title: Lung transplantation for cystic fibrosis.
abstract: BACKGROUND
  Lung transplantation is a robust therapeutic option to treat patients with cystic fibrosis.
  
  
  METHODS
  Since 1996, 109 patients with cystic fibrosis were accepted onto our waiting list with 58 bilateral sequential lung transplants performed in 56 patients and two patients retransplanted for obliterative bronchiolitis syndrome.
  
  
  RESULTS
  Preoperative mean FEV(1) was 0.64 L/s, mean PaO(2) with supplemental oxygen was 56 mm Hg, and the mean 6-minute walking test was 320 m. Transplantation was performed through a "clam shell incision" in the first 29 patients and via bilateral anterolateral thoracotomies without sternal division in the remaining patients. Cardiopulmonary bypass was required in 14 patients. In 21 patients the donor lungs had to be trimmed by wedge resections with mechanical staplers and bovine pericardium buttressing to fit the recipient chest size. Eleven patients were extubated in the operating room immediately after the procedure. Hospital mortality of 13.8% was related to infection (n = 5), primary graft failure (n = 2), and myocardial infarction (n = 1). Acute rejection episodes occurred 1.6 times per patient/year; lower respiratory tract infections occurred 1.4 times per patient in the first year after transplantation. The mean FEV(1) increased to 82% at 1 year after operation. The 5-year survival rate was 61%. A cyclosporine-based immunosuppressive regimen was initially employed in all patients; 24 were subsequently switched to tacrolimus because of central nervous system toxicity, cyclosporine-related myopathy, or renal failure, obliterative bronchiolitis syndrome, gingival hyperplasia, or hypertrichosis. Ten patients were subsequently switched to sirolimus. Freedom from bronchiolitis obliterans at 5 years was 60%.
  
  
  CONCLUSIONS
  Our results confirm that bilateral sequential lung transplantation is a robust therapeutic option for patients with cystic fibrosis.
authors.affiliation: UO Chirurgia Toracica, II Clinica Chirurgica, Università La Sapienza, Rome, Italy.
authors.forename: G F
authors.initials: GF
authors.lastname: Coloni
authors.forename: F
authors.initials: F
authors.lastname: Venuta
authors.forename: A M
authors.initials: AM
authors.lastname: Ciccone
authors.forename: E A
authors.initials: EA
authors.lastname: Rendina
authors.forename: T
authors.initials: T
authors.lastname: De Giacomo
authors.forename: M J
authors.initials: MJ
authors.lastname: Filice
authors.forename: D
authors.initials: D
authors.lastname: Diso
authors.forename: M
authors.initials: M
authors.lastname: Anile
authors.forename: C
authors.initials: C
authors.lastname: Andreetti
authors.forename: M T
authors.initials: MT
authors.lastname: Aratari
authors.forename: E
authors.initials: E
authors.lastname: Mercadante
authors.forename: M
authors.initials: M
authors.lastname: Moretti
authors.forename: M
authors.initials: M
authors.lastname: Ibrahim
mesh_terms: D003550:Cystic Fibrosis / Q000503:physiopathology / Q000601:surgery*; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006801:Humans; D016040:Lung Transplantation / Q000401:mortality / Q000502:physiology*; D010100:Oxygen / Q000097:blood; D010313:Partial Pressure; D011183:Postoperative Complications / Q000145:classification / Q000453:epidemiology; D012086:Reoperation; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors
chemical_list: D010100:Oxygen