BILATERAL EBSTEINS MALFORMATION ASSOCIATED WITH MULTIPLE ORIFICES IN THE ATRIOVENTRICULAR VALVES

Ebstein's malformation involving both atrioventricular valves is a ver y rare entity. We report two cases in which the atrioventricular valve s were also associated with multiple orifices. The first was an adult with known congenital heart disease. The true nature of the valvar mal formation, associated with atrioventricular septal defect, was only di scovered at autopsy. The second case was a neonatal heart specimen ass ociated with hypoplastic left ventricle and aortic atresia. A review o f the literature showed that Ebstein's malformation was rarely associa ted with atrioventricular septal defect. While accessory orifices were common in the left atrioventricular valve, they were rarely found in the right atrioventricular valve. Our cases, therefore, showed a very rare combination of seemingly disparate defects at the level of the at rioventricular junction. (C) 1998 by Elsevier Science Inc.