Heart Murmurs and Valvular Heart Disease

Heart Murmurs/Valvular Heart Disease*

• Most common causes of mitral/aortic valve disorders are congenital defects; other causes include rheumatic heart disease, connective tissue disorders, infection, sensile conditions
• Most common presenting symptoms: dyspnea, fatigue, decreased exercise tolerance
• Other: Cough, rales, paroxysmal nocturnal dyspnea or hemoptysis, hoarseness
• Physical exam - heart murmur +/- palpable thrill  
• Diagnostic Studies
• EKG - not useful for specific diagnosis → shows chamber hypertrophy
• CXR
• Aortic → left sided atrial enlargement, ventricular hypertrophy
• Mitral → atrial enlargement only
• Echo (transesophageal) and cardiac cath → definitive
• Treatment
• Surgical repair or replacement of defective valve
• Good exercise tolerance → diuretics and vasodilators for pulmonary congestion and digoxin or BB for dysrhythmias
• Anticoagulant therapy for thromboemboli prophylaxis
• Antibiotics to prevent endocarditis and recurrent rheumatic fever

Mitral Valve Prolapse Regurgitant flow across mitral valve

Decreased left ventricular volume results in earlier prolapse and click heard earlier in systole, closer to S1 Increased left ventricular volume results in delayed prolapse, click heard later in systole Thin females with minor chest wall deformities Associated: Ehlers-Danlos, Marfan Syndrome, Mitral Regurgitation

1. Mid-systolic click 2. Followed by a mid-to-late systolic murmur (mitral regurgitation) 3. Loud S2 “Click” is caused by prolapse of leaflets into left atrium and tensing of mitral valve apparatus

1. Maneuvers that increase left ventricular end diastolic volume (preload)/worsen: seated/standing → squatting 2. Maneuvers causing delay in prolapse: handgrip, standing from seated position, valsalva

Health Maintenance 1. Leads to mitral regurgitation 2. Predisposed to infective endocarditis 3. ADA no longer recommends prophylactic antibiotics for MVP

Mid-systolic (HAPI) Murmurs

Hypertrophic Cardiomyopathy	Asian descent Elderly (distinct form) Most: autosomal dominant trait	1. Systolic ejection, crescendo- decrescendo harsh murmur 2. Heard best at LSB	1. Radiation: LSB 2. Decreased (better) with: squatting, lying down or straight leg raise; sustained handgrip 3. Worse (aggravated) with: exercise, valsalva, standing -Decreases preload and afterload	1. EKG: nonspecific ST and T wave changes, septal Q waves, LVH 2. Echo (KEY): LVH, asymmetric septal hypertrophy, small left ventricle, diastolic dysfunction 3. Bisferious pulse (carotid pulse with 2 upstrokes)
Aortic Stenosis	Elderly, syncopal episodes Narrows valve opening, impeding the ejection function of the left side of the heart Most common valvular disease in US	1. Crescendo- decrescendo harsh systolic ejection click 2. Paradoxical split S2 3. Best heard on right near base of heart 4. Severe: Brisk upward deflection of carotids -Thready carotid pulse	1. Radiation: carotids 2. Decreased (better) with: squat → stand 3. Worse (aggravated) with: sit, lean forward and squatting	1. EKG: Left ventricular hypertrophy
Pulmonary Stenosis	Congenital disorder Left sided HF: DOE, jugular pulsation, parasternal lift	1. Preceding systolic sound during expiration 2. Systolic murmur 3. Best heard at 2nd-3rd LICS	1. Radiation: Left shoulder 2. Worse (aggravated) with: inspiration	1. EKG: right axis deviation
Innocent (Still’s) Murmur	Most common murmur of childhood (2-7 years of age)	1. ‘Musical’ or ‘vibratory’ Grade I-III early systolic murmur 2. Short, high-pitched 3. Loudest midway between apex and LLSB	1. Decreased (better) with: squat → stand, valsalva, sitting, inspiration   2. Worse (aggravated) with: squatting, supine, fever, anemia, sinus tachycardia

Holosystolic (MTV) Murmurs

Mitral Regurgitation	Associated:  acute rheumatic fever, Marfan Syndrome Retrograde blood flow and volume overload of the left atrium	1. High-pitched, holosystolic murmur 2. Heard best at Apex	1. Radiating to left sternal border (Axilla) 2. Worse with: isometric hand grip 3. Reduced (better) with: valsalva	1. EKG: left axis deviation or LVH
Tricuspid Regurgitation	H/o pulmonary HTN and cor pulmonale	1. Blowing holosystolic murmur 2. Heard best at LLSB 3. NO EJECTION CLICK 4. Opening snap + diastolic murmur 5. S3 may be present 6. Increased JVP 7. Palpable venous pulse in liver	1. Radiation: RSB, 5th ICS 2. Worse with: inspiration 3. Reduced (better) with: valsalva	1. EKG: right axis deviation
Ventricular Septal Defect (VSD)	L-to-R during systole	1. Harsh high-pitched holosystolic murmur 2. Heard best in 3-4th Left-ICS (parasternal line)	1. Radiation: diffuse 2. Worse with: isometric hand grip 3. Reduced (better) with: valsalva

