Esophageal varices in association with unilateral pulmonary vein atresia

A 27-yr-old woman with recurrent episodes of hemoptysis (and hematemesis due to esophageal varices) was found to have unilateral pulmonary vein atresia. Reversed flow in the left pulmonary artery, a finding highly suggestive of this rare congenital anomaly, was demonstrated during cardiac catheterization. The definitive diagnosis was afforded by pulmonary wedge angiography, which not only demonstrated the atretic leftsided pulmonary veins, but also revealed a serpiginous system of systemic collateral veins to be the cause of her esophageal varices. Pneumonectomy of the nonfunctioning hypoplastic lung, the most successful approach to this disorder, may be complicated by severe bleeding of the numerous systemic arterial collateral vessels transected during surgical mobilization of the affected lung. Percutaneously delivered vascular occlusion coils were used in this case to occlude the large systemic to pulmonary artery collateral arteries immediately prior to pneumonectomy. Intra- and perioperative bleeding was minimal, and the patient has done well without further episodes of hemoptysis or hematemesis. Percutaneous occlusion of systemic to pulmonary collaterals may prove useful as a preoperative step for other congenital heart disease patients requiring lung or heart/lung transplantation.