Surgical intervention in cases of Ebstein's anomaly. Abnormal origin and structure of the tricuspid valve

During the ten-year period 1972-81 four patients, two men and two women, with Ebstein's disease underwent operation at our institution. Their average age was 34. Central cyanosis at rest, clubbing and polycythaemia were the most common clinical features of the patients. Enlarged heart, a small pulmonary arterial arch and transluminal lung fields were seen in chest X-ray. Operative findings were a grossly enlarged right atrium and ventricle, the latter having a segment that was typically atrialized and thin-walled but was contracting synchronously with the true right ventricle, and a wide variation in the leaflets of the tricuspid valve and their origins. The atrial septal defect was small in all cases. Artificial heart valves (1 Cutter-Smeloff, 2 Björk-Shiley, 1 St. Jude) were used in the tricuspid reconstruction in addition to closure of the ASDs. One of the patients died postoperatively, the other three are doing well.