Sail Sound in Ebstein's Anomaly of the Tricuspid Valve

Ebstein's anomaly represents an anatomic, pathologic, and physiologic spectrum. There have been few hemodynamic correlates for the observed auscultatory events. Multiple components of the first sound and "ejection" sounds are frequently described. Cardiac catheterization, intracardiac sound-pressure studies (Telco), and cineangiograms were performed in three patients with Ebstein's anomaly who had a prominent early systolic sound. The right ventricular pressure pulse was abnormal in all; an initial delta-wave configuration, followed by a more rapid pressure rise, produced a prolonged rise to peak pressure. The right ventricular pressure pulse is not that of a conduction defect alone; rather it suggests that the altered pattern of ventricular contraction and abnormal leaflet placement are contributing factors. The early systolic sound was recorded in the atrialized right ventricle or right ventricle in all. It occurred just after the peak of the c wave in the atrialized right ventricle. In the right ventricle the sound occurred at the point where initial slow delta portion of right ventricular pressure pulse gave rise to rapid upstroke. The early systolic sound most likely occurs when the large, sail-like tricuspid valve reaches the limit of systolic excursion. The sound has been designated as the "sail sound," and may be the most specific auscultatory event in Ebstein's anomaly.