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Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem to contribute to its onset. To further understand the complex relationship between endothelial and pulmonary hypertension and try to contribute to the development of future therapies, we report a comprehensive and updated review on endothelial function in pulmonary arterial hypertension.
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http://dx.doi.org/10.3390/jcm13082444 | DOI Listing |
Turk J Pediatr
September 2025
Department of Cardiorespiratory Physiotherapy and Rehabilitation, Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Türkiye.
Background: Vascular changes are observed in children with cystic fibrosis (cwCF), and gender-specific differences may impact arterial stiffness. We aimed to compare arterial stiffness and clinical parameters based on gender in cwCF and to determine the factors affecting arterial stiffness in cwCF.
Methods: Fifty-eight cwCF were included.
World J Pediatr Congenit Heart Surg
September 2025
Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea.
: This study aimed to compare the long-term outcomes of a modified réparation à l'étage ventriculaire (REV) and the Rastelli repair for ventricular septal defect (VSD) and pulmonary outflow tract obstruction without ventriculoarterial concordance. : The study included 100 consecutive patients who underwent a modified REV ( = 50) or Rastelli repair ( = 50) for transposition of the great arteries, double outlet right ventricle, or double outlet left ventricle with VSD and pulmonary outflow tract obstruction. The mean ages of the patients who underwent the modified REV and Rastelli repair were 2.
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September 2025
Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Venusberg-Campus 1, 53127, Bonn, Germany.
Aims: Fetal circulation undergoes complex changes in congenital heart disease (CHD) that are challenging to assess with fetal echocardiography. This study aimed to assess clinical feasibility and diagnostic value of 4D flow cardiac magnetic resonance (CMR) in fetal CHD.
Methods And Results: Pregnant women in advanced third trimester pregnancy with fetal CHD were prospectively recruited for fetal CMR between 08/2021 and 11/2024.
PLoS One
September 2025
Respiratory and Critical Care Medicine, The Second Affiliated Hospital of Guangdong Medical University, Zhanjiang, Guangdong, China.
Background: Disulfidptosis, a novel cellular death manner, has yet to be fully explored within the context of pulmonary arterial hypertension (PAH). This study aims to identify genes implicated in PAH that are involved in disulfidptosis.
Method: Based on data from the GEO database, this study employed co-expression analysis, Weighted Gene Co-Expression Network Analysis (WGCNA), hub gene identification, and Gene Set Enrichment Analysis (GSEA) to uncover genes associated with PAH and disulfidptosis.
Am J Physiol Heart Circ Physiol
September 2025
Division of Pediatric Critical Care, Department of Pediatrics, University of California, San Francisco, USA.
Right ventricular (RV) failure is the primary cause of death among patients with pulmonary arterial hypertension (PAH). Patients with congenital heart disease-associated PAH (CHD-PAH) demonstrate improved outcomes compared to patients with other forms of PAH, which is related to the maintenance of an adaptively hypertrophied RV. In an ovine model of CHD-PAH, we aimed to elucidate the cellular, microvascular, and transcriptional adaptations to congenital pressure overload that support RV function.
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