Ebstein's Anomaly Associated with Cardiomyopathy and Pulmonary Hypertension

A patient is described with cardiomyopathy and intracavitary electrocardiographic findings typical of Ebstein's anomaly of the tricuspid valve. The cardiomyopathy was characterized by extreme thickness of the left ventricular wall on angiocardiography, elevated end-diastolic pressures of the ventricles, and prominent "a" waves in the atrial pressure pulses. Moderately severe pulmonary hypertension, which has not previously been described in Ebstein's anomaly, was also attributed to the cardiomyopathy. This case illustrates the increasing number of associations with which Ebstein's anomaly has been reported and emphasizes the importance of considering this diagnosis in planning the cardiac catheterization of patients with congenital heart disease. The necessity of demonstrating coexisting Ebstein's anomaly in patients with congenital heart disease is indicated by the formidable mortality of such patients at surgery.