Cardiology — Short Cases

1 Cardiology—Short Cases Chu-Pak Lau, A. John Camm, Hung-Fat Tse, Kai-Hang Yiu, and Kathy Lai-Fun Lee Overview « Usual patients: those that are post-myocardial infarction; and those with valvular disease, artificial heart valves, and implanted pacemakers ¢ Go through the usual general examination (unless stated other- wise) and the routine of checking pulses, blood pressure, jugular venous pressure, and precordial examination. General ¢ Look for malar flush. » Central and peripheral cyanosis » Signs of heart failure * Features of infective endocarditis Pulses » The radial pulse may be used to define the rate and rhythm. Pulse volume is best appreciated at a more proximal pulse, e.g., brachial or the carotids. Be sure to count the rate for at least 30 seconds. * Check peripheral pulses if needed.

2 Problem-Based Medical Case Management Case 1.1 Jugular venous pressure » The position of the jugular venous pulse with respect to surface anatomy is posture dependent but its vertical height is not. * Note loss of “a” wave in atrial fibrillation. » Distinguish between jugular venous pressure and carotid pulse: jugular venous pulse has 2 peaks, is posture dependent, seen but not palpable, can be obliterated, and shows a positive hepato- jugular reflux. Precordium inspection and palpation ¢ Ask for permission to measure blood pressure. « Inspect the chest wall for scars and deformities. « Palpate for the apex beat, parasternal heave, and any thrill. Cardiac auscultation + Position: be sure to examine the patient at 45° to the horizontal, left lateral, sitting, and leaning forward. ) * Use the bell for mitral diastolic murmur, diaphragm for the rest of heart sounds and murmurs. * Auscultate the lung base for fine crackles of heart failure. ¢ Use all available signs to interpret a murmur, e.g., a normal pulse volume makes aortic stenosis unlikely. Conversely, a dis- placed apex usually implies an additional regurgitation lesion rather than pure mitral stenosis. 1.1 Aortic regurgitation Classical signs ¢ Look for features of Marfan syndrome and infective endocarditis. * Collapsing pulse (Corrigan’s sign), de Musset’s sign (“head- nodding”), Quincke’s pulse (marked capillary pulsation) and

Case 1.1 Cardiology 3 Pistol shot murmur of Traube and a to-and-fro murmur of Duroziez (appreciated in the femoral pulse) Wide pulse pressure (> 60 mmHg) Displaced apex Early high-pitch blowing diastolic murmur loudest during expi- ration in sit-up and lean-forward position Austin Flint murmur A systolic flow murmur in aortic area is usual and does not indicate aortic stenosis (in the absence of other signs of aortic stenosis). Causes Rheumatic Congenital bicuspid aortic valve Degenerative Syphilis Causes of a dilated aortic root/aortic aneurysm, e.g., Marfan syndrome, aortic dissection. Seronegative arthritis, e.g., ankylosing spondylitis, Reiter’s syndrome, colitis, and psoriatic arthropathy Important investigations Blood tests: VDRL and blood culture (for suspected infective endocarditis) Chest X-ray: dilated aortic arch and cardiomegaly Echocardiogram: left ventricular size and function, aortic root size, degenerative or bicuspid valve, severity of aortic regurgitation Important differential diagnoses Pulmonary regurgitation: no collapsing pulse, no left ventricu- lar hypertrophy Mitral stenosis: an Austin Flint murmur does not have loud first heart sound nor an opening snap

4 Problem-Based Medical Case Management Case 1.2 Discussion + When is surgery indicated in asymptomatic aortic regurgitation? Progressive increase in left ventricular size (systolic diameter > 5.5 cm), worsening ejection fraction, and impaired exercise capacity. Pitfalls and tips The high-pitch murmur of aortic regurgitation can be missed if the ears are not “tuned”. Suspect if a wide pulse pressure is detected. 1.2 Aortic stenosis Classical signs . Small volume and slow-rising pulse Sustained and thrusting apex (left ventricular hypertrophy) which is usually at normal apex position or only slightly displaced Systolic thrill in aortic area Decreased aortic second heart sound (if calcified and severely stenotic) Ejection systolic murmur at aortic area radiating to both sides of neck Causes Degenerative calcification Rheumatic heart diseases Congenital bicuspid aortic valve

