Uncommon Variant of Ebstein Anomaly With Tricuspid Stenosis

Abstract

A 27-year-old previously healthy woman with no prior history of exercise limitations, shortness of breath, cyanosis, or arrhythmia became short of breath 10 months after her second successful pregnancy. Her shortness of breath was at rest accompanied by progressive edema, ascites, and later anasarca. An ECG revealed normal sinus rhythm with right atrial enlargement (Figure 1A), and chest x-ray revealed normal lung parenchyma with mildly enlarged cardiac silhouette (Figure 1B). A transthoracic echocardiogram demonstrated reduced left ventricular size with an ejection fraction estimated at 50%, severe right atrial enlargement, and severe right ventricular enlargement with dysfunction and was suspicious for severe displacement of the tricuspid valve leaflets toward the right ventricular apex, suggestive of Ebstein anomaly (EA) (Figure 2). She responded to intravenous diuresis with improvement in her right-sided heart failure. In light of her dilated, poorly functioning right ventricle, she was started on Coumadin. Repeat transthoracic echocardiogram 2 months later demonstrated displacement of the tricuspid valve into …