Pediatric lung transplantation for childhood interstitial lung disease: Indications and outcome.

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 outcomes after LuTx for chILD are limited. We compared characteristics of LuTx for chILD to the indications of cystic fibrosis (CF) and pulmonary hypertension (PH).

METHODS

Patients with chILD <18 years who underwent LuTx at our center between January 1, 2011 and September 30, 2023 were retrospectively analyzed and divided into 2 groups depending on their age at disease manifestation: children in the chILD A group predominantly became ill during the first 2 years of life, chILD B patients thereafter. Outcomes were compared to patients with CF and PH.

RESULTS

One hundred and one 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 intensive care unit (ICU) stay (23 vs 4, 2, 13 days) and median hospital stay (48 vs 27, 30, 42 days) after LuTx were longer. Patients in chILD B had the lowest pretransplant 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%).

CONCLUSIONS

LuTx for patients with chILD shows favorable outcome, although younger chILD A patients had a higher pretransplant morbidity and longer ICU and hospital stay surrounding the transplantation.