Case 1.3 > Cardiology 5 Important differential diagnoses Aortic sclerosis: normal volume pulse, normal or wide pulse pressure, intact second heart sound, and no left ventricular hypertrophy Hypertrophic cardiomyopathy: jerky pulse, normal second heart sound, and murmur becomes louder on standing Mitral regurgitation: pansystolic murmur best heard at the apex with radiation to the axilla Important investigations ECG: left ventricular hypertrophy with “strain” pattern Chest X-ray: heart size may be normal unless at the late stage; aortic valve calcification Echocardiogram: left ventricular hypertrophy, pressure gradi- ent across aortic valve (significant if > 50 mmHg) and aortic valvular area (critical if < 0.8 cm?) Discussion When is valve replacement indicated? Symptomatic aortic stenosis (chest pain, syncope, and heart failure) carries a grave prognosis, and valve replacement is indi- cated. Left ventricular function can improve dramatically after valve replacement. In an asymptomatic patient, valve replace- ment may be needed for progressive increase in gradient across the aortic valve and significantly reduced valve area. 1.3 Atrial septal defect Classical signs . Jugular venous pressure may be increased if in heart failure. Parasternal heave Fixed wide splitting of second heart sound Loud pulmonary component of second heart sound

6 Problem-Based Medical Case Management Case 1.4 ¢ Ejection systolic murmur at pulmonic area ¢ Mid-diastolic murmur at tricuspid area Important differential diagnoses e Pulmonary stenosis: soft second heart sound ° Pulmonary hypertension (loud second heart sound without fixed splitting) Important investigations * ECG: right bundle branch block (suspect primum defect if there is first degree heart block and left axis deviation). ¢ Chest X-ray: plethoric lung fields with right ventricular enlargement * Echocardiogram: dilated right ventricle, pulmonary hyperten- sion, assess size of defect Discussion * Prophylactic antibiotics not indicated for secundum defect = Consider atrial septal defect closure if the pulmonary : systemic shunt ratio = 2:1 * Atrial septal defect can be closed surgically or by transcatheter means. 1.4 Eisenmenger’s syndrome Classical signs o Stunted growth if it occurs early in childhood (measure body weight and height) e Central cyanosis . mubfiing of fingers (may occur only in toes in patent ductus arteriosus) [ * A large “a” wave in jugular venous pulse

Case 1.5 Cardiology 7 o Left parasternal heave suggesting right ventricular hypertrophy « Loud pulmonic second heart sound « May have early diastolic murmur over pulmonary area (due to secondary pulmonary regurgitation) May have a pansystolic murmur over lower left sternal border (due to secondary tricuspid regurgitation) + Original cardiac defect causing the Eisenmenger’s syndrome may or may not be detectable. Causes * Ventricular septal defect: single or closely split second heart sound because the right and left ventricular pressures are similar + Atrial septal defect: fixed and wide splitting of second heart sound + Patent ductus arteriosus: normally split second heart sound, differential cyanosis and clubbing Discussion « What are the complications of Eisenmenger’s syndrome? Stunted growth, right heart failure, infective endocarditis, arr- thythmia, and polycythaemia. 1.5 Atrial fibrillation Classical signs « Signs of thyrotoxicosis such as hand tremor, goitre, lid lag, and retraction + Trregularly irregular pulse (may be slow if it is controlled) with variable volume * Feel all peripheral pulses (embolic complication) » Absence of “a” wave in jugular venous pulse ¢ Variable loudness of first heart sound

8 Problem-Based Medical Case Management Case 1.5 Causes * Mitral valve disease ¢ Ischaemic heart disease ¢ Thyroid heart disease ¢ Alcohol * Hypertension Important differential diagnoses ¢ Atrial flutter with variable block * Atrial tachycardia with variable block * Frequent multifocal ectopic beats Important investigations ¢ Blood: electrolytes, thyroid function test * ECG: atrial fibrillation waves and irregular QRS cycles. An underlying left ventricular hypertrophy may show ST depres- sion due to strain pattern. ST depression may be also caused by ischaemia or due to digoxin (reversed tick sign). * Echocardiogram: look for valvular abnormalities, increase left atrial size, and left ventricular hypertrophy. Left atrial clots are best visualized with transoesophageal echocardiogram. Discussion * What are the pros and cons of rate versus rhythm control? Rate control is needed for all patients. Drugs for rate control are simple to use and are useful for symptom control, but do not reduce stroke. Rhythm control can theoretically improve heart function, reduce risk of stroke and heart failure, but this class of drugs have significant side effects and is of moderate efficacy. Recent trials suggest that either can be used as a first strategy. Anti-thrombotic treatment especially using anti-coagulation (warfarin and novel oral anti-coagulant) is important in both scenarios. Non-pharmacological measures such as catheter ablation can be useful for rhythm control.

