A. Physiological or normal.

The murmur is <3/6 in intensity and diminishes with standing, when venous return is less, indicating a flow murmur. S2 split during inspiration only is physiological. During inspiration, increased venous return and pulmonary flow prolongs right ventricular (RV) ejection. This delays P2.

A. Pulmonary stenosis (PS).

A murmur associated with a thrill indicates that it is pathological and not just due to increased cardiac output. A thrill in the suprasternal notch is pathognomonic of PS. Valvular PS would be associated with an ejection click which diminishes with inspiration. Left parasternal heave would be indicative of RV hypertrophy (RVH).

A. ASD.

The features are suggestive of Holt–Oram syndrome.

B. HOCM.

In HOCM, the left ventricular (LV) outflow obstruction is dynamic and is increased by an increase in LV contractility or a reduction in LV size. Standing, Valsalva maneuver, and amyl nitrite reduce venous return, reduce LV filling, and increase systolic anterior motion of the anterior mitral leaflet (SAM), resulting in increased LV outflow tract (LVOT) obstruction. Squatting kinks leg arteries, raising peripheral resistance and hence an increase in LV volume. This reduces SAM and LV outflow obstruction. Valvular aortic stenosis murmur intensity is flow dependent, and hence reduction in venous return with standing or Valsalva maneuver as well as increase in peripheral resistance with squatting would diminish the murmur. In mitral valve prolapse (MVP), an increase in LV volume would reduce prolapse, and a reduction in LV volume would lengthen the murmur by producing earlier prolapse. MVP is generally associated with a mid-systolic click.

A. Posterior mitral leaflet prolapse.

The systolic click and late systolic murmur indicate MVP. In anterior leaflet prolapse, the mitral regurgitation (MR) jet is directed posteriorly and murmur may be conducted to the axilla. In posterior leaflet prolapse, the jet is anterior wall hugging, along the aortic root, which facilitates its conduction to the aortic area.

B. Moderate or severe aortic stenosis.

This is a classic aortic stenosis murmur with carotid conduction. Soft A2 (first component of S2 indicates significant aortic stenosis). Features of severe aortic stenosis are late-peaking murmur, absent A2, paradoxic splitting of S2 (i.e. split during expiration instead of inspiration), and slow-rising carotid pulse (pulsus parvus et tardus).

A. Patent ductus arteriosus (PDA).

Though the other conditions can produce continuous murmurs, they are not associated with a thrill or machinery character.

D. Pistol shot sounds on femoral arteries.

Pistol shot sounds occur in severe aortic regurgitation (AR). Others are features of aortic coarctation.

C. Advise termination of pregnancy.

The findings are typical of tetralogy of Fallot, and the murmur is due to PS. Murmur of ASD is due to flow and softer and not associated with clubbing or cyanosis unless associated with Eisenmenger's syndrome. Tetralogy of Fallot is associated with extremely high risk, and pregnancy should be terminated.

B. Ejection systolic due to increased flow across the pulmonary valve.

Flow across the defect and the lungs does not produce murmurs. It takes a torrential shunt to produce a mid-diastolic flow murmur across the tricuspid valve.

A. Pulmonary valvular stenosis.

In severe PS, due to low pulmonary artery (PA) pressure, an increase in venous return to right ventricle during inspiration may open the pulmonary valve before systole, eliminating the ejection click.

A. Pulmonary hypertension.

This is suggested by loud P2. In pulmonary hypertension, P2 moves closer to A2 due to higher pulmonary valve closure pressure, and S2 may be single when PA pressure approaches systemic pressure. Ejection click and soft ejection systolic murmur may occur because of PA dilation. Other features of pulmonary hypertension may include a palpable PA in second left intercostal space, a palpable P2 or diastolic knock, parasternal heave due to RVH and prominent “a” wave in jugular venous pulsation due to RVH resulting in accentuated right atrial (RA) systole. PS results in a louder murmur and diminished P2. ASD results in wide, fixed, splitting of S2.

