Paediatric Lung Transplantation for Childhood Interstitial Lung Disease: Indications and Outcome.

Abstract

BACKGROUND Childhood interstitial lung disease (chILD) is heterogeneous, associated with significant morbidity and can cause organ failure. In these cases, lung transplantation (LuTx) is a treatment option. Data on indications and outcome after LuTx for chILD is limited. We compared characteristics of LuTx for chILD to the indications cystic fibrosis (CF) and pulmonary hypertension (PH). METHODS chILD-patients <18 years who underwent LuTx at our center between Jan 1st, 2011 and Sep 30th, 2023, were retrospectively analysed and divided into two groups depending on their age at disease manifestation: Children in the chILD A group predominantly became ill during the first two years of life, chILD B patients thereafter. Outcomes were compared to patients with CF and PH. RESULTS 101 children were included (chILD A 12; chILD B 19; CF 49; PH 21). Patients in the chILD A group were younger (mean age 1.5 vs., 12.9, 15.2, 10.9 years) and frequently required mechanical ventilation before LuTx (41.7%, vs. 10.5%, 2%, 9.5%, respectively). Their median ICU stay (23 vs. 4, 2, 13 days) and median hospital stay (48 vs. 27, 30, 42 days) after LuTx was longer. Patients with chILD B had the lowest pre-transplant ICU requirement (21.1% vs. 66.7% for chILD A, 30.6% for CF and 47.6% for PH) and short median hospital stay. Five year survival was comparable in all groups (80.2%, 86.5%, 80.4%, and 81.2%). CONCLUSION LuTx for patients with chILD shows favourable outcome, although younger chILD A patients had a higher pre-transplant morbidity and longer ICU and hospital stay surrounding the transplantation.