Background: Childhood interstitial lung disease (ChILD) is rare and comprises a multitude of heterogeneous diseases.  Data on severe courses leading to lung transplantation (LuTx) and outcome of these patients are lacking.

Objective: We hypothesized that specific subentitities of ChILD lead to transplantation and these differ with patient age at transplantation.

Methods: We analyzed indications leading to LuTx (Heart-Lung-Tx and Re-Tx excluded) for patients aged zero to eighteen years at our centre from 2011 to 2022. ChILD subentities were categorized according to the Deutsch-Classification (Deutsch GH et al. AJRCCM 2007) by lung histology and/or genetic testing. Outcome of transplanted ChILD patients is reported as of January 2023.

Results: A total of 97 patients (53% female) underwent LuTx. 27 (28%) were transplanted for a ChILD diagnosis. The most common diagnosed subentity of ChILD leading to LuTx in n=11 (41%) was ?B3: Disorders of the immunocompromised host? (mean age 11.7±3.2 years) in children after stem cell transplantation/chemotherapy/radiation leading to severe ChILD followed by n=4 (15%) ?A2: Growth abnormalities reflecting deficient alveolarization? (mean age 4.6±4 years) and ?B1: Disorders related to systemic disease processes? (mean age 5.8±4.4 years) and n=3 (11%) ?A1: Diffuse developmental disorders? (mean age 6.7±3.4 years) and ?A4: Surfactant Dysfunctions? (mean age 6.3±0.9 months). At time of reporting, 24 (89%) patients are alive (one after Re-Tx) after a median time of 4.65 years post-LuTx for ChILD.

Conclusion: Paediatric LuTx for endstage ChILD is feasible for all age groups and demonstrates excellent outcome.