title: Ebstein's anomaly: the clinical features and natural history of Ebstein's anomaly of the tricuspid valve.
abstract: 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.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000790:Angiocardiography; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006321:Heart / Q000000981:diagnostic imaging; D006347:Heart Sounds; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D008910:Minnesota