Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve.

Seventeen patients with Ebstein's malformation of the tricuspid valve have been evaluated. The majority of patients (11/17) presented in the newborn period with cyanosis and cardiomegaly. The remainder (6/17) were referred because of cardiomegaly on chest X-ray and/or a heart murmur heard on routine examination. Three patients have died; one in the newborn period and two suddenly at the age of 11 and 15 years. Thirteen patients have undergone right and left heart cardiac catheterization within the last seven years. Twelve of these 13 patients (92%) had angiocardiographic left ventricular contraction abnormalities. Five patients also had mitral valve prolapse associated with left ventricular dysfunction. Since many patients with Ebstein's anomaly may have significant left ventricular abnormalities, careful and systematic evaluation of the left ventricle is warranted.