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. Key words: Double outlet right ventricle/complications/diagnosis/surgery, echocardiography, transesophageal, echocardiography, transthoracic, heart catheterization, heart defects, congenital/pathophysiology/surgery, heart septal defects, ventricular/complications, heart ventricles/abnormalities, ventricular outflow tract obstruction/diagnosis Double-chambered right ventricle (DCRV) is a form of right ventricular (RV) outflow tract obstruction caused by anomalous muscular or fibromuscular bundles that divide the right ventricle into proximal high-pressure and distal low-pressure chambers. 1 These cases most often present in children, and in 80% to 90% of patients DCRV is associated with other congenital anomalies. 2 Rarely, a patient remains asymptomatic while the obstruction progresses gradually, until it presents in adulthood. 3,4 Under these circumstances, the presentation can be difficult to diagnose. We present the cases of 2 adult patients who varied in their symptomatic presentations, in the sites of their anomalous obstructive bundles, and in their associated lesions. Both cases were managed successfully by surgical intervention.
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