Severe pediatric pulmonary arterial hypertension: Long-term outcomes of reverse Potts shunt and transplantation

Abstract

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 our 30 years of experience, we sought to refine the surgical indications of RPS. This single-center retrospective cohort study included 61 consecutive pediatric patients with PAH managed by RPS (2004–2020) or transplantation (1988–2020). Their long-term outcomes were assessed. 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) and had worse right-ventricular impairment (tricuspid annular plane systolic excursion, 12.5 mm versus 18.0 mm, P = 0.03). After implementation of a high-priority allocation program in 2007, waitlist mortality decreased from 52.6% to 13.6% ( P = 0.02) and 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. The cumulative transplantation incidence after RPS was 0% and 25.3% at 5 and 10 years, respectively ( Fig. 1 ). RPS was associated with functional capacity improvements, decreased pharmacotherapy needs, and delayed need for transplantation over the long-term in selected children with suprasystemic PAH, preserved right ventricular function, and suitable anatomy.