Abstract Rationale Children with childhood interstitial and diffuse lung disease (cHILD) account for a rising proportion of pediatric lung transplant recipients. There are no consensus guidelines addressing when to consider lung transplant evaluation for these children. The objective of this study is to describe the characteristics and outcomes of children with chILD who were evaluated for lung transplant. Methods This is a single center retrospective cohort study including children with cHILD evaluated for initial lung transplant at the Children’s Hospital of Philadelphia between 2014-2024. Data was obtained by chart review. Results 40 children with chILD underwent lung transplant evaluation, 24 were listed for transplant, and 16 received a lung transplant. Bronchiolitis obliterans was the most common diagnosis at evaluation (35%), accounting for 56% of all chILD transplants (Figure 1). Most children evaluated (60%) and 50% of those listed were referred from other institutions. Of children not listed for transplant (n = 16), the median age at evaluation was 11.2 years (IQR 5-14.9), and 30% were male. 56% had a Fan clinical severity score of ≥ 4, and 44% of children were supported with continuous or nocturnal mechanical ventilation. The most common reasons for lack of transplant listing included improvements with medical interventions (50%) or a medical or surgical contraindication to transplant (38%). Of children listed for transplant (n = 24), the median age was 7.9 years (IQR 0.6-11.5) and 63% were male. 92% had a Fan score ≥4, and 67% of children were supported with mechanical ventilation. Six (24%) children died while awaiting transplant. One child improved and was removed from the waitlist, and another was removed by family choice. The median age of children who were not transplanted after listing was only 4.2 years (IQR 0.6-6.4). Three patients underwent more than one evaluation during this period, and two were transplanted after reevaluation. Conclusions Children undergo lung transplant evaluation with a range of chILD diagnoses. Most children evaluated required respiratory support, and the majority of children evaluated were listed for lung transplant. Referral to a pediatric transplant center may identify alternate therapies in lieu of transplant. Unfortunately, a significant minority of patients die awaiting transplant. This study is limited due to sample size and retrospective nature. Changes in practice patterns and organ allocation over a decade may impact these findings. Future study is needed to identify clinical characteristics that suggest optimal timing of lung transplant evaluation for children with severe lung disease due to chILD. This abstract is funded by: None
Dunn et al. (Fri,) studied this question.