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Rare coding variants in the FOXF1 gene are linked to pulmonary arterial hypertension (PAH), implicating impaired endothelial FOXF1 expression and disrupted BMPR2 regulation in disease development.
Sotatercept improves right heart mechanics in patients with pulmonary arterial hypertension by decreasing right ventricular afterload and contractility in a balanced manner, enhancing right ventricular free wall function and right atrial pe
A newly developed clinical likelihood score accurately distinguishes pulmonary arterial hypertension patients with venous or capillary involvement (PVOD/PCH), enabling earlier diagnosis, tailored therapy, and timely transplant referral.
Germline and somatic variants in DNMT3A and other CHIP genes elevate pulmonary arterial hypertension risk, including connective tissue disease-associated forms, through pro-inflammatory pathways, highlighting novel biomarkers and therapeuti
Combining left ventricular global longitudinal strain and right atrial reservoir strain greatly improves risk prediction and stratification in pulmonary arterial hypertension, outperforming conventional imaging and clinical models.
Echocardiography-derived right ventricular phenotypes improve prognostic accuracy beyond standard risk tools in pulmonary arterial hypertension patients at first follow-up, refining survival predictions and guiding management strategies.
Sotatercept improves exercise tolerance in pulmonary arterial hypertension through pulmonary vascular remodeling, systemic decongestion with hemoconcentration, improved right ventricular-pulmonary artery coupling, enhanced cardiac output re
The ESRA trial demonstrates that riociguat is safe and effectively reduces pulmonary vascular resistance in early pulmonary vascular disease, though secondary benefits remain unproven in this early-stage, mostly connective tissue disease-as
Latent class analysis of 889 adult PAH-CHD patients identified unique phenogroups with distinct survival outcomes and treatment responses, offering refined risk stratification and guiding personalized therapy.
This study identifies the transition of cardiac fibroblasts to myofibroblasts as a critical driver of right ventricular failure in PAH, highlighting the role of UCP2 deficiency and the rs659366 SNP as key clinical indicators of decompensati
Early-stage portopulmonary hypertension, defined by revised 2022 ESC/ERS criteria, independently predicts mortality in patients with cirrhosis, underscoring the importance of systematic screening and early intervention.
This article reviews a phase 3 trial analyzing sotatercept’s efficacy and safety in newly diagnosed pulmonary arterial hypertension patients, highlighting its potential to reduce clinical worsening early in the disease course.
Sotatercept significantly reduces the risk of death, lung transplantation, or hospitalization in high-risk pulmonary arterial hypertension patients, marking a major advance in the management of this life-threatening disease.
