Improved results with lung transplantation for cystic fibrosis: a 6-year experience.

Since 1996, 102 patients with cystic fibrosis were accepted on our waiting list and 57 bilateral sequential lung transplants were performed in 56 patients. Before transplantation, the mean FEV1 was 0.64 l/s, the mean PaO2 with supplemental oxygen was 56 mmHg and the mean 6-min walking test was 320 m. Transplantation was performed through a 'clam shell incision' in the first 29 patients; in the rest of the patients we performed a bilateral anterolateral thoracotomy without sternal division. In 21 patients the donor lungs had to be trimmed by extra-anatomical wedge resections with mechanical staplers and bovine pericardium buttressing to fit the recipient chest size. Hospital mortality was 11.6%. Acute rejection occurred 1.6 times per patient/year; pulmonary infections occurred 1.4 times per patient in the first year after transplantation. The mean FEV1 increased to 82% 1 year after the operation. The 2-year survival rate was 79%. Fifteen patients had BOS: one died 26 months after the operation with fungal sepsis after reinduction of immunosuppression, one has been successfully retransplanted, five were stabilized after modifications of immunosuppression and four are continuing to deteriorate. Lung transplantation is a concrete therapeutic option for patients with cystic fibrosis.