edema  Lower leg & relieved by elevation – opposite is true in arterial insufficiency . Skin  Stasis dermatitis, pruritus, hyperpigmentation, venous ulcers o Diagnosis: . Doppler U/S  Most commonly used imaging technique . Ambulatory Venous Pressure (AVP)  Gold standard – but invasive & rarely done . Plethysmography  Noninvasive o Treatment: . Conservative  Elastic compression stockings, leg elevation, avoid prolonged sitting/standing . Surgery  If conservative measures fail or recurrent/large ulcers  Ligation of perforators – in region of ulcer  Greater saphenous vein stripping  Venous bypass – if short segment obstruction . Ulcers  Wet-to-dry saline dressings, zinc-oxide wraps, antibiotics, debridement, unna boot  Split-thickness skin grafts – if unna venous boot (external compression stocking) fails

. Superficial Thrombophlebitis o Erythema, induration & tenderness along superficial vein – MC in greater saphenous system . MC at sites of IV infusion in upper extremities & usually ass. w/varicose veins in lower extremities o Etiology: . Infectious  Suppurative phlebitis – due to complication of IV cannulation . Inflammatory  Varicose veins, Buerger’s disease, SLE, trauma . Other  Polycythemia, thrombocytosis, occult malignancy (esp. pancreatic) o Clinical Presentation: . Pain w/cord-like swelling along course of involved vein . Induration, erythema & tenderness – corresponds to dilated & often thrombosed veins o Diagnosis  R/O associated DVT w/non-invasive tests o Treatment: . Conservative  Bed rest, limb elevation, heat, compression bandages, ASA . Suppurative thrombophlebitis  IV Antibiotics + I&D of involved vein o Complications  Simultaneous DVT (<20%) or recurrent superficial thrombophlebitis

. Varicose Veins o Distention of tortuous superficial veins resulting from incompetent valves in venous system . Often due to inherited structural valve weakness – important risk factor o MC in greater saphenous system – dilated, tortuous superficial veins along thigh & leg . Also in esophagus, anorectum, scrotum – esophageal varices (GI bleed), hemorrhoids, varicocele o Risk Factors  Age, females, OCP, occupations requiring standing, pregnancy, obesity, pelvic tumors o Clinical Presentation: . Diffuse aching pain & nocturnal cramping – aggravated by prolonged standing . Ulceration, hyperpigmentation & induration . Brodie-Trendelenberg test  Raise leg, compress saphenous vein at thigh & then have pt. stand  If veins fill quickly from top down  Test  for incompetent valves o Treatment: . Conservative management  Elevation of leg or elastic stockings . Surgical  Stripping of long saphenous vein, Sclerotherapy or Endovenous laser therapy (EVLT) o Complications  Recurrent superficial thrombophlebitis, hemorrhage, ulceration, eczema

. Lymphedema o Obstruction of lymphatic drainage resulting in non-pitting edema w/high protein content . Primary  Milroy’s syndrome . Secondary  Filariasis (MCC worldwide), LN removal (MCC in US), malignant infiltration o Clinical Presentation  Non-pitting edema,  limb mobility, discomfort/pain o Treatment  Compression bandages/sleeve, skin hygiene, manual lymph drainage, low-intensity exercise . If untreated, may become resistant to treatment due to subcutaneous fibrosis

25 CONGENITAL HEART DISEASE

 Prenatal Circulation o Circulation before birth: . Placenta  Umbilical vein  Ductus venosus  IVC  RA  FO  LA  LV  Aorta . Fetal lungs bypassed via following fetal shunts:  Shunting of deoxygenated blood: o Ductus Arteriosus  Connection between pulmonary artery & aorta  Shunting of oxygenated blood: o Foramen Ovale (FO)  Connection between RA & LA o Ductus Venosus  Connection between umbilical vein & IVC o Circulation after birth: . With first breath, lungs open & pulmonary resistance  allowing pulmonic blood flow . Systemic circulation becomes high resistance system – causes ductus venosus closure .  Pulmonic flow causes  left atrial pressures – causes foramen ovale closure .  O2 concentration after first breath cause  prostaglandins – causes ductus arteriosus closure

