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 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.