Congenital unilateral pulmonary vein stenosis complicating transposition of the great arteries.

Four patients with transposition of the great arteries and unilateral pulmonary vein (PV) stenosis, all left-sided, were studied. Two patients had an intact ventricular septum (1 with a patent ductus arteriosus), 1 patient had a ventricular septal defect and 1 had a ventricular septal defect with pulmonary stenosis. Clinical signs, such as recurrent pneumonia, unilateral pulmonary edema and pleural effusion, were present preoperatively in 2 patients. Diagnosis was made at cardiac catheterization by cineangiography in 2 patients and at Mustard operation when the PV orifices were inspected in the other 2. PV dilatation was attempted in 3 patients, 1 before correction (age 6 months), 1 during and after it (ages 1 and 3 years, respectively) and 1 during corrective surgery (age 15 months). In the fourth patient only the intracardiac baffle was enlarged near the left PV orifices. In the first patient, at Mustard operation (age 18 months), only a fibrotic PV without an orifice was found; this patient died after surgery. The mean follow-up in the 3 survivors was 3.2 years (range 2 months to 7.6 years). All have severe residual PV obstruction documented by technetium-99m lung perfusion scans that show decreased flow to the left lung (0 to 16% total counts; normal 45%); 2 have unilateral pulmonary edema and 1 has pulmonary artery pressure at systemic level. It is believed that in patients with transposition of the great arteries, left-sided unilateral PV stenosis is a congenital anomaly that becomes progressive as a result of postnatal preferential flow to the right lung.