Lung transplantation for cystic fibrosis

G. F. Coloni, F. Venuta, A. M. Ciccone, E. A. Rendina, T. De Giacomo, M. J. Filice, D. Diso, M. Anile, C. Andreetti, M. T. Aratari, E. Mercadante, M. Moretti, M. Ibrahim

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


Background Lung transplantation is a robust therapeutic option to treat patients with cystic fibrosis. Patients and 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 FEV1 was 0.64 L/s, mean PaO2 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 FEV1 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.

Original languageEnglish
Pages (from-to)648-650
Number of pages3
JournalTransplantation Proceedings
Issue number3
StatePublished - Apr 2004


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