Double outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis.

Abstract

. (1976). Thorax, 31,[588][589][590][591][592][593][594] outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis. We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass. We consider the clinical history and angiocardiographic and surgical findings of this rare anomaly.We stress the difficulties of reconstruction of the outflow tract of the right ventricle, because of the anomalous pathway of the right coronary artery, the posterior situation of the pulmonary artery, and the abnormal anatomy present in the outflow tract of the right ventricle. Double outlet right ventricle (DORV) is a fairly rare cardiac anomaly.A number of papers concerning the anatomy, clinical findings, and surgical approach to the anomaly as well as descriptions of new anatomical types have been published (Kirklin, Harp, and McGoon, 1964;Hallermann et al., 1970;Gomes et al., 1971;Lev et al., 1972;Garcia Sanchez et al., 1973;Van Praagh et al., 1975).One type presents with situs solitus, atrioventricular concordance, and L-position of the aorta associated with pulmonary stenosis and a subaortic ventricular septal defect (VSD). To our knowledge, this malformation has been successfully treated in three patients by Danielson et al. (1972), Lincoln (1972), and Kirklin (quoted by Van Praagh, 1975). We present an additional case successfully treated in our hospital. CASE REPORTA 12-year-old girl, born of a normal pregnancy, was investigated and diagnosed as having double outlet right ventricle in another hospital. She was cyanosed and a heart murmur was discovered at the age of 3 months. She had never had cyanotic spells or congestive heart failure. Lately she had complained of dyspnoea and palpitations on minor exertion.On physical examination she was well developed. Generalized cyanosis was present, and clubbing of the fingers was evident. The jugular pulse was visible at 300, and the right ventricular impulse was palpable. On auscultation of the heart there was a systolic ejection murmur grade IV/VI at the left sternal border with maximum intensity over the pulmonary area. The second heart sound was single and very loud. The blood pressure was 120/75 mmHg. The heart rate was 80 per minute. There were no signs of congestive heart failure. The haematocrit was 55% and peripheral 02 saturation was 86%.The ECG (Fig. 1) showed sinus rhythm. The axis was 1500. Right ventricular hypertrophy and possible left ventricular hypertrophy were present.The chest radiograph (Fig. 2) taken on admission showed the apex of the heart pointing to the left and the gastric air bubble under the left dome of the diaphragm with a normal heart size and decreased pulmonary vascularity.