WS03.03 Clinical outcomes and long-term survival in cystic fibrosis (CF) lung transplant recipients in Belgium

Objectives: Lung transplantation (LTx) is a therapeutic option for cystic fibrosis (CF) patients suffering from end-stage lung disease. Our objective is to report the experience of LTx for CF in Belgium. Methods: CF patients who benefitted from a LTx between 1998 and 2020 were retrieved from the Belgian CF Registry (BCFR). Demography, clinical outcomes, complications, and actuarial survival are reported. Results: 263 (120 males) patients received either a LTx (n = 249) or a LTx combined with other organ (liver, kidney, liver-kidney) (n = 14); 25 received a re-LTx and 18 a 2nd or 3rd other organ transplant (Tx). 60% of the patients were F508del homozygous and 94% were pancreatic insufficient. Median age (IQR) at surgery was 28.3 (22.2–35.4) yrs and was higher in patients transplanted after 2009 (30.5 (23.1–38.3) vs 26.7 (21.8–33.3) yrs). A LTx <18 yrs was performed in 18 (7%) of the patients with the most recent one in 2016. Median FEV1% predicted increased from 24.4 (19.7–29.1) before LTx to 79.6 (66.4–88.3), 76.7 (61.0–89.0), 73.4 (61.8–88.7) at 1, 5 and 10 yrs post-LTx. Over the same periods, the % of underweight patients decreased from 50% to 26, 25 and 29% and CFRD was present in 38, 57, 54 and 56%. At 1, 5 and 10 yrs after Tx, respectively 6, 15 and 28% were diagnosed with a chronic lung allograft dysfunction (CLAD) (mostly bronchiolitis obliterans syndrome) and 45, 56 and 51% had a chronic renal insufficiency (CRI). A post-transplant lymphoproliferative disease (PTLD) was diagnosed in 21 patients and other cancer in 23 patients. Actuarial survival was 94, 82 and 73% respectively at 1, 5 and 10 yrs after Tx. 75 patients died. In 2020, 188/1352 (14%) CF patients in Belgium were living with a LTx. Conclusions: LTx has become rare in children with CF. In CF patients, LTx results in a consistent excellent improvement in FEV1, BMI and survival despite the increased occurrence of comorbidities related to CF or to the Tx like CFRD, CRI, PTLD and cancer.