Functional status of patients with large ventricular septal defect and pulmonary vascular disease 6 to 16 years after surgical closure of their defect in childhood.

We have reviewed 27 patients now 6 to 16 years after closure of a ventricular septal defect at 3 to 12 years of age associated with pulmonary hypertension with a view to assessing their quality of life, respiratory function, and exercise performance. All the patients led normal unrestricted lives. Their subjective lack of symptoms was, in general, confirmed by the results obtained from assessment of lung function tests and two-stage exercise testing in 19 volunteers. Abnormal ventilation in relation to work load was noted in 5 patients and a slightly low exercise cardiac output in 6. Although the patients led normal lives and had a satisfactory response to exercise, measurement of pulmonary artery pressure showed striking pulmonary hypertension on effort. Despite the satisfactory progress of these patients their more long-term future must be one of conjecture and their residual pulmonary hypertension, indicative of residual pulmonary obstruction, must lend weight to arguments for earlier closure of ventricular septal defect before 1 to 2 years of age when changes in the pulmonary vascular bed may be reversed after closure of the defect.