ESOPHAGEAL-VARICES IN ASSOCIATION WITH UNILATERAL PULMONARY VEIN ATRESIA

A 27-yr-old woman with recurrent episodes of hemoptysis (and hematemes is due to esophageal varices) was found to have unilateral pulmonary v ein atresia. Reversed flow in the left pulmonary artery, a finding hig hly suggestive of this rare congenital anomaly, was demonstrated durin g cardiac catheterization, The definitive diagnosis was afforded by pu lmonary wedge angiography, which not only demonstrated the atretic lef tsided pulmonary veins, but also revealed a serpiginous system of syst emic collateral veins to be the cause of her esophageal varices. Pneum onectomy of the nonfunctioning hypoplastic lung, the most successful a pproach to this disorder, may be complicated by severe bleeding of the numerous systemic arterial collateral vessels transected during surgi cal mobilization of the affected lung, percutaneously delivered vascul ar occlusion coils were used in this case to occlude the large systemi c to pulmonary artery collateral arteries immediately prior to pneumon ectomy, 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 patie nts requiring lung or heart/lung transplantation. (C) 1996 Wiley-Liss, Inc.