Late development of severe pulmonary venous obstruction following the Mustard operation

While four patients with d-transposition of the great arteries who had a Mustard operation before 2 years of age developed severe pulmonary venous obstruction 2, 3 1/2, 3 9/12, and 5 years following their operation, initial postoperative catheterization (less than 1 year) revealed only minimal pulmonary artery wedge and right ventricular end-diastolic pressure differences, with normal peak systolic pulmonary artery pressures. Serial clinical examination, including height and weight percentiles, chest X-rays, electrocardiograms, and the presence of a continuous murmur in one patient suggested development of pulmonary venous obstruction. Repeat catheterizations demonstrated pulmonary artery pressures greater than or equal to systemic and pulmonary artery wedge minus right ventricular end-diastolic differences greater than 13. Successful relief of the pulmonary venous obstruction included insertion of a Dacron patch to enlarge the pulmonary venous atrium, incision of the obstructing orifice, and repair of associated intra-atrial defects. Operation and postoperative course have been unremarkable. It is concluded that pulmonary venous obstruction can be both an early and progressively late sequela of the Mustard operation. Serial examinations with a high index of suspicion should allow repeated catheterizations to document the occurrence. Operative revision of the obstruction appears to be effective with minimal risk.