Yi Yan, Zhuoyuan Xu, Yanghanyue Zhou, Xike Wang, Hao Zhang
Shanghai Children’s Medical Center, National Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Guizhou Branch of Shanghai Children’s Medical Center and Guizhou Provincial People’s Hospital.
China
Medical Review
Med Rev 2026; 6: 120-141
DOI: 10.1515/mr-2026-0002
Abstract
Pulmonary hypertension (PH) is a severe cardiopulmonary disorder characterized by pulmonary vascular remodeling, which leads to increased pulmonary vascular resistance (PVR), right ventricular (RV) hypertrophy, and ultimately heart failure and death. This review explores the dual landscape of PH, integrating recent insights into its complex pathogenesis with evolving clinical management strategies. The pathogenesis of PH involves a complex interplay of genetics, metabolism, immune dysfunction, biomechanical force and other factors, all contributing to vascular remodeling and presenting opportunities for novel therapies and biomarkers. Among these, certain pathogenic mechanisms are of particular relevance and may hold distinct importance in the pediatric population. Concurrently, notable progress has been made in clinical front including diagnosis, risk stratification, drug development, interventional or surgical procedures, and imaging technologies. Hence, this review integrates current evidence in both PH pathogenesis and clinical management to guide future research toward novel therapies and precision medicine.
Catagory
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult 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: Yes