Summary for Clinicians: Clinical Practice Guideline on Interventional Strategies for Children with Progressive Pulmonary Hypertension Despite Optimal Therapy.

Abstract

This guideline provides evidence-based recommendations for managing pediatric patients with progressive pulmonary hypertension (PH) despite optimal medical therapy1. Key interventions systematically reviewed include atrial septal defect (ASD) intervention/atrial septostomy (creation and/or enlargement), pulmonary-to-systemic shunt (reverse Pott's shunt) creation, lung transplantation, and the use of extracorporeal membrane oxygenation (ECMO) support as bridge to other treatments (Table 1). These recommendations aim to guide clinicians managing this fragile patient population, with the strength of recommendation and evidence quality specifically annotated (Table 2). Also of note, these recommendations are primarily focused on patients with progressive pulmonary arterial hypertension despite use of multi-pathway pharmacotherapy; under certain circumstances, however, these recommendations may also apply to children with other types of progressive PH as discussed. As the literature discussing these interventions is only continuing to grow, clinicians are encouraged to consider individual patient circumstances. This summary is intended to provide practicing physicians with key points from the guideline.