Lung transplantation for cystic fibrosis: effective and durable therapy in a high-risk group

Ann Thorac Surg. 1998 Aug;66(2):337-46. doi: 10.1016/s0003-4975(98)00496-2.


Background: The purpose of this study was to review our experience with lung transplantation in patients with end-stage cystic fibrosis.

Methods: Eight-two patients with cystic fibrosis have undergone bilateral lung transplantation (n=76) or bilateral lower lobe transplantation (n=6) since October 1990.

Results: Actuarial survival for the entire cohort is 79% at 1 year and 57% at 5 years. The development of bronchiolitis obliterans syndrome is the leading cause of death after the first year. Freedom from bronchiolitis obliterans syndrome is 84% at 1 year and 51% at 3 years. Pulmonary function tests improve dramatically in recipients. There was no association between death within 1 year and recipient age, weight, graft ischemic time, cytomegalovirus seronegativity, or the presence of pan-resistant organisms. Similarly, there was no association between the development of bronchiolitis obliterans syndrome within 2 years and ischemic time, number of rejection episodes, cytomegalovirus seronegativity, or the presence of panresistant organisms.

Conclusions: Despite their poor nutritional status and the presence of multiply resistant organisms, patients with cystic fibrosis can undergo bilateral lung transplantation with acceptable morbidity and mortality.

MeSH terms

  • Adolescent
  • Adult
  • Anesthesia / methods
  • Bronchiolitis Obliterans / etiology
  • Child
  • Cystic Fibrosis / mortality
  • Cystic Fibrosis / surgery*
  • Female
  • Graft Rejection
  • Humans
  • Immunosuppression Therapy / methods
  • Lung Transplantation* / methods
  • Male
  • Middle Aged
  • Postoperative Care / methods
  • Postoperative Complications
  • Risk Factors
  • Survival Rate
  • Treatment Outcome