Progressive pulmonary vascular disease after surgical correction (Mustard procedure) of transposition of great arteries with intact ventricular septum.

Severe pulmonary vascular disease developed 1 year after a Mustard operation in a 2 1/3 year old boy with transposition of the great arteries and intact ventricular septum. The possible etiologic factors responsible for the development of pulmonary vascular disease are discussed, and it is suggested that this disturbing late postoperative complication may occur more frequently than is recognized.