What is Lutembacher’s syndrome?

It is the combination of mitral stenosis and atrial septal defect. The significance of this lesion is the presence of a left-to-right shunt that is increased by worsening severity of mitral stenosis. In addition, the mitral valve gradient and pressure half-time will be inaccurate because of rapid deterioration of the pressure gradient due to left atrial-to-right atrial shunting.

The author thanks Doug Voorhees and Sue Rainguet for preparation of the echocardiography figures, and Jo-Anne Orwig for preparation of the manuscript.

What are the usual physical findings in aortic stenosis?

The hallmark of aortic stenosis is a crescendo-decrescendo systolic murmur. This murmur is heard best at the upper right or left sternal border, radiates to the carotids, and is harsh. The murmur of significant aortic stenosis typically is at least III/VI in intensity; in severe stenosis with left ventricular systolic failure, the murmur may be less intense.

The carotid pulses offer important clues in the diagnosis of aortic stenosis. As the valvular disease progresses, the carotid upstroke becomes slowed, the contour sustained, and the amplitude small. In elderly patients, however, relatively inelastic arteries many make the pulse seem normal even in severe disease.

Palpation of the precordium may demonstrate the findings of left ventricular hypertrophy”a sustained, forceful apical impulse, and a palpable atrial filling wave.

The second heart sound (actually the aortic component of S2) is diminished in intensity. In aortic stenosis caused by a congenitally bicuspid valve, an ejection sound may be heard.

In advanced disease, when systolic dysfunction occurs, the findings of congestive heart failure are present: a displaced apical impulse, rales, jugular venous distension, hepatomegaly, and the like.

What is Lutembacher’s syndrome? Photo Gallery



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