Cyanosis and pulmonary disease in infancy.

ease secondary to congenital heart disease. I woud like to show you two children, 0110 of whom was considered to have congenital heart disease and was found at postmortem examination to have primary pulmonary disease. In the other case the child was considered to have primary pulmonary disease and then was found to have pulmonary disease secondary to heart disease. The first case is that of a girl who was first seen in this hospital as an infant because of a mild degree of cyanosis. She had been getting along quite vell, but had been noted to breathe a little harder than the other children. Aside from the cyanosis there was no abnormality on examination of this baby except that the second heart sound was very loud. There was no distinct cardiac murmur during the period of infancy’. However, as the child was followed, a systolic murmur became audible. This was a soft, Grade II to Grade III pansystolic murmur and was maximum in the third left interspace medial to the apex. The second sOulld was narrowly split, fixed and was quite sharp. The electrocardiogram showed right ventricular hypertrophy. The roentgenogram at the time showed that the ileart was only slightly enlarged and that there was a prominence in the pulmonary artery area. This was a number of years ago, and at that time, the clinical diagnosis was Eisenmenger syndrome; that is, a ventricular septal defect with pulmonary vascular obstructive changes and high pulmonary artery pressure. This child was followed for a number of years, and finally, at the age of 8 years, came to this hospital in extremis. She was quite cyanotic. She still had a faint systolic murmur, and a loud snapping second heart sound. Roentgenograms showed that the heart was 110W quite large. The pulmonary artery was still quite prominent, and the major branches of the pulmonary artery also seemed to be somewhat enlarged. The vascular markings in the lung fields were essentially normal, and the most important clinical impression one got of this child was that she was in right heart failure and was cyanotic. The diagnosis was the syndrome of pulmonary vascular obstruction, probably associated with a ventricular septal defect or patent ductus arteriosus. The patient began to have “black-out” episodes. She would suddenly become extremely pale and ashen and would become unconscious for brief periods. These spells became increasingly severe, until she died during one of them. At postmortem examination, the heart was normal except that the right ventricle was thick and dilated. Examination of the lungs revealed a marked pulmonary vascular sclerosis invoiving the media and intima of the large and medium size arteries. There was a small probepatent foramen ovale to explain the site of right to left shunting. This was clearly a case of primary pulmonary vascular disease. The second patient is a 2-month-old infant