Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation

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; Sébastien Hascoët

doi:10.1016/j.jtcvs.2023.11.045

Thirty years of surgical management of pediatric pulmonary hypertension: Mid-term outcomes following reverse Potts shunt and transplantation

J Thorac Cardiovasc Surg. 2023 Dec 3:S0022-5223(23)01121-2. doi: 10.1016/j.jtcvs.2023.11.045. Online ahead of print.

Affiliations

¹ 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.
² INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Université Paris-Saclay, Le 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, Faculté de Médecine, Université Paris Saclay, Le Plessis-Robinson, France.
³ INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Université Paris-Saclay, Le Plessis-Robinson, France; Assistance Publique-Hôpitaux de Paris (AP-HP), Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Faculté de médecine, Université Paris Saclay, Le Kremlin-Bicêtre, France.
⁴ 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.
⁵ INSERM UMR_S 999, Pulmonary Hypertension: Pathophysiology and Novel Therapies, Hôpital Marie Lannelongue, Université Paris-Saclay, Le 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, Faculté de Médecine, Université Paris Saclay, Le Plessis-Robinson, France; Assistance Publique-Hôpitaux de Paris (AP-HP), Department of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Faculté de médecine, Université Paris Saclay, Le Kremlin-Bicêtre, France.
⁶ 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. Electronic address: s.hascoet@ghpsj.fr.

PMID: 38052251
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 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.

Conclusions: In 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.

Keywords: Eisenmenger syndrome; Potts shunt; lung transplantation; pulmonary arterial hypertension.