Case 1.6 Cardiology 9 1.6 Mitral regurgitation Classical signs May have ankle and/or sacral oedema Often in sinus rhythm, may be in atrial fibrillation if severe or in association with mitral stenosis Displaced apex May have a systolic thrill Left parasternal heave with severe pulmonary hypertension First heart sound absent, soft, or buried in systolic murmur Second sound heard but is often obscured by the murmur Third heart sound is usual Fourth heart sound in acute regurgitation of recent onset Pansystolic murmur best heard at apex with radiation to axilla Causes Rheumatic Degenerative Mitral valvular prolapse Heart failure with left ventricular dilatation Ischaemic heart disease with papillary muscle dysfunction Infective endocarditis Important differential diagnoses Ventricular septal defect: murmur best heard in left parasternal border and radiate to right side of sternum Tricuspid regurgitation: giant V wave of jugular venous pulse, systolic murmur in left sternal border, and pulsatile liver Important investigations ECG: may show atrial fibrillation, evidence of left ventricular hypertrophy

10 Problem-Based Medical Case Management Case 1.7 * Chest X-ray: increased cardiothoracic ratio, pulmonary congestion * Echocardiogram: to assess left ventricular dilatation and func- tion, severity of regurgitation, and underlying valvular defects * Cardiac catheterization: not indicated for appraising the degree of regurgitation; assess concomitant coronary artery disease in preparation for surgery on mitral valve. Discussion * Mitral valve repair or replacement? Chordae and papillary muscles are important for left ventricular function. Therefore mitral valve repair is preferable to replace- ment if the pathological changes are not too advanced. Long- term anti-coagulation may also be avoided. * Which patients with asymptomatic mitral regurgitation should undergo surgery? Dilated left ventricle (e.g., end systolic diameter = 5.5 cm), worsening ejection fraction Pitfalls and tips * Murmur of mitral valvular prolapse may be systolic and may have atypical radiation. * Mitral regurgitation may be confused with aortic stenosis murmur if the regurgitation is due to ruptured chordae tendinae or a predominant posterior leaflet involvement. 1.7 Mitral stenosis Classical signs * Malar flush with pulmonary hypertension ° May have ankle/sacral oedema * Irregularly irregular pulse if in atrial fibrillation, overall volume is small

Case 1.7 Cardiology 11 Loss of “a” wave if in atrial fibrillation, increased jugular venous pressure in right ventricular failure Parastenal heave with right ventricular hypertrophy/pulmonary hypertension “Tapping”, non-displaced cardiac impulse (“tapping” = palpa- ble first heart sound) Loud first heart sound (+ loud pulmonic second heart sound, opening snap) Mid-diastolic rumbling murmur (+ presystolic accentuation if patient is in sinus rhythm) localized to apex, best heard in left lateral position/after exercise Causes > 95% is rheumatic Infrequently due to congenital causes Important investigations ECG: often shows atrial fibrillation Chest X-ray: pulmonary congestion, enlarged left atrium (“straight” left heart border) Echocardiogram: thickened or calcific mitral valve, measure size of orifice (< 1.5 cm® significant, < 1 cm? severe), pulmo- nary hypertension Cardiac catheterization to define associated coronary artery disease (and for measuring pressure gradient across stenotic mitral valve) Important differential diagnoses Austin Flint murmur: first heart sound soft, no opening snap, presence of severe aortic regurgitation Left atrial myxoma

12 Problem-Based Medical Case Management Case 1.8 Discussion « How to assess clinically the severity of mitral stenosis? Apart from symptoms, the long duration of diastolic murmur, closeness of opening snap to the second heart sound, and the presence of pulmonary hypertension all suggest severe mitral stenosis. * Is the mitral valve pliable clinically? The presence of a loud first heart sound and opening snap sug- gests a less calcific valve. Pitfalls and tips e Signs of infective endocarditis, complications, e.g., stroke « Inslow atrial fibrillation the pulse may appear regular. Appreciate irregularity by feeling the pulse for at least 30 seconds and by auscultation. » Do not mention “a” wave if there is atrial fibrillation. » In case of “silent” or “soft” mitral stenosis murmur, must re- locate the apex after turning patient to left side and auscultation with the bell. Exercise the patient if needed. * Patient with mild mitral stenosis can be in sinus rhythm (and the mitral stenosis murmur can easily be missed). 1.8 Mitral valve prolapse Classical signs ° May have features of heritable disorders of connective tissue, e.g., Marfan syndrome, osteogenesis imperfecta, in which mitral valve prolapse is one of the associated abnormalities. * Mid- or late-systolic click. * Clicks may be followed by a high-pitch late systolic regurgita- tion murmur, best heard at apex with atypical radiation. » Both clicks and murmurs are accentuated by interventions that decrease left ventricular volume, e.g., strain phase of Valsalva manoeuvre and standing.

