The development of pulmonary vascular obstructive disease after successful Mustard operation in early infancy.

Pulmonary vascular obstructive disease developed postoperatively in an infant with aortopulmonary transposition and intact ventricular septum who underwent a Mustard operation in 3 months of age. Preoperative catheterization had shown normal pulmonary artery pressures. Four months after surgery, catheterization showed pulmonary artery systolic pressure above the systemic level and a tortuous, attenuated pulmonary vascular tree visualized angiographically. Early corrective surgery may not preclude the development of pulmonary vascular obstructive disease in patients with aortopulmonary transposition.