E. ASD.

Prominent “a” wave occurs due to forceful RA systole, and this can occur against stenotic tricuspid valve, hypertrophied RV (PS and pulmonary hypertension) or hypertrophied ventricular septum (aortic stenosis, hypertrophic cardiomyopathy, or hypertension). In ASD, defect in the atrial septum would not allow a prominent “a” wave, even in the presence of pulmonary hypertension, as the right atrium would decompress into the left atrium during atrial systole.

A. ASD.

ASD or common atrium as part of Ellis–van Creveld syndrome or mesoectodermal or chondroectodermal dysplasia is an autosomal recessive inheritance disorder that occurs in old-order Amish population. The EVC gene is on chromosome number 4, short arm. It is a form of ciliopathy. Other ciliopathies that result in abnormal organogenesis include Bardet–Biedl syndrome, polycystic kidney and liver disease, Alstrom syndrome, Meckel–Gruber syndrome, and some forms of retinal degeneration, and so on.

A. Holosystolic.

B. PDA with pulmonary hypertension.

In PDA Eisenmenger's, the shunt reversal through PDA causes desaturation in lower part of the body only, resulting in central cyanosis and clubbing in lower extremities and not upper extremities.

B. Severe tricuspid regurgitation (TR).

Liver that is pulsatile (expansile) in systole is indicative of a powerful RA “V” wave, and this suggests severe TR. This can also be seen in the jugular venous pulsation. Sternal compression during the motor vehicle accident likely caused a flail tricuspid valve and severe TR. Liver pulsation in tricuspid stenosis is pre-systolic. Liver pulsations are not seen in constriction or restriction.

A. Mitral stenosis.

This is typical mitral stenosis with pliable leaflets, which causes opening snap. A2-OS interval is a good measure of mitral stenosis severity. Normally, 70–100 ms (same as isovolumetric relaxation time). Less than 70 ms indicates high left atrial pressure, suggesting severe mitral stenosis.

C. Constrictive pericarditis.

Rise in JVP is paradoxical (Kussmaul's sign), and sharp protodiastolic sound is pericardial knock – classic features of constriction. Kussmaul's sign occurs because of lack of transmission of negative intrathoracic pressure to the right atrium through the rigid pericardium. Hence, increased venous return during inspiration causes a rise in RA pressure. In tricuspid stenosis, inspiratory increase in venous return may not readily empty into right ventricle, causing a paradoxical rise in JVP with inspiration. Kussmaul's sign can occur in RV infarct but occurs in the setting of inferior myocardial infarction. Restrictive cardiomyopathy may be associated with S3, which is less sharp (a thud), but no venous paradox.

C. Large pericardial effusion.

This is typical cardiac tamponade with classic paradoxic pulse, hypotension, and tachycardia. Constriction causes venous paradox.

C. Kussmaul's sign.

As pericardium is normal, inspiratory negative intrathoracic pressure is transmitted to cardiac chambers, and hence the venous paradox does not occur. Diastolic knock is palpable loud P2, a feature of pulmonary hypertension that is common in restrictive LV physiology.

A. ASD.

Pulsus paradoxus may not be present when LV filling is not affected by phase of inspiration due to lack of interventricular dependence, as in ASD, or LV filling from other sources, such as severe AR or MR.

D. Aortic stenosis.

D. Congestive heart failure.

See explanation in Box 1.1 Clinical Pearls. This is because though a Valsalva maneuver reduces left-sided venous return, it does not affect LV stroke volume as it is already well filled and beyond the peak of Starling's curve. Increase in intrathoracic pressure is transmitted to the aorta, causing an increase in aortic pressure.

D. Aortic stenosis.

This test is named after South African cardiologist for diagnosis of clubbing. In clubbing, the angle between the nail and nail fold is >165° and when nails of fingers from two sides are apposed, the gap between them disappears. Clubbing is seen in cardiac conditions (in addition to a variety of pulmonary diseases) in subacute bacterial endocarditis, congenital cyanotic heart diseases, and left atrial myxoma.

