Ebstein's anomaly: the clinical features and natural history of Ebstein's anomaly of the tricuspid valve.

The clinical features and natural history of Ebstein's anomaly are analyzed on the basis of experience with 67 consecutive patients who have had a mean follow-up of 12 years. Of the total group, 26 (39%) of the patients remained in functional Class I or II and 41 (61%) progressed at some time into Class III or IV. Death occurred in 14 (21%) of the patients, and these were characterized by one or more of the following features: (1) they were in functional Class III or IV, (2) they had moderate to severe cardiomegaly with a cardiothoracic ratio greater than 0.65, (3) they had cyanosis or an arterial oxygen saturation of less than 90%, and (4) they were infants when the diagnosis was made (10 patients, of whom 5 died). In patients in Class III or IV, a comparison was made between patients treated medically (10) and those treated surgically--either with tricuspid valve replacement (15) or with a modified tricuspid annuloplasty (16). It appears that modified tricuspid annuloplasty is the procedure of choice, because despite an operative mortality of 25% (4 patients), 12 of the 16 patients so treated improved clinically or had a reduction in heart size.