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A 44-year-old woman presented with increasing dyspnea. The patient had Ebstein's anomaly, which had first been diagnosed when she was in her early 20s. She had declined heart transplantation three years before her admission to our hospital. The findings on physical examination included atrial fibrillation, a diffuse anterior chest heave and friction rub, and severe right heart failure. The electrocardiogram showed atrial fibrillation and right bundle-branch block. Her chest x-ray films (Panels A and B) showed a cardiothoracic ratio of 1, splaying of the carina, and cardiac enlargement posterior to the vertebral bodies. On transthoracic echocardiography (Panel C and Video Clip), a small left ventricle was seen next to massively dilated right heart chambers. In addition, malposition of the anterior and septal tricuspid-valve leaflets with "atrialization" of the right ventricle and severe tricuspid regurgitation were found. Initially, the patient had a response to fluid restriction and intravenous furosemide and dobutamine. On the fifth day after her admission, she had a cardiac arrest and died four days later in the intensive care unit. Ebstein's anomaly is an abnormality in the embryonic development of the tricuspid valve, with apical displacement and attachment of the septal, posterior, or (rarely) anterior leaflet to the right ventricle wall.
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