Halis Yilmaz, Deniz Elcik, Aydin Tuncay, Ozge Pamukcu, Serpil Taheri, Fatma Sena Topcu, Ismail Duzgun, Nazmi Narin, Ecmel Mehmetbeyoglu Duman, Begum Er, Gökmen Zararsiz
Erciyes University School of Medicine and Erciyes University.
Turkey
Scientific Reports
Sci Rep 2026;
DOI: 10.1038/s41598-026-61056-x
Abstract
Pulmonary arterial hypertension (PAH) is a major complication of congenital heart disease (CHD) with left-to-right shunts. Early identification of pulmonary vascular alterations is critical for improving clinical outcomes. In this study, we aimed to determine circulating and tissue-associated miRNA expression profiles in children with CHD-associated PAH and to evaluate their association with disease severity. The expression profiles of 384 miRNAs were analyzed by real-time PCR in peripheral whole blood and pulmonary artery tissue samples obtained from children with CHD-associated PAH (n = 25) and healthy controls (n = 15). Blood samples were collected preoperatively and 10 days postoperatively. Pulmonary artery tissue samples were obtained during corrective surgery. Multiple circulating miRNAs were significantly dysregulated in PAH patients compared with controls. Among these, miR-200a-3p, miR-204-5p, and miR-184 showed the most prominent increases in preoperative blood samples. Distinct miRNA expression patterns were also observed according to pulmonary arterial pressure severity, with miR-499a-5p predominantly elevated in patients with PAP ≤ 40 mmHg and miR-656 increased in patients with PAP > 40 mmHg. Several circulating miRNAs demonstrated reduced expression in postoperative samples. In pulmonary artery tissue, miR-218, miR-146a-5p, and miR-144-3p were significantly increased in patients with more severe disease. Our findings demonstrate significant alterations in circulating and tissue-associated miRNA expression profiles in children with CHD-associated PAH. In particular, miR-200a-3p, miR-204-5p, and miR-184 emerged as potential candidate biomarkers associated with disease progression and pulmonary vascular remodeling. These findings support the potential role of miRNA dysregulation in the molecular pathogenesis of CHD-associated PAH.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Potential Biomarkers Associated with 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: Yes