The prognosis in Ebstein's disease of the heart. Long-term follow-up of 22 patients.

A follow-up study of 22 patients with Ebstein's anomaly has been performed. Nine patients died 1–21 years (mean 9) after the initial admission while the 13 patients alive at the end of the observation period had been followed for 5–26 years (mean 15). Clinical, ECG, radiological, and haemodynamic features were analyzed with reference to their prognostic significance. The presence or absence of cyanosis due to right-to-left shunt through an atrial septal defect (ASD) distinguished best between a good and a poor prognosis. Right-sided heart failure and dyspnoea at rest, often associated with palpitations, precordial pains and syncopes, were grave prognostic findings. After the initial signs of heart failure there was a rapid deterioration, death ensuing within a few years. Operation with insertion of a prosthetic valve (and closure of the ASD) should be seriously considered at the appearance of heart failure.