Clinical Study of Twenty-Three Cases of Ebstein's Anomaly of the Tricuspid Valve

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Abstract
A comprehensive clinical study of this anomaly in 13 males and 10 females. Twelve of the 23 cases had neonatal symptoms of signs, usually a murmur and cyanosis. The cyanosis decreased or disappeared with time, but recurred later in life in all cases. The most frequent symptoms after the neonatal period were dyspnea, cyanosis, and excessive fatigue. Eighteen of these cases showed a "triple" or "quadruple" rhythm on auscultation. Roentgen studies regularly showed cardiomegaly, normal to decreased pulmonary vasculature, and a normal-sized left atrium. Cardiac size and contour varied widely. The most frequent ecg findings were incomplete or complete right bundle-branch block with low voltage in the right precordial leads (none had an R wave in V1 exceeding 8 mm in height), but 6 cases showed the type B Wolff-Parkinson-White (W-P-W) pattern. The presence of type B W-P-W in a cyanotic child with congenital heart disease should immediately suggest the diagnosis of Ebstein''s defect. Twenty right heart catheterizations were performed without any mortality or serious complications. Two of these patients underwent a new type of surgical procedure, but neither survived. Physiologic data and studies on 8 postmortem specimens indicate that Ebstein''s anomaly represents a continuum from predominant tricuspid stenosis to predominant tricuspid insufficiency, with some having elements of both, and others approaching normal.