Transplantation for Pulmonary Arterial Hypertension with Congenital Heart Disease: Impact of current therapeutic approach including a high-priority allocation programme on outcomes

Patients with end-stage pulmonary arterial hypertension due to congenital heart disease had limited access to heart-lung transplantation or double-lung transplantation. Methods and results We aimed to assess the effects of a high-priority allocation programme established in France in 2007. We conducted a retrospective study to compare waiting-list and post-transplantation outcomes before versus after implementation of the high-priority allocation programme. We included 67 consecutive patients (mean age at listing 33.2 ± 10.5 years) with pulmonary arterial hypertension due to congenital heart disease listed for heart-lung transplantation or double-lung transplantation from 1997 to 2016. At one month, the incidences of transplantation and death before transplantation were 3.5% and 24.6% in 1997–2006, 4.8% and 4.9% for patients on the regular list in 2007–2016, and 41.2% and 7.4% for patients listed under the high-priority allocation programme (P  Conclusion Increased incidence of transplantation, decreased waiting list mortality, and improved early and long-term outcomes were observed in patients with pulmonary arterial hypertension due to congenital heart disease listed for transplantation in the recent era, characterized by implementation of a high-priority allocation programme.