Lung Transplantation for Pulmonary Vascular Disease in Children: A United Network for Organ Sharing Analysis.

Background: Pulmonary vascular disease (PVD) represents an important clinical indication for lung transplant (LTx) in infants, children, and adolescents. There is limited information on LTx outcomes in these patients. We explored LTx volumes and post-LTx survival in children with PVD compared to other diagnoses.

Methods: The UNOS Registry was queried from 1989-2020 to identify first-time pediatric LTx recipients (<18 yo). PVD was categorized as idiopathic pulmonary arterial hypertension (IPAH) and non-idiopathic arterial hypertension (non-IPAH) and compared to all other patients as other diagnoses. Univariate and multivariate regression models were performed.

Results: 984 pediatric LTx patients (593 before 2010 and 391 during/after 2010) were identified, of which 145 (14.7%) had PVD. There has been no significant change in annual rate of all LTxs over comparative eras. However, there has been a decrease in rate of LTxs for PVD patients. Children with PVD had similar survival to other LTx groups in the early era (p=0.2) and the latter era (p=0.9). Univariate Cox models, showed that LTx in patients with PVD was associated with a significantly less risk of mortality for children aged 6-11 years compared to younger and older cohorts (HR=0.4 [0.17-0.98];p=0.045), whereas multivariate analysis showed a trend towards higher mortality in 11-17-year-olds (HR=1.54 [0.97-2.45];p=0.06). For PVD patients, oxygen supplementation and ventilator support at LTx were associated with worse post-transplant survival (p=0.029 and p=0.01).

Conclusions: There has been a decrease in LTx volume for pediatric patients with PVD in the modern era. Post-LTx outcomes for children with PVD are similar to those of other diagnoses in both eras, with children aged 6-11 years having the best survival. Given these findings, LTx should be considered for this patient population.