A. Normal.

Note the mean RA pressure of <5 mmHg and the “a” wave due to atrial systole is slightly higher than the “V” wave, which occurs because of RA filling on the closed tricuspid valve. A smaller “V” wave indicates a compliant right atrium. The sharp “C” wave that coincides with the QRS complex of the electrocardiogram is due to a combination of tricuspid valve closure and transmitted carotid impulse.

E. Tricuspid stenosis.

Note the large “a” wave as the RA contracts against a stenosed tricuspid valve; the gradient is reflected as large “a” wave. Note that the mean RA pressure is slightly elevated as well commensurate with transtricuspid gradient. In conditions causing RVH (pulmonary hypertension, PS, aortic stenosis with septal hypertrophy), the “a” wave may be prominent due to noncompliant RV (RV fourth heart sound), but mean RA pressure may not be high unless there is heart failure.

B. Pericardial constriction.

Note elevated RA pressure with rapid “Y” descent.

D. ASD.

In ASD, the “a” and “V” waves would be of similar height because the defect leads to equilibration of the LA and RA pressures.

F. TR.

Large “V” wave is due to TR filling up RA during systole. When the “V” wave pressure is about 25–30 mmHg, it may become palpable to the examining finger and associated with expansile liver.

B. Pericardial constriction.

The increasing mean RA pressure with inspiration is the venous paradox or Kussmaul's sign. This is because of dissociation between intrathoracic and intrapericardial pressures because of thick pericardium. During inspiration, the intrathoracic pressure drops, increasing venous return to the right atrium, but the intrapericardial and intra-RA pressure does not drop and returning blood increases the pressure further. Kussmaul's sign can occur even in acute pericarditis and RV infarcts, the latter invoking pericardial restraint.

J. PS.

Note a large “a” wave with near-normal mean RA pressure. Contrast this with tricuspid stenosis.

G. Cardiac tamponade.

Note the raised RA pressure with prominent “X” and “Y” troughs.

K. Complete heart block.

The intermittent large waves are “cannon a” waves when atria and ventricles happen to contract simultaneously due to AV dissociation.

A. Normal.

The RA pressure is <5 mmHg and drops with inspiration.

H. Superior vena cava syndrome.

Note the high JVP which does not drop on inspiration as superior vena cava is blocked and jugular vein is not in communication with right atrial hemodynamics or pulsatile changes. In contrast to constriction, the JVP in superior vena cava syndrome is nonpulsatile. It would be high in both.

I. Heart failure.

High JVP that drops with inspiration.

A. Normal.

This is the typical normal tracing. Note the fairly rapid upstroke, pulse pressure of about 40 mmHg, dicrotic notch and dicrotic wave.

C. Aortic stenosis.

Note the very slow rise, attributable to high blood flow velocity across the valve which converts pressure to kinetic energy.

D. AR.

Note rapid upstroke, rapid downstroke (water-hammer pulse), wide pulse pressure, and low diastolic pressure due to peripheral vasodilation. This can also occur in PDA and large AV fistulae.

F. Mixed aortic stenosis and AR.

This is called pulsus bisferiens or double pulse. Can also occur in HOCM.

I. Heart failure.

This is pulsus alternans. Alternating strong and weak pulse with regular RR interval due to alternating stronger and weaker myocardial contraction with every other beat attributable to altered calcium handling by contractile proteins. It is a sign of severe systolic dysfunction.

G. Cardiac tamponade.

BP drop with inspiration is called pulsus paradoxus. An inspiratory drop of >10 mmHg may indicate tamponade.

L. Premature ventricular contraction (PVC).

With appropriate increase in pulse pressure in the post PVC beat due to a combination of increased preload due to long filling period and increased contractility due to force–frequency relationship.

H. HOCM.

In this patient, after PVC, instead of an augmented pulse there is a smaller pulse volume. This is due to the fact that increased contractility in the post PVC beat increases dynamic LVOT obstruction and reduces stroke volume. This is called Brockenbrough phenomenon on cardiac catheterization. This contrasts with valvular aortic stenosis, where pulse pressure increases after a PVC.