Cystic fibrosis (CF) is an autosomal recessive disorder affecting approximately 1 in 2,500 live births worldwide, with double this estimated frequency in Ireland. CF is characterized by a genetic defect of the CF transmembrane regulator protein, causing impairment of chloride ion transportation. This has multisystem consequences, particularly in the lungs, where it results in intensely mucoid secretions, which increases susceptibility to infection. Lung transplantation is indicated in CF when there is progressive decline in a patient's functional reserve. In this report, the authors present a 6-year case review of allograft lung transplantations in 41 CF patients from the Irish National Centre for Lung Transplantation from 2010 through 2015. Preoperative risk factors for morbidity and major mortality are discussed. The authors' experience with intraoperative anesthetic challenges and management options are outlined, and postoperative complications are discussed.
Keywords: anesthesia; cystic fibrosis; lung transplantation.
Copyright © 2017 Elsevier Inc. All rights reserved.