A comprehensive review recently published in the Review of Cardiovascular Medicine covers the epidemiology, pathophysiology, diagnosis and management of pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), a heterogeneous and serious condition accounting for up to one third of all pulmonary arterial hypertension cases in adults.
Key topics include the updated 2022 European Society of Cardiology (ESC)/European Respiratory Society (ERS) haemodynamic definitions, the role of major registries in defining prognosis, and an in-depth exploration of the underlying disease mechanisms — from vascular shear stress to the TGF-β/BMPR2 genetic pathway that has opened the door to new disease-modifying therapies. The review also addresses the distinct challenges of paediatric versus adult management, the complexities of Eisenmenger syndrome, risk stratification and the “treat and repair” strategy in borderline cases.
From a therapeutic perspective, evidence from key trials is reviewed alongside the shift towards initial combination therapy, with sotatercept is highlighted as the most significant recent advance.
Read more at this link on the IMR Press
Citation
Blanca-Jover E, Contreras-Chova F, Jerez-Calero A, Uberos-Fernandez J, Pérez-Lara L. Congenital Heart Disease and Pulmonary Arterial Hypertension: Current Perspectives. Rev Cardiovasc Med. 2026 Mar 20;27(3):48337. doi: 10.31083/RCM48337. PMID: 41923748; PMCID: PMC13036531.