Outcome of Heart-Lung and Double Lung Transplantation in Pulmonary Arterial Hypertension Due to Congenital Heart Disease is Related to the Complexity of the Defects

Abstract

Heart and lung transplantation (HLT) or double lung transplantation (DLT) with shunt closure is an end-stage therapeutic option in patients with pulmonary arterial hypertension (PAH) due to congenital heart diseases (CHD). Its benefit remains controversial METHODS: We retrospectively investigated outcomes of 108 patients with PAH-CHD listed for transplantation over 32 years and assessed the impact of defects complexity. Complex defects (n=47; 43.5%) included CHD with single ventricle physiology, n=13; mixed defects, n=15; great vessels transposition, n=7.Patients had Eisenmenger syndrome in 81 cases (75.0%) and PAH persisting post corrective surgery in 27 cases (25.0%). Age at listing was 34.0 years [23.4-42.3] with 61 patients (56.5%) in WHO status 4. Before being transplanted, 27 patients (25.0%) died at a median delay of 2.4 months [0.5-4.2]. Cumulative incidences of death on waiting list was higher in patients with complex CHD compared to patients with simple CHD (17.1%, and 41.4% versus 7.3 and 18.9% at 1 and 12 months, Log-Rank p<0.03). HLT and DLT were performed in 65 cases (60.2%) at a median delay of 3.2 months after listing [0.2-14.3] (HLT, n=49; 75.4%; DLT with percutaneous shunt closure, n=6; 5.6%; DLT, n=10; 19.0%). Following transplant, survival was higher in patients with simple CHD compared to patients with complex CHD (83.8% and 70.6% versus 50.1% and 44.5% at 1 and 5 years respectively, Log-Rank test p<0.002). Considering the whole population, being transplanted was associated with a better outcome in patients with simple defects and in patients with complex CHD (Log-Rank test p<0.003 and p<0.0001 respectively). In selected patients with PAH-CHD listed for transplant, complex defects are associated with higher mortality both before and after transplant. Outcome of transplanted patients is significantly better even in patients with complex CHD.