Lung and Heart-Lung Transplantation for Children with PAH: Dramatic Benefits from the Implementation of High Priority Allocation Program in France

Abstract

Pulmonary arterial hypertension (PAH) is rare but remains a fatal disease in infants and children despite the recent introduction of targeted therapies. Lung transplantation (LT), first performed in pediatric patients in the 1980s, is the only potentially life-extending option in patients with end-stage PAH but is possible only in tightly selected patients. Size-matching challenges severely restrict the donor organ pool which, in addition to peculiarities of PAH in infants, results in high mortality while on the waiting list. To investigate the impact on survival of a high-priority allocation program (HPAL) in children with PAH listed for double lung (DLT) or heart-lung (HLT) transplantation METHODS: We conducted a single-center, retrospective, before-after study of consecutive children with severe Group 1 PAH listed for DLT or HLT between 1988 and 2019. The HPAL was implemented in France in 2006 and 2007 for HLT and DLT, respectively. Fifty-four children with PAH were listed for transplantation. Mean age of transplantation was 15.8 ± 2.8 years. Main etiologies of PAH were idiopathic (65%) and congenital heart disease (25%). 72% had a heart-lung transplantation. HPAL implementation resulted in the following significant benefits: decrease in the cumulative incidence of waiting-list death within 1 and 2 years (P<0.0001); increase in the cumulative incidence of transplantation within 6 months, from 44% to 67% (P<0.01); and improved survival after listing (61%, 50%, and 44% vs. 92%, 84%, and 72% 1, 3, and 5 years before and after HAPL implementation, respectively; P=0.02). HAPL implementation significantly improved access to transplantation and survival after listing in children with end-stage PAH.