Lung transplantation for cystic fibrosis.

Cystic fibrosis (CF) is an inherited disease in which the fundamental physiological defect is failure of cAMP regulation of chloride transport. More than 90% of patients with CF will die of chronic, suppurative, obstructive lung disease, with the median survival in the United States currently being 29 years of age. Currently, although other therapies are being aggressively investigated, bilateral lung transplantation offers the only hope for short-term and mid-term survival in patients with CF and end-stage pulmonary disease. Since 1989, 103 bilateral sequential lung transplants (BLT) for CF have been performed at our institution (46 pediatric, 48 adult, 9 redo) at a mean age of 21+/-10 years. Cardiopulmonary bypass was used in all but one pediatric (age <18) transplantation, and in 15% of adults. The hospital mortality rate was 4.9%, with 80% of early deaths related to infection. Bronchial anastomotic complications occurred with equal frequency in the pediatric and the adult populations (7.3%). One- and 3-year actuarial survival rates are 84% and 61%, respectively (no significant difference between pediatric and adult age groups; average follow-up 2.1+/-1.6 years). Mean forced expiratory volume in 1 second increased from 25%+/-9% pretransplantation to 79%+/-35% 1 year posttransplantation. Acute rejection occurred 1.7 times per patient-year, with the majority of these episodes taking place the first 6 months posttransplantation. Need for treatment of lower respiratory infections occurred 1.2 times per patient in the first year after transplantation. Actuarial freedom from bronchiolitis obliterans was 63% at 2 years and 43% at 3 years. Redo transplantation was performed only in the pediatric population, and was associated with an early mortality of 33%. Eight living donor transplants (4 primary transplants, 4 redo transplants) were performed with an early survival of 87.5%. Patients with end-stage CF can undergo BLT with morbidity and mortality comparable with that observed in pulmonary transplantation for other disease entities.