Timing and priorities for cystic fibrosis patients candidates to long transplantation

Bilateral lung transplantation is actually considered a valuable option for patients with endstage lung disease related to cystic fibrosis. Timing is crucial to transplant successfully as many patients as possible and it is mainly based on the progressive worsening of pulmonary function tests and quality of life. We reviewed the charts of all patients accepted for lung transplantation at our Institution, in order to assess the role of several functional and demographic parameters; we compared the group of patients able to successfully wait for transplantation (Group A) with patients dying on the waiting list (Group B). Twenty-eight patients were accepted: 15 were successfully transplanted (2 at other institutions) (mean waiting time: 117 days), 7 died waiting (mean waiting time: 108 days) and 6 are still on the list. We recorded FEV-1, FVC, PaO2, PaCO2, supplemental O-2 requirement, 6-minute walking test, right ventricular ejection fraction (RVEF) and cardio-pulmonary hemodynamics measured at right heart catheterization; we recorded also age at time of diagnosis and at time of evaluation, sex, weight and Schwachman score. These parameters were compared between Group A and B. Age at time of evaluation, sex, weight and Schwachman score did not present any difference between the two groups, as well as pulmonary function tests, PaO2, 6-minute walk test and RVEF. A statistically significant difference was found in terms of PaCO2 (43.9 +/- 9.3 in Group A vs 69.1 +/- 32.4 in Group B, heart rate at rest (102 +/- 21 vs 131 +/- 12) mean pulmonary artery pressure (20.6 +/- 2.9 vs 36 +/- 15.7), pulmonary vascular resistances (350 +/- 96 vs 460 +/- 119.4), cardiac index (3.2 +/- 0.6 vs 5.4 +/- 0.9). On the base of our initial experience we conclude that a careful evaluation of CF candidates for lung transplantation is recommended. A deterioration of pulmonary function tests and quality of life are useful parameters to accept patients in the waiting list; however priority should be attributed also on the base of cardio-pulmonary hemodynamics. A larger series of patients is required to draw definitive conclusions.