Diastolic (ARMS) Murmurs

Aortic Regurgitation	Younger pt with h/o Marfan’s syndrome or older patient   Volume overloading due to retrograde blood flow into the left ventricle Classic: diastolic murmur at RSB due to backflow of blood across aortic valve	1. High-pitched Blowing diastolic murmur   2. Decrescendo- crescendo (“V”) murmur 3. Heard best at left sternal border	1. Radiation: apex 2. Worse with: sitting up, leaning forward, exhaling & holding	1. Bounding “water hammer” pulse 2. Wide pulse pressure 3. Rumbling sound at apex (Austin Flint Murmur) - retrograde blood across aorta mixes with anterograde blood from left atrium
Mitral Stenosis	Common after rheumatic fever Impedes blood flow between left atrium and ventricle Enlarged left ventricle	1. Loud first heart sound: wide closing excursion of leaflets 2. Prominent P2 of second heart sound (split S2): elevated pulm-art pressures 3. Opening Snap: sudden tensing of leaflets after they’ve completed their opening excursion 4. Low pitched (Mid)-diastolic rumble - increased flow across stenotic MV during atrial contraction 5. Heard best at apex	1. Radiation: none 2. Worse with: left lateral decubitus position (exhale & hold)	1. Hemoptysis, presents in 30-40s Complications 1. Leads to pulmonary hypertension and heart failure → high LA pressures = high pulmonary vasculature and right heart pressures

Congenital Heart Disease

Cyanotic Defects

Tetralogy of Fallot	6-10% R → L shunting	Crescendo- decrescendo holosystolic at LSB 1. Right ventricular hypertrophy 2. Pulmonic Stenosis 3. Overriding aorta 4. VSD 1. Cyanosis 2. Blubbing 3. Increased RV impulse at LLSB 4. Loud S2	1. Radiation: back 2. Reduced with: squatting (increases SVR)	EMERGENCY 1. Polycythemia; tet spells (hypercyanotic) include extreme cyanosis, hyperpnea, and agitation 2. Tet-spells: irritability, tachypnea, cyanosis worse with exertion (feeding or intense crying)
Pulmonary Atresia	1-3% Presentation depends on presence of tricuspid regurgitation TX: Indomethacin	1. Cyanosis with tachypnea at birth 2. Tachypnea without dyspnea 3. Hyperdynamic apical pulse 4. Single S1/S2 5. Sudden onset severe cyanosis and acidosis		EMERGENCY
Hypoplastic Left Heart Syndrome	7-9% More often in males, 25% of cardiac death before age 7	1. Shock 2. Early heart failure 3. Respiratory distress 4. Single S2
Transposition of the great Vessels	5-7% No exchange between right and left circulation TX: Prostaglandins	1. Systolic ejection murmur if associated with VSD or pulmonary stenosis 2. Cyanosis in newborn (MC) 3. Tachypnea without respiratory distress 4. If large, sx of CHF and poor feeding 5. Single loud S2		1. Absent LE pulses if aortic arch obst

Non-Cyanotic Defects

• Mnemonic: All Ventricles Provide Circulation (AVPC)

Atrial Septal Defect	7% of all defects Cause: patent foramen ovale	1. Wide, fixed splitting of S2 2. Systolic ejection murmur at 2nd LCIS 3. Early to mid systolic rumble Signs/SX 1. Failure to thrive 2. Fatigue 3. RV heave
VSD	Most common of all congenital heart defects Outlet VSDs more common in Japanese and Chinese	1. Systolic murmur 2. Symptomatology depends on size of defect - from asymptomatic to CHF	1. Radiation: 3-4th LLSB
PDA	12-15% of significant congenital heart disease; higher in premature infants	1. Continuous (machinery-like) murmur 2. Wide pulse pressure 3. Hyperdynamic apical pulse
Coarctation of the Aorta	TX: PGE to maintain ductus arteriosus	1. Systolic ejection click (may be continuous) 2. Infants - CHF 3. Older children - systolic hypertension or new murmur or underdeveloped lower extremities	1. Radiation: LUSB and left scapular area	1. Differences between arterial pulses and blood pressure in UE/LE is pathognomonic -BP right arm > left 2. Absent femoral pulse

UE = upper extremity

LE = lower extremity