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Transplantation for pulmonary arterial hypertension with congenital heart disease: Impact on outcomes of the current therapeutic approach including a high-priority allocation program |
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| Authors: | Sébastien Hascoët Margaux Pontailler Jérôme Le Pavec Laurent Savale Olaf Mercier Dominique Fabre Sacha Mussot Gérald Simonneau Xavier Jais Séverine Feuillet Francois Stephan Sarah Cohen Damien Bonnet Marc Humbert Philippe Dartevelle Elie Fadel |
| Affiliation: | 1. Department of Congenital Heart Diseases, Centre de Référence Malformations Cardiaques Congénitales Complexes M3C, Hôpital Marie Lannelongue, Groupe Hospitalier Paris-Saint Joseph, Plessis-Robinson, Paris, France;2. Department of Congenital Heart Diseases, Centre de référence Malformations Cardiaques Congénitales Complexes M3C, Centre de Compétence de l'Hypertension Artérielle Pulmonaire Sévère, Hôpital Universitaire Necker Enfants Malades, Assistance Publique des Hôpitaux de Paris, Université de Paris, Paris, France;3. UMR-S 999, Inserm, Hôpital Marie Lannelongue, Faculté de Médecine Paris-Saclay, Université Paris-Saclay, Plessis-Robinson, France;4. UMR-S 999, Inserm, Hôpital Marie Lannelongue, Faculté de Médecine Paris-Saclay, Université Paris-Saclay, Plessis-Robinson, France Department of Thoracic and Vascular Surgery, Centre de Référence de l'Hypertension Artérielle Pulmonaire Sévère, Hôpital Marie Lannelongue, Groupe Hospitalier Paris-Saint Joseph, Plessis-Robinson, France;5. UMR-S 999, Inserm, Hôpital Marie Lannelongue, Faculté de Médecine Paris-Saclay, Université Paris-Saclay, Plessis-Robinson, France Department of Pulmonology, Centre de Référence de l'Hypertension Pulmonaire Sévère, DHU Thorax Innovation, Hôpital Bicêtre, Le Kremlin-Bicêtre, France |
| Abstract: | Patients with end-stage pulmonary arterial hypertension due to congenital heart disease have limited access to heart-lung transplantation or double-lung transplantation. We aimed to assess the effects of a high-priority allocation program established in France in 2007. We conducted a retrospective study to compare waitlist and posttransplantation outcomes before versus after implementation of the high-priority allocation program. 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 program (p < .001 and p = .0001, respectively). Overall survival was higher in patients listed in 2007–2016 (84.2% and 61.2% at 1 and 10 years vs. 36.8% and 22.1%, p = .0001). 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 program. |
| Keywords: | clinical research/practice health services and outcomes research heart disease: congenital heart transplantation/cardiology lung transplantation/pulmonology organ allocation organ procurement and allocation |