Case 1.8 Cardiology 13 Cause . Myxomatous degeneration Important differential diagnoses . Other causes of mitral regurgitation Tricuspid regurgitation Aortic stenosis Hypertrophic obstructive cardiomyopathy Important investigations ECG: often normal; there may be non-specific ST depression in inferior leads. Chest X-ray: changes such as heart size and presence of pulmo- nary congestion depend on the severity of regurgitation Echocardiography: left ventricular size, posterior systolic dis- placement of the mitral valve into the left atrium Cardiac catheterization: assess the severity of concomitant coronary artery disease and the severity of mitral regurgitation, when surgical intervention is considered. Discussion What is the importance of detecting mitral valve prolapse? The clinical implications depend on the severity of mitral regur- gitation and the need for antibiotic prophylaxis (if a systolic murmur is detected). Mitral valve prolapse can be associated with atypical chest pain and ventricular arrhythmias. Pitfalls and tips Some patients have a systolic click without any murmur; others may have a murmur without a click.

14 Problem-Based Medical Case Management Case 1.9 1.9 Patent ductus arteriosus Classical signs Usually manifests in female Cyanosis and clubbing of the toes but not fingers if Eisenmenger’s syndrome has developed (termed “differential” cyanosis) Large pulse volume or collapsing pulse Increased pulmonic second heart second if complicated by pul- monary hypertension Continuous machinery murmur at left subclavicular area radiat- ing to the back Causes Congenital: idiopathic or secondary to maternal rubella Isolated or associated with other congenital heart disease Low birth weight Birth at high altitude Important differential diagnoses Coarctation of aorta Ruptured sinus of Valsalva aneurysm into the right heart Coronary arteriovenous fistula Ventricular septal defect and aortic regurgitation Venous hum Pulmonary arteriovenous fistula Important investigations Chest X-ray: pulmonary plethora, prominent proximal pulmo- nary artery and aorta Echocardiogram: dilated right ventricle, demonstration of Doppler flow from aorta to pulmonary artery Cardiac catheterization: passage of contrast from aorta to pul- monary artery

Case 1.10 Cardiology 15 Discussion « Ligation/catheter closure indicated for large shunt (pulmonary vs systemic = 2:1) to prevent Eisenmenger’s syndrome e Even small patent ductus arteriosus may require closure to prevent infective endocarditis. « Inneonatal period, prostaglandin E inhibitor may allow sponta- neous closure of ductus. Pitfalls and tips * Auscultate at non-conventional sites. ¢ Diastolic component may be less prominent and easily missed. 1.10 Tricuspid regurgitation Classical signs * Atrial fibrillation may be present * Giant v waves evident in jugular venous pulse * Parasternal heave if there is right ventricular hypertrophy Pansystolic murmur best heard in lower left sternal border, accentuated during inspiration and reduced during expiration ¢ May have ankle and/or sacral oedema « Pulsatile (expansile) liver ¢ Ascites Causes * Functional—right ventricular enlargement secondary to left ventricular failure * Rheumatic ¢ Infective endocarditis * Pulmonary hypertension (e.g., cor pulmonale and atrial septal defect) * Infarction of right ventricular papillary muscles * Tricuspid valve proplapse

16 Problem-Based Medical Case Management Case 1.10 Carcinoid heart disease Endomyocardial fibrosis Trauma Congenital Important differential diagnoses Mitral regurgitation Ventricular septal defect (tricuspid regurgitation murmur is localized to the left parasternal area, and giant jugular venous v wave and pulsatile liver are present). Important investigations Chest X-ray: increase cardiothoracic ratio Echocardiogram: dilated right ventricle and tricuspid regurgita- tion on Doppler (the velocity of tricuspid regurgitation allows pressure difference across the tricuspid valve to be measured and can be used to determine the pulmonary arterial pressure). Biochemical tests: to assess liver function (may be abnormal with cardiac cirrhosis), tests for carcinoid syndrome, features of hypersplenism Discussion Cardiac cirrhosis may occur with prolonged pressure overload. Ascitis rather than ankle oedema can be predominant. Treatment is medical (diuretics) or surgical (tricuspid valvuloplasty). Pitfalls and tips Examine for pulsating liver if tricuspid regurgitation is suspected. Giant v wave may be missed in case of very high jugular venous pressure. Examine patient in a sit-up posture.

