Pulmonary vascular disease in infants with complete atrioventricular septal defect

Abstract

In order to assess incidence and time of occurrence of pulmonary vascular disease, the lungs of 49 patients were studied histologically. Thirty-four lung specimens were obtained at autopsy, and 15 were open lung biopsies. Patients consisted of 24 males and 25 females ranging in age from 10 days to 14 years (median 9 months); 37 had Down's syndrome. Overall incidence of irreversible pulmonary vascular disease (grade 3 or more) under 1 year of age was 34.6%; grade 4 of pulmonary vascular disease was observed only in Down patients. In correlating pulmonary vascular resistance, measured during heart catheterization, with pulmonary vascular disease severity at histology, 6 out of 7 patients with pulmonary vascular resistance over 7 units per metre squared showed grade 4 of pulmonary vascular disease, and of these 4 were under 1 year of age. These findings suggest that a singificant rate of pulmonary vascular disease occurs under 1 year of age, with most severe degrees in the time interval 7–12 months. They show that good correlation exists between pulmonary vascular resistance over 7 units per metre squared and grade 4 of pulmonary vascular disease. They demonstrate that the most severe pulmonary vascular disease is seen in Down's syndrome. Finally, they indicate that early surgical correction is mandatory and should be accomplished within 6 months of age.