ABSTRACT
Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect which consists of an apical displacement of the valvar annulus with a deformity of the septal and posterior leaflets. We report 35 cases studied from 1969 to 1993 (45% males and 55% females). One patient presented a polymalformation syndrome and another had a case of Ebstein's anomaly amongst his siblings. We performed an echocardiography in 31 patients and a catheterization in 15. The mean follow-up was 7.6 years (range: 1 day to 16.1 years). The diagnosis was established during the neonatal period in 66.7%. We found other associated cardiac anomalies in 57.1%, with radiologic cardiomegaly in 80.7%. Upon analysis of the EKGs, the P wave was higher than 0.3 mV in 48.4% and 16.1% showed Wolff-Parkinson-White syndrome. Paroxysmal supraventricular tachycardia developed in 12.9%. Long term cardiotonic treatment was needed in 27.3%. In 24.2% cardiac surgery was performed, including one heart transplantation. The mortality rate was 27.3%. The severity of Ebstein's anomaly is extremely variable, not only in its anatomy, but also in the clinical presentation. The severe neonatal form, the associated cardiac defects and heart failure worsen the prognosis.