Case 1.11 Cardiology 17 « Tricuspid regurgitation murmur may be soft or ejection systolic in nature. It is important to corroborate with other signs (giant v wave and pulsatile liver). 1.11 Ventricular septal defect Classical signs « As with all congenital heart diseases, measure body weight and height, and look for features of Eisenmenger’s syndrome. « Raised jugular venous pressure if in heart failure + Displaced apex due to left ventricular volume overload « Parasternal heave due to right ventricular dilatation e Systolic thrill + Increased pulmonic second heart sound « Pansystolic murmur at lower left sternal border (can be at upper left sternal border if subaortic defect, often associated with aortic regurgitation). + Large ventricular septal defect may have ejection systolic murmur at pulmonary area and mid-diastolic murmur at apex Causes « Congenital: isolated or in conjunction with other cardiac defects * Anterior myocardial infarction Important differential diagnoses * Mitral regurgitation * Tricuspid regurgitation Important investigations Chest X-ray: large heart, pulmonary plethora « Echocardiogram: increase size of both ventricles, visualize site and size of septal defect, Doppler flow across septal defect

18 Problem-Based Medical Case Management Examination case Discussion * Almost half of all ventricular septal defects detected in child- hood will close spontaneously. ¢ A loud ventricular septal defect murmur can be due to a small defect (Maladie de Roger). * Ventricular septal defect should be surgically repaired if the pulmonary : systemic shunt = 2:1, when rupture is due to myo- cardial infarction, and when associated with aortic regurgitation. Pitfalls and tips ¢ The murmur of ventricular septal defect is generally audible all over the precondium but maximally propagated to the right sternal border. Ventricular septal defect is associated with an anterior infarct, whereas mitral regurgitation due to papillary muscle dysfunction is associated with an inferior infarct. Examination case scenarios i. Examine this man’s precordium. Skip the peripheral signs. Important signs * Displaced apex * Mechanical second heart sound Diagnosis Mechanical aortic valve replacement Questions 1. What is the underlying valvular pathology? » Aortic regurgitation or stenosis. 2. What are the causes of aortic regurgitation? » Rheumatic, degenerative, infective, congenital, aortic dissection.

Examination case Cardiology 19 ii. Examine the precordium of this patient. Important signs « Atrial fibrillation » Cardiac apex not deviated « Tapping apical pulsation « Parasternal heave » Loud first heart sound with opening snap e Mid-diastolic murmur Diagnosis Mitral stenosis Questions 1. Causes of mitral stenosis * > 95% rheumatic, rarely congenital or murmur simulated by atrial myxoma. 2. How to differentiate multiple extrasystoles from atrial fibrillation? « Both give totally irregular pulse. On exercise, multiple extrasystole is usually suppressed, whereas atrial fibrillation remains irregular. iii. This patient presented with dyspnoea, please examine his heart. Important signs « Apex at 6th intercostal space 2 cm lateral to the mid-clavicular line * Gallop rhythm » Soft systolic murmur at apex with no radiation Diagnosis Heart failure

20 Problem-Based Medical Case Management Examination case Questions 1. Name four different causes of heart failure? * Ischaemic, hypertension, valvular, and idiopathic dilated cardiomyopathy. 2. What are different causes for mitral regurgitation? * Functional secondary to heart failure, chronic rheumatic heart disease, mitral valve prolapse, papillary muscle dysfunction, and infective endocarditis. 3. In a patient with heart failure and mitral regurgitation, how do you decide if heart failure is caused by the mitral regurgitation, or mitral regurgitation results from heart failure? * The following are helpful features: — History of long-standing mitral regurgitation. — Pulmonary hypertension suggests mitral regurgitation as the underlying cause of heart failure. * The murmur of mitral regurgitation secondary to heart failure can appear ejection systolic in type.