[The 2-chambered right ventricle with an anomalous muscle bundle]

The clinical picture, course, electrocardiogram and chest X-Ray of 30 patients with the angiocardiographic diagnosis of double-chambered right ventricle, along with the echocardiogram in 18 and surgical results in 26 are analyzed. A ventricular septal defect was associated in most of the patients, that had spontaneous closure in some of them. The clinical picture and serial catheterization in some cases suggested progressive obstruction of the right ventricular outflow tract. In this, as in most of the reported series, the history, physical examination, ECG and roentgenogram were inconclusive for diagnosis of this entity. Two-dimensional echocardiography was the most reliable non invasive method to assess this malformation, even superior to catheterization in some aspects. The angiocardiographic findings are quite distinctive, allowing differentiation of other types of subpulmonary stenosis. In order to achieve a good surgical result is essential a precise diagnosis of this malformation and of the associated anomalies. The long-term prognosis was good as in most of the reported series.