IMPROVED RESULTS OF LUNG TRANSPLANTATION FOR PATIENTS WITH CYSTIC-FIBROSIS

Patients with cystic fibrosis pose particular challenges for lung tran splant surgeons. Earlier reports from North American centers suggested that patients with cystic fibrosis were at greater risk for heart-lun g or isolated lung transplantation than other patients with end-stage pulmonary disease. During a 31/2 year period, 44 patients with end-sta ge lung disease resulting from cystic fibrosis underwent double lung t ransplantation at this institution. During the same interval, 18 patie nts with cystic fibrosis died while waiting for lung transplantation. The ages of the recipients ranged from 8 to 45 years, and mean forced expiratory volume in 1 second was 21% predicted, Seven patients had Ps eudomonas cepacia bacteria before transplantation. Bilateral sequentia l implantation with omentopexy was used in all patients. There were no operative deaths, although two patients required urgent retransplanta tion because of graft failure. Cardiopulmonary bypass was necessary in six procedures in five patients and was associated with an increased blood transfusion requirement, longer postoperative ventilation, and l onger hospital stay. Actuarial survival was 85% at 1 year and 67% at 2 years. Infection was the most common cause of death within 6 months o f transplantation (Pseudomonas cepacia pneumonia was the cause of deat h in two patients), and bronchiolitis obliterans was the most common c ause of death after 6 months, Actuarial freedom from development of cl inically significant bronchiolitis obliterans was 59% at 2 years. Resu lts of pulmonary function tests improved substantially in survivors, w ith forced expiratory volume in 1 second averaging 78% predicted 2 yea rs after transplantation. Double lung transplantation can be accomplis hed with acceptable morbidity and mortality in patients with cystic fi brosis.