Lung transplantation for cystic fibrosis--a single center experience over 8 years

Colonization of the lung and mediastinal lymph nodes with multi-resistant bacteria, diabetes and malnutrition represent potential risk factors for lung transplantation in cystic fibrosis. We therefore reviewed our experience in this patient population.

Between December 1988 and March 1997, 219 lung and heart-lung transplantations were performed at our institution. Of these, 39 procedures were done in 35 patients with cystic fibrosis. All candidates (mean age 26 years) were oxygen dependent (preoperative mean PO2: 44.8 +/- 9.1 Torr, preoperative mean PCO2: 53.4 +/- 10.5 Torr, one patient on respirator). Of the primary operations, 34 were performed as bilateral sequential lung transplants, one as a heart-lung transplantation.

Mean duration on respirator for survivors was 3.1 (1-12) days, mean ICU and hospital stay were 4.7 (1-13) and 28 (12-79) days, respectively. The 3-month mortality rate was 5.7% (two patients died due to acute graft failure on days 36 and 73). Other causes of death in the follow-up were cerebral bleeding (one patient) and chronic graft failure (three patients). The survival rates were 91% at 1 year, 83% at 3 years and 76% at 5 years. In eight patients, a bronchiolitis obliterans syndrome (BOS) developed (in four cases grade 3). The freedom of BOS (grade 1 or more) at 1, 3 and 5 years was 87, 79 and 55%, respectively. Four retransplantations were performed. Of the 29 patients alive, only seven are physically limited.

Bilateral lung transplantation for cystic fibrosis allows for acceptable early- and long-term results. Postoperative survival is not impaired by infection, diabetes and malnutrition. Long-term functional outcome seems to be comparable to lung transplantation in patients without infectious pulmonary disease.