In recent years there has been growing interest in pulmonary disease in infancy, especially in the newborn period. Therefore, it has become more important to try to differentiate primary pulmonary disease as a cause of cyanosis in the newborn period, from congenital heart disease as a cause of the same symptoms.
Since recognition of such clinical entities as hyaline membrane disease, plasma cell pneumonia and some types of interstitial fibrosis of the lung is occurring in younger and younger patients, one may get the impression from the literature that it is fairly easy to separate primary pulmonary disease from pulmonary disease secondary to congenital heart disease.
I woud like to show you two children, one 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 well, 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 sound was narrowly split, fixed and was quite sharp. The electrocardiogram showed right ventricular hypertrophy. The roentgenogram at the time showed that the heart 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 now 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.
- Copyright © 1959 by the American Academy of Pediatrics