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

S Hascoet, J Le Pavec, A De Lemos, M Pontailler, L Savale, O Mercier, S Mussot, S Feuillet, F Stephan, M Humbert, D Bonnet, E Fadel
Journal of Heart and Lung Transplantation 2020, 39 (4S): S510

PURPOSE: 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.

RESULTS: 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).

CONCLUSION: 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.

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