Ryan D. Coleman, Sarah P. Cohen, Pirooz Eghtesady, R. Mark Grady, David L. S. Morales. Don Hayes Jr., Joseph K. Ruminjo, W. Graham Carlos
Baylor College of Medicine. Nationwide Children’s Hospital. Washington University School of Medicine. Cincinnati Children’s Hospital Medical Center. Indiana University School of Medicine.
United States
Annals of the American Thoracic Society
Ann Am Thorac Soc 2025;
DOI: 10.1513/AnnalsATS.202501-132AG
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.
Category
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Lung Transplantation for Pulmonary Vascular Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No