Objective: We reviewed the clinical findings and surgical results of Ebstein's anomaly in adult and adolescent age.
Design: Retrospective analysis on clinical data, surgery and follow up.
Setting: Patients (pts) studied in the Cardiology and Cardiothoracic Surgery Departments of the Santa Marta Hospital, Lisbon.
Patients: All pts in whom Ebstein's anomaly was diagnosed by cardiac catheterization or echocardiography or both, between 1875 and 1989 were included.
Results: There were 8 cases (4 female and 4 male). The age varied from 15 and 53 years. Cyanosis and dyspnoea were the presenting features in 7 pts. One of the youngest pts had asymptomatic murmurs. Late appearance of cyanosis had no relation with prognosis. Three pts had pulmonary tuberculosis. One woman had no deterioration in cardiac function during two pregnancies. Clinical findings were variable and ranged from absent murmur with third and fourth sounds to systolic murmurs of high intensity. The ECG was abnormal in all pts. A case with delta wave and normal PQ interval was noted. On the chest X-ray we found cardiomegaly in all pts. Echocardiograms were recorded in 6 pts. The delay of tricuspid valve closure on mitral closure was not always diagnostic. Uncomplicated catheter studies were done in 6 pts. Two pts had associated cardiac lesions: ventricular septal defect (VSD) with pulmonary hypertension and mild pulmonary valve stenosis. Right ventricular angiographies were diagnostic in all cases. During medical follow up from 1 year to 10 years (mean-4) one death occurred in one pt with VSD. In the remaining 4 cases no deterioration in cardiac function or cyanosis was noted. Three pts were operated. The indications for surgery were: severe heart failure and/or increasing cyanosis. Two pts had tricuspid valve replacement with biological prosthesis. One pts had Danielson's tricuspid annuloplasty. During surgical follow up from 2 to 15 years (mean-7) functional class improved and cyanosis disappeared in 2 pts and it decreased in the other.
Conclusion: In our series of pts with Ebstein's anomaly clinical findings were variable. The indications for surgery were heart failure and cyanosis. The 3 pts operated are alive and have mild symptoms.