Familial ebstein's anomaly with emphasis on the surgical treatment

Abstract Ebstein's anomaly has been reported in a family affecting a brother and a sister. The rest of the family members, including the nonidentical twin brother of the second patient, were free of symptoms. The clinical and hemodynamic data indicated Ebstein's anomaly in both. This was confirmed on necropsy of the first patient and at operation in the second, in whom a prosthetic valve replacing the tricuspid was inserted at the age of 13 years. This patient also died eight months following operation. Despite this outcome and from the experience of others, it is our belief that certain cases of Ebstein's anomaly may be amenable to surgical treatment. The available surgical procedures and the indications for operation are briefly discussed.