Clinical Observation of Double-Chambered Right Ventricle

ABSTRACT

Most congenital obstructions of right ventricular outflow occur at the infundibular, pulmonary valvular, or pulmonary arterial levels. Less frequently, stenosis is present in the sinus portion of the right ventricle and is caused by an obstructing aberrant muscle bundle or band crossing the chamber below the infundibulum. This abnormality has been called the double chambered right ventricle. We have reviewed our experience with 18 patients with a double chambered right ventricle who had been admitted to the SNUH. 1) Age distribution was from 2 to 36 years old with a mean age of 12.8 years. There were 13 female patients and 5 male patients with a 2.61 female to male ratio. 2) The chief complaints of the patients were dyspnea on exertion(50%), known congenital heart disease(27.8%), and cyanosis(16.7%), in that order. The physical findings were grade III-V/VI systolic murmurs and other findings of associated cardiac anomalies. 3) The electrocardiographic findings were as follow left ventricular hypertrophy(33.3%), right ventricular hypertrophy(22.2%), and biventricular hypertrophy(22.2%), Pressure gradients within the right ventricle were noted in 13 cases(72.2%), and filling defects on right ventricular cineangiography were noted in 14 out of 15 cases(93.3%). The echocardiographic findings were not diagnostic, except for the associated cardiac anomalies. 4) 14 cases were operated with a result of aberrant muscle bundles in the right ventricular cavity. All were removed surgically. 1 case was confirmed by autopsy Other 3 cases(Who had pressure gradients within the right ventricular cavity and filling defects on the right ventricular cineangiogram) showed little symptoms, and there has been a continuous follow up on these patients. 5) The associated cardiac anomalies were as follows 13 cases(72.2%) of ventricular septal defect, 1 case(5.6%) of bicuspid tricuspid valve, 1 case(5.6%) of persistent left sided superior vena cava, and 1 case(5.6%) of aortic regurgitation. There was 3 cases of isolated double chambered right ventricle(16.5%).