Ebstein anomaly, a rare congenital cause of heart failure coexisting with Chiari network

Abstract

Abstract Ebstein’s anomaly is a rare congenital condition that presents with an apical displacement of the tricuspid valve leaflets. Much rarer is its coexistence with Chiari network (CN), and only a very few cases had been documented in the literature. We report a 30-year-old man who presented with a 5-month history of progressively worsening abdominal distension with associated weight loss, easy fatigability, early satiety, and loss of appetite. A chest X-ray revealed cardiomegaly with right ventricular preponderance. A 12-lead electrocardiogram showed atrial fibrillation. Transthoracic echocardiography revealed apical displacement of the tricuspid valve, severe right atrial enlargement, a small right ventricle, atrialization of the right ventricle, and CN. A diagnosis of Ebstein anomaly coexisting with CN in atrial fibrillation was made. To our knowledge, this is the first reported case of Ebstein’s anomaly coexisting with CN in Nigeria.