Ebstein's disease

Abstract Four patients with Ebstein's disease are described and an account of the autopsy findings is given in two. Breathlessness on exertion, cyanosis and palpitation were common symptoms. On physical examination there was a praecordial systolic murmur and a well marked triple rhythm at the apex in all four patients. Two patients were dull to percussion to the right of the sternum. Central cyanosis was present at rest in one and during attacks of paroxysmal tachycardia in another. There was electrocardiographic evidence of right auricular hypertrophy in the form of peaked P waves in three and a right bundle branch block pattern in two. On radiologic examination all the patients had an enlarged globular cardiac silhouette mainly due to the abnormal right atrium. Angiocardiography showed a huge right atrium in all cases and early filling of the left atrium in one case subsequently found to have a patent foramen ovale at autopsy. Three of the four patients were investigated by cardiac catheterization and in each case the catheter coiled in a large right atrium. The right atrial pressure was raised in two; only in one was it possible to catheterize the pulmonary artery and in this instance the pressures were normal. At autopsy the abnormalities characteristic of Ebstein's disease were found in both cases. A patent foramen ovale was present in only one of these. The pulmonary vessels were examined histologically in one case and showed changes normal for the age of the patients. There is at present no indication for surgical treatment of this anomaly.