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A Review of Lung Transplantation for Patients With &agr;-1 Antitrypsin Deficiency

&agr;-1 antitrypsin deficiency (AATD) is an autosomal codominant condition that predisposes one to emphysema because of the lack of proteolytic defense in the lung. Emphysema leading to respiratory failure is the most common cause of death in AATD, and AATD is currently the fourth most common indication for lung transplantation (LT) in the United States. Although the intraoperative management of LT for AATD resembles that of usual (AAT replete) chronic obstructive pulmonary disease, several special circumstances regard the selection, pretransplant and posttransplant management, and outcomes after LT in AATD. The current review begins with an overview of AATD, followed by a discussion regarding indications for LT and the impact of AATD on LT candidacy. We then review the available experience with outcomes after LT for AATD, while highlighting specific considerations for posttransplant care of these patients.