Estibaliz Valdeomillos, Jerome Le Pavec, Marion Audie, Laurent Savale, Xavier Jais, David Montani, Oliver Sitbon, Severine Feuillet, Olaf Mercier, Jerome Petit, Marc Humbert, Elie Fadel, Emre Belli, Sebastien Hascoet
Centre de Référence Malformations Cardiaques Congénitales Complexes M3C, HÔpital Marie Lannelongue, Groupe Hospitalier Paris-Saint Joseph and Université Paris Saclay.
France
Journal of Thoracic and Cardiovascular Surgery
J Thorac Cardiovasc Surg 2023;
DOI: 10.1016/j.jtcvs.2023.11.045
Abstract
Background: Reverse 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.
Methods: This 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.
Results: Compared to the 20 patients managed by RPS, the 41 transplant waitlist patients, of whom 28 were transplanted, were older (14.9 versus 8.0 years, P=0.0001), had worse right-ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm versus 18.0 mm, P=0.03) and were managed later in the evolution of the disease (6.0 versus 1.7 years, P=0.002). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% (P=0.02) and 5-years survival raised 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 RPS group. Two patients successfully underwent double-lung transplant six and nine years after RPS.
Conclusion: In selected children with suprasystemic PAH, RPS is associated with functional capacity improvements and decreased pharmacotherapy needs over the mid-term. RPS deserves consideration earlier in the course of pediatric PAH, with transplantation being performed in the event of refractory RV failure.
Category
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Lung Transplantation for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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