Pediatric cardiologyVentricular septal defect with increased pulmonary vascular resistance: Late results of surgical closure☆

The fate of 57 children who underwent surgical closure of ventricular septal defect in the presence of increased pulmonary vascular resistance was analyzed. Eighteen (32 percent) died at or immediately after operation. Seven late deaths, due to the Eisenmenger syndrome, occurred 1 to 7 years after operation. Follow-up studies, including cardiac catheterization, were performed in 25 survivors, 1 to 11 years (mean 5 years) after operation. In patients operated on after age 2 years, the degree of preoperative elevation of pulmonary vascular resistance largely determined the prognosis: The condition of patients with mild elevation (pulmonary vascular resistance less than one third the systemic level) returned to normal after operation, with one exception. Patients with more severe elevation (pulmonary vascular resistance more than one third the systemic level) most often showed progressive pulmonary vascular disease; the condition of a few remained unchanged and that of only one returned to normal after operation. Operative mortality rate was high in children with elevated pulmonary vascular resistance during the second year of life who were operated on before age 2 years, but pulmonary vascular resistance returned to normal in the three survivors. When pulmonary vascular resistance was increased in the first year of life, it generally decreased spontaneously (independently of operation); the early increase in these cases is believed to represent delayed maturation of the pulmonary vascular bed rather than pulmonary vascular disease. The overall results of surgery in ventricular septal defect associated with a high level of pulmonary vascular resistance are unfavorable and call for interruption of the natural history before significant pulmonary vascular obstructive changes have taken place; this can be achieved by surgical closure before age 2 years or pulmonary artery banding in infancy.