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 extraanatomical 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. q 2003 Elsevier B.V. All rights reserved. Keywords: Lung transplantation; Cystic fibrosis 1. Introduction Cystic fibrosis (CF) is an inherited disease affecting one in every 2000 newborns; it is the most frequent genetic disease in Caucasians and one of the most common genetic killers in the United States. CF is a disease with many faces affecting many organs; however, the lung is the most seriously involved and more than 90% of the patients die of respiratory failure related to chronic obstructive lung disease and bronchiectasis. The improved therapeutic strategies currently employed had progressively increased the median survival to more than 30 years; however, it is estimated that approximately 300 CF patients die annually in the United States [1]. After the initial clinical application in the early 1980s lung transplantation has been considered with increasing enthusiasm as a concrete therapeutic option for a selected group of patients with CF-related end stage respiratory failure; at the present time CF is the most frequent indication to bilateral sequential lung transplantation (BSLT) [2] that completely replaced heart– lung and en-bloc double lung transplantation; survival rates in this specific subset of patients are reported as high as 85% [3] and the average survival after 1 and 3 years reported by the International Society of Heart and Lung Transplantation Registry is 71% and 56% [4]. We reviewed our 6-year experience with bilateral sequential lung transplantation for cystic fibrosis. 2. Patients and methods Since 1996, 102 patients with CF were accepted in our waiting list for lung transplantation; we performed 57 BSLT in 56 patients (one patient was retransplanted for obliterative bronchiolitis syndrome 3 years after the first procedure). Thirty-four (33%) patients died on the waiting list and 12 (12%) are still waiting for a suitable organ