Abstract

Since 1961 the Cystic Fibrosis Foundation has designated certain institutions devoted to treating cystic fibrosis (CF) as Cystic Fibrosis Care Centers (CFCC). Little is known about the benefits of undergoing lung transplantation (LT) at a transplant center with an accredited CFCC. The purpose of this study was to evaluate outcomes in people with CF who undergo LT at a transplant center with an accredited CFCC. We reviewed the Scientific Registry of Transplant Recipients for all adult (18 and older) patients with CF who received a first-time LT from 2005 to present. The primary exposure was whether a patient received LT at a designated CFCC. The primary outcome was death or re-transplantation within ten years of LT. Kaplan-Meier analysis and multivariate Cox proportional hazards regression were used to assess outcomes. From January 2005 to February 2019, 2,887 patients with CF underwent LT. Of the cohort, there were 1,403 (49%) females. The mean (SD) age at transplant was 32.0 (9.9) years. Out of 72 centers in the US performing LT, 53 (74%) were CFCCs, and most patients (88%) were transplanted at a CFCC. A total of 1,009 (35%) patients died over the study period, and 158 (6%) patients underwent a second transplant. The most common cause of death was infection (n=209, 21%). On adjusted Cox modeling, patients transplanted at a CFCC had a 36% reduced risk of death or re-transplantation compared to those transplanted at a non-CFCC (HR: 0.64, 95% CI: 0.54-0.75, p<0.001). In Kaplan-Meier analysis (Figure 1), at all relevant time-points, receiving a transplant at a CFCC was associated with increased survival or freedom from re-transplantation (p<0.01). People with CF who undergo LT at a CFCC have improved survival and longer freedom from re-transplantation compared to those transplanted at non-CFCCs. These patients suffering from CF likely have greater access to specialized CF care at CFCCs which may contribute to improved post-transplant survival.

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