Okabe M, Sasaguri M, Ohba H, Kawano T, Nakazima Y, Fukuda K, Hiroki T, Yoshida T, Kikuchi M
Second Department of Internal Medicine, Fukuoka University, School of Medicine.
Kokyu To Junkan. 1990 Dec;38(12):1253-8.
Double outlet right ventricle (DORV) is a rare cardiac malformation especially in adulthood. We report a woman with DORV who survived to the age of 33 years. She had not been seriously limited, before she visited our hospital for cardiac evaluation at the age of 25 years. She was cyanotic, and had clubbing of fingers and toes and facial edema. Holosystolic murmur and diastolic regurgitant murmur were audible along the left sternal border. Chest X-ray showed cardiomegaly and enlarged pulmonary trunks. Electrocardiography showed right axis deviation and biventricular hypertrophy. Laboratory examination revealed polycythemia (Hb: 22.4 g/dl), increased levels of hepatic enzymes due to congestive liver and marked hypoxemia (Pao2: 40 mmHg), Diagnosis of DORV was made with cardioangiography. Surgical repair was not indicated. Thereafter, she experienced recurrent heart failure which progressed gradually. She died probably of ventricular arrhythmia at the age of 33 years. At autopsy, the heart showed dilatation of the right atrium and ventricle, and hypertrophy of the bilateral ventricles. Both the pulmonary artery without stenosis and the aorta originated completely from the right ventricle, which were almost normally positioned with bilateral conus. Large ventricule septal defect was found at the subaortic portion. Patent ductus arteriosus and preductal aortic coarctation co-existed. To our knowledge, few cases with DORV who survived over 30 years have been reported, and they all manifested pulmonary stenosis. The present case is a very rare DORV that survived over 30 years without the complication of pulmonary stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)
