[Ebstein's anomaly in adults].

Eighteen patients (11 women and 7 men) with Ebstein's anomaly recognized in adult age (18-55 years, mean 31.5) were followed in our Department for 3-13 years (mean 7 years). Echocardiographic and cardiac catheterization studies were performed in the majority. Clinical and echocardiographic findings were evaluated in the follow-up period. During the first assessment mild cyanosis was present in 14 cases. Nine patients were in NYHA class I, 6 in NYHA class II, 2 in NYHA class III and 1 in NYHA class IV. Eleven patients complained of palpitations. A Wolff-Parkinson-White (WPW) syndrome was present in 4 cases. At cardiac catheterization, done in 14 patients, a moderate-severe tricuspid regurgitation was found in 7 patients. In 8 cases there was a mild right-to-left shunt at atrial level. During the follow-up period there was 1 sudden death. In all patients with the WPW syndrome, the symptoms were well controlled by medical treatment. Systemic embolism was a major complication in 3 patients (all with patent foramen ovale and right-to-left shunt). During the follow-up period surgery was performed in 3 patients for a worsening of the tricuspid incompetence. Good early and late results were obtained in all 3 patients. The clinical conditions remained stable in the other 14 cases. Seven women out of 11 had 1 or more pregnancies (all together 14 pregnancies). In conclusion, in our experience Ebstein's anomaly diagnosed in adult life is a benign and stable disease, particularly if the patient is asymptomatic; surgical correction must be performed if the patient becomes symptomatic either because of paradoxical embolism or because of worsening of the tricuspid regurgitation.