 Congenital Heart Disease (CHD)  Cyanotic vs. Acyanotic o Most critical period of heart development between 3-8wks gestation – most susceptible to insult o Acyanotic CHD: . Left-to-Right shunts  ASD, VSD, PDA  Cause  pulmonary blood flow leading to  pulmonary pressures  If untreated can lead to Eisenmenger's syndrome – reversal of shunt (right-to-left) . Obstruction  Coarctation, aortic stenosis, pulmonary stenosis  Present w/pallor,  urine, cool extremities, poor pulses,  syncope o Cyanotic CHD: . Right-to-Left shunts  TOF or Ebstein’s anomaly . Other  TGA, hypoplastic left heart, TAPV, truncus arteriosus . Hyperoxic Test  To determine if cyanosis is cardiac in origin  Obtain preductal, right radial ABG – repeat ABG after child inspires 100% O2  If PaO2 improves 150mmHg – then cyanosis not cardiac in origin

 Atrial Septal Defect (ASD) o Classification: . Ostium Primum  Presents in early childhood – ass. w/Down’s syndrome  Findings include murmur or fatigue w/exertion . Ostium Secundum  MC type – tends to present in late childhood or early adulthood . Sinus Venosus  Defect located at entry of SVC into RA o Clinical Presentation: . Asymptomatic – easy fatigability, frequent respiratory infections, FTT . Systolic ejection murmur (SEM) – grade 3/6 pulmonic outflow murmur  Wide & fixed split S2 w/right ventricular heave  May also present w/mid-diastolic rumble at LLSB . If ASD remains patent – CHF & pulmonary HTN can develop in adult life o Diagnosis: . ECHO w/color-flow Doppler  Reveals blood flow between atria – diagnostic  Also shows paradoxical ventricular wall motion & dilated RV . ECG  Right axis deviation (RAD), RVH, PR prolongation . CXR  Cardiomegaly &  pulmonary vasculature o Treatment: . Spontaneous closure rate >80%  If ASD diameter <8 mm . Surgical closure  Indicated in CHF or 2:1 ratio of pulmonary to systemic blood flow o Associations  Holt-Oram syndrome, down’s, fetal alcohol syndrome

 Ventricular Septal Defect (VSD) o Clinical Presentation: . Small defects  Asymptomatic at birth & often normal development  Harsh, high-pitched holosystolic murmur over LLSB 26 . Large defects  Dyspnea w/respiratory distress,  URTIs, FTT,  CHF  Soft, blowing holosystolic murmur w/systolic thrill & loud P2  Mid-diastolic apical rumble reflects  flow across mitral valve  Size of VSD inversely related to intensity of murmur o Diagnosis: . ECHO  Diagnostic . ECG  May show LVH, LAH & RVH . CXR  May show  pulmonary vascular, cardiomegaly & CHF o Treatment: . Small defects  Most close spontaneously in first 1-2yrs – monitor via ECHO . Surgical repair indications:  Symptomatic w/failed medical management  Children <1yr w/signs of pulmonary HTN  Older children w/larger VSDs not reducing in size over time o Associations  Apert’s, down’s, fetal alcohol, TORCH, cri du chat, trisomy 13 or 18

 Patent Ductus Arteriosus (PDA) o Failure of ductus arteriosus to close in first few days of life . Leads to left-to-right shunt from descending aorta to left pulmonary artery o Risk Factors  1st trimester rubella infection, prematurity, female gender o Clinical Presentation: . Often asymptomatic – may have apneic or bradycardic spells, poor feeding, accessory muscle use  Large defects – may present w/FTT, recurrent respiratory infections & CHF . Continuous “machinery” murmur at sternal border of 2nd left intercostal space & loud S2 . Wide pulse pressure, bounding pulses, hyperactive precordium o Diagnosis: . ECHO w/color-flow Doppler  Diagnostic . ECG  May show LAE, LVH or RVH . CXR  May show cardiomegaly &  pulmonary vasculature o Treatment: . Indomethacin  Induces closure of PDA  Contraindicated if PDA needed for survival: o Transposition of the great vessels, tetralogy of fallot, hypoplastic left heart . Surgical closure  If indomethacin fails or if child >6–8 months of age

