Danya Z. Jafri, James M. Beck, Samantha A. Kaplan, Allegra Keeler, Bryan Sanchez, Daniela Brady, William H. Frishman, Wilbert S. Aronow, Avi Levine, Erika Rosenzweig, Gregg M. Lanier
New York Medical College School of Medicine. Westchester Medical Center.
United States
Cardiology in Review
Cardiol Rev 2026;
DOI: 10.1097/CRD.0000000000001239
Abstract
Pediatric pulmonary arterial hypertension (PAH) is a relentless and potentially fatal disease marked by elevated pulmonary vascular resistance and pulmonary pressure as a result of unchecked vascular remodeling, proliferation, and dysfunction. While pharmacological treatment algorithms for adult PAH are well-established and frequently updated, pediatric PAH treatment guidelines in the United States are generally based on limited clinical trials and expert opinion, leaving clinicians to extrapolate from adult data and expert consensus rather than pediatric-specific, evidence-based protocols. Endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclin-pathway agents remain the therapeutic cornerstone, yet clinical practice is being reshaped by pivotal therapeutic developments. Recent advances highlight differences in monotherapy versus combination pharmacological regimens, alongside active trials investigating interventional/surgical procedures in the amelioration of long-term outcomes. This review seeks to consolidate contemporary data and clinical trial highlights to reflect a necessary shift toward precise, developmentally informed pharmacotherapy in children, underscoring the pressing need for an updated pharmacological guideline that incorporates pediatric-specific trials and harmonizes labeling and long-term safety surveillance in the treatment of pediatric PAH.
Category
Medical Therapy. Efficacy or Lack of Efficacy
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