Surgical management of double-chambered right ventricle in adults

Double-chambered right ventricle is a congenital anomaly in which the right ventricle is divided into 2 portions by anomalous muscle bundles. These cases often present in children, but rarely in adults. We discuss 2 cases of double-chambered right ventricle, in patients aged 42 and 35 years. When cases remain asymptomatic until adulthood, they can present with unusual symptoms that lead to incorrect diagnosis. Our cases represent 2 very different manifestations of double-chambered right ventricle, which differ in presentation, in the site of abnormal obstructive muscle bundles, and in the presence of associated lesions. Both of our patients underwent successful surgical resection of the obstruction. One patient also underwent closure of a ventricular septal defect. We also review the literature on the various mechanisms that have been proposed to account for the complex morphology of the abnormal muscle bundles.