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Pediatric lung transplantation for childhood interstitial lung disease: Indications and outcome
Hendrik Schneider1, Fabio Ius2, Carsten Müller1
1Clinic for Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
Insights
Lung transplantation (LuTx) offers favorable outcomes for children with interstitial lung disease (chILD). Younger chILD patients (chILD A) experienced higher pre-transplant morbidity and longer recovery times, but overall survival rates were comparable across all groups.
Area of Science:
- Pediatric Pulmonology
- Thoracic Surgery
- Critical Care Medicine
Background:
- Childhood interstitial lung disease (chILD) is a heterogeneous condition with significant morbidity and potential for organ failure.
- Lung transplantation (LuTx) is a viable treatment option for severe chILD cases.
- Limited data exists on the specific indications and outcomes of LuTx in pediatric chILD patients.
Purpose of the Study:
- To compare the characteristics and outcomes of LuTx in children with chILD to those with cystic fibrosis (CF) and pulmonary hypertension (PH).
- To analyze differences in LuTx outcomes based on age of disease manifestation in chILD patients.
Main Methods:
- Retrospective analysis of 101 pediatric patients (<18 years) who underwent LuTx between 2011 and 2023.
- Patients were categorized into chILD A (onset <2 years), chILD B (onset >2 years), CF, and PH groups.
- Outcomes including pre-transplant morbidity, intensive care unit (ICU) and hospital stay, and 5-year survival rates were compared.
Main Results:
- The chILD A group was younger and required more mechanical ventilation pre-LuTx, with longer ICU and hospital stays post-LuTx compared to other groups.
- chILD B patients demonstrated the lowest pre-transplant ICU requirement and shorter hospital stays.
- Five-year survival rates were comparable across all groups: chILD A (80.2%), chILD B (86.5%), CF (80.4%), and PH (81.2%).
Conclusions:
- Lung transplantation in pediatric patients with chILD demonstrates favorable outcomes.
- Younger chILD patients (chILD A) present with higher pre-transplant morbidity and require extended ICU and hospital care.
- Despite differences in pre-transplant morbidity and recovery times, long-term survival after LuTx is similar for chILD, CF, and PH.
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.
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