• Estibaliz Valdeolmillos, Jérôme Le Pavec, Marion Audié, Laurent Savale, Xavier Jais, David Montani, Olivier Sitbon, Séverine Feuillet, Olaf Mercier, Jérôme Petit, Marc Humbert, Elie Fadel, Emre Belli, and Sébastien Hascoët.
• Department of Congenital Heart Diseases, Centre de Référence Malformations Cardiaques Congénitales Complexes M3C, Hôpital Marie Lannelongue, Groupe Hospitalier Paris-Saint Joseph, Faculté de Médecine, Université Paris Saclay, Le Plessis-Robinson, France; INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Université Paris-Saclay, Le Plessis-Robinson, France.
• J. Thorac. Cardiovasc. Surg. 2024 Sep 1; 168 (3): 943954943-954.

BackgroundReverse Potts shunt (RPS) and lung or heart-lung transplantation are life-extending surgical interventions for pediatric patients with severe pulmonary arterial hypertension (PAH). Robust criteria for identifying patients who will benefit from these procedures remain elusive. Based on 30 years of experience, we sought to refine the surgical indications.MethodsThis single-center retrospective cohort study included 61 consecutive pediatric patients with PAH managed by RPS (2004-2020) or transplantation (1988-2020). Their mid-term outcomes were assessed.ResultsCompared with the 20 patients managed by RPS, the 41 transplant waitlist patients, of whom 28 were transplanted, were older (14.9 vs 8.0 years, P = .0001), had worse right ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm vs 18.0 mm, P = .03), and were managed later in the evolution of the disease (6.0 vs 1.7 years, P = .002). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% (P = .02) and 5-year survival increased from 57.1% to 74.7% after RPS and 55.6% to 77.2% after transplantation. At a median follow-up of 8.6 years after RPS and 5.9 years after transplantation, functional capacity had improved significantly, and PAH-specific drug requirements had diminished markedly in the RPS group. Two patients successfully underwent double-lung transplant 6 and 9 years after RPS.ConclusionsIn selected children with suprasystemic PAH, RPS is associated with functional capacity improvements and decreased pharmacotherapy needs over the midterm. RPS deserves consideration earlier in the course of pediatric PAH, with transplantation being performed in the event of refractory RV failure.Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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