Hypoplastic right heart complex in a 46-year-old woman.

Abstract

The case is reported of a 46-year-old woman with congenital right ventricular hypoplasia, severe tricuspid stenosis, pulmonary hypoplasia, and an intact ventricular septum. This cardiac malformation is extremely rare in adults. Pulmonary circulation was augmented by bronchial collaterals.The recently designated 'hypoplastic right heart complex' (Khoury et al., I969), consisting of various degrees of right ventricular hypoplasia, congenital tricuspid stenosis, and pulmonary stenosis, is extremely rare beyond childhood (Sackner et al., I96I; Williams, and Lowe, I963). This report is of a woman who survived 46 years with this complex. Case reportThe patient had been cyanotic since birth. She was first evaluated at Duke University Medical Center when she was 20 years old at which time her heart was slightly enlarged but no murmurs were heard. At the age of 24 she developed pelvic thrombophlebitis which resulted in paradoxical emboli and an acute diaphragmatic myocardial infarction. Shortly thereafter she began to have occasional chest pain, but she did not require cardiac medication.Deterioration of cardiac function, in fact, did not begin until six months before death when the chest pain became more frequent and signs of congestive heart failure appeared. A phonocardiogram one month before death disclosed an apical systolic murmur, a widely split second heart sound which moved slightly with respiration, and an early pulmonary ejection murmur.Three weeks later the patient was admitted to hospital with congestive heart failure. Her Radiological examination of the heart showed left ventricular and biatrial enlargement. The pulmonary vasculature was diminished. There was evidence, however, of prominent bronchial circulation.Cardiac catheterization revealed bidirectional shunting across a large atrial septal defect. Mean pressures in the right and left atria were 20 and 2 I mm. Hg, respectively, with a left ventricular pressure of II5/4-22 mm. Hg. The catheter could not be passed through the tricuspid valve. Oxygen Angiocardiography showed sequential opacification of the right atrium, left atrium, left ventricle, and the aorta. The right ventricle and a small pulmonary artery could be detected only on the last films of left ventriculography.After injection of the contrast material the patient suddenly became unresponsive and more cyanotic. Three days later she died in ventricular fibrillation.Necropsy findings The heart weighed 480 g. The great vessels arose normally, but the pulmonary artery was small and the aorta was dilated. An atrial septal defect, 2 cm. in diameter, was present. The ring of the tricuspid valve was hypoplastic, 6-9 cm. in circumference (Fig.). The valvular cusps had apparently never separated, so that a single circumferential leaf was present, leaving a stenotic orifice I cm. in diameter. The right ventricle was small. Though the pulmonary valve was hypoplastic with a ring circumference of 4.1 cm., the valve leaflets appeared normal