0182: Double-chambered right ventricle with intact interventricular septum in adults

Introduction The double-chambered right ventricle (DCRV) is a rare congenital heart disease in which anomalous muscle bundles divide the right ventricle into two cavities, causing variable degrees of obstruction. Typically, DCRV is diagnosed at childhood or adolescence, and most DCRV patients have associated congenital anomalies, such as ventricular septal defect, pulmonary stenosis, and subaortic stenosis. The aim of this study is to determinate the clinical presentation, the echocardiographic patterns and the outcome of DCRV with intact ventricular septum in 4 adults. Materials and results : They were 3 men and one woman. The mean age was 29 years [17;43]. The clinical manifestations were a class II of NYHA dyspnea in 3 patients and right congestive heart failure was observed in one patient. A systolic ejection murmur was heard on the left parasternal border in all cases. Electrocardiogram revealed atrial fibrillation in 2 cases, incomplete right bundle block in 3 patients and right ventricular hypertrophy in all patients. Transthoracic echocardiography established the diagnosis in all cases. It objectified right cavities enlargement in 2 patients and right ventricular hypertrophy in all patients. Moderate tricuspid regurgitation was found in one patient and it was severe in one other patient. The pressure gradient in the right ventricle was evaluated at 72, 80, 80 and 75 mmHg. DCRV was an isolated lesion in all patients. Cardiac catheterization was performed in all patients; it confirmed echocardiographic findings in them. The 4 patients were referred to surgery. Surgical inspection confirmed echocardiographic and catheterisation data in all subjects. They underwent a resection of the obstructing muscle bundles. The postoperative course was uneventful in all patients with no death. Conclusion DCRV should be suspected in adults when there is a RV outflow tract obstruction with unusual symptoms. Echocardiography is considered as a useful method for the diagnosis of isolated DCRV. Cardiac catheterisation is performed in difficult cases. DCRV should in general be treated surgically, because the obstruction is progressive and ends in heart failure. Operative results and long-term outcomes are usually excellent.