 Coarctation of the Aorta o Narrowing of aorta almost always at level of ductus arteriosus – occurs just below left subclavian artery . Associated bicuspid aortic valve seen >2/3 of pts. o Clinical Presentation: . Asymptomatic – few pts. have HTN in infancy . Classic finding – systolic BP higher in upper extremities (140-145mmHg)  Difference in BP between left & right arm can indicate point of coarctation . Weak/absent pulses in lower extremities & radial-femoral delay . Differential cyanosis –  O2 saturation in left arm & LE (postductal) compared to right arm .  Short systolic murmur w/late peak at apex, left axilla & left back . May also have LE claudication, syncope, epistaxis or headache . Critical coarctation in infancy requires patent PDA for survival  Closure of ductus may cause shock in first few weeks of life o Diagnosis: . ECHO w/color-flow Doppler  Diagnostic . ECG  RVH (infancy) & LVH (late) . CXR  Cardiomegaly & pulmonary congestion  “3” sign due aortic wall indentation at site of coarctation  “Rib notching” due to collateral circulation via intercostal arteries o Treatment: . PGE1  Keeps ductus arteriosus patent – for severe coarctation in infancy . Surgical correction or Balloon angioplasty (controversial) o Associations  Turner’s syndrome (35%), berry aneurysms, aortic dissection 27  Transposition of the Great Vessels o Parallel pulmonary & systemic circulations – aorta connected to RV & pulmonary artery to LV . Incompatible w/life without ASD, VSD or PDA - ass. w/maternal diabetes & rarely DiGeorge’s o Clinical Presentation: . Critical illness & early cyanosis occur immediately after birth . Unresponsive to O2 as ductus arteriosus closes – can cause severe hypoxemia, acidosis & death . Single loud S2 – but no murmur heard if VSD not present . If VSD present – cyanosis not prominent & presents w/CHF after few weeks of life . Reverse differential cyanosis may be seen if LV outflow tract obstruction present – coarctation, AS o Diagnosis  CXR – “egg on a string” egg-shaped heart w/narrow mediastinum & ECG – RAD & RVH o Treatment: . IV PGE1  To maintain or open PDA . Surgical correction  Arterial or atrial switch . Balloon atrial septostomy  To create or enlarge ASD – last resort if above measure fail

 Tetralogy of Fallot o PROV  Pulmonary stenosis, Overriding aorta, RVH & VSD o Early cyanosis results from right-to-left shunt across VSD due to pulmonary stenosis o Risk Factors  Maternal PKU & DiGeorge syndrome o Clinical Presentation: . “Tet” spells  Hypoxia leads to  pulmonary vascular resistance (PVR) &  systemic resistance  Occurs in exertional states (crying, playing) – peak incidence at 2-4 months of age  Squatting ( vascular resistance) often seen during hypoxemic episodes  Hyperpnea – paroxysm of rapid & deep breathing . Cyanosis  Often not present in infancy but develops over first 2 yrs of life   Cyanosis often leads to deep sleep &  murmur intensity . Murmur  Systolic ejection murmur at LSB & single loud S2 – due to RV outflow tract obstruction  RVOTO is progressive resulting in  right-to-left shunt . Hypoxemia  May cause FTT or AMS – if severe seizures or loss of consciousness o Diagnosis: . ECG  RAD & RVH . CXR  “Boot-shaped” heart due to small PA & RVH &  pulmonary vascular markings o Treatment: . Severe pulmonary stenosis  PGE1 to maintain PDA – along w/urgent surgical consultation . Tet-spells  O2, Propranolol, Phenylephrine, Morphine – squatting & fluids also help . Blalock-Taussig shunt  Definitive surgical correction

 Ebstein’s Anomaly o Congenital defect of tricuspid valve – septal & posterior leaflets are malformed & displaced into RV . RA massively enlarged & patent foramen ovale often exists allowing R-to-L shunt . TR & accessory conduction pathways (WPW) often present & ass. w/arrhythmias o Treatment  Surgical – tricuspid valve repair or replacement o Associations  Lithium or Benzodiazepine use in 1st trimester

 Hypoplastic Left Heart Syndrome o Severe hypoplasia of LV – MCC of death from CHD in first month of life o Systemic circulation dependent on ductus patency – closure causes circulatory shock & metabolic acidosis . Treat w/intubation, IV PGE1 & correct metabolic acidosis o Treatment  Surgical correction (50% survival to late childhood) or heart transplant

 Total Anomalous Pulmonary Venous Connection (TAPVC) o Characterized by all pulmonary veins draining into right-sided circulation . No direct oxygenated pulmonary venous return to left atrium o ASD must be present to allow blood to shunt into LA & systemic circulation – treat w/surgical repair

 Truncus Arteriosus o Single great vessel gives rise to aorta, pulmonary & coronary arteries – truncal valve overlies a large VSD o Treatment  Surgical repair within first 6 months of life 28