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Although the disease originates in the pulmonary vasculature, it ultimately leads to right heart failure and death. PAH is associated with high mortality rates and poor prognosis, with no therapies currently available to reverse pulmonary vascular remodeling, imposing substantial socioeconomic burdens. Growing interest in the gut\u2013lung axis has highlighted the role of gut microbiota and their metabolites in the occurrence and development of PAH. Evidence showed that gut dysbiosis and metabolite imbalances, involving reduced short\u2010chain fatty acids (SCFAs), increased trimethylamine\u2010N\u2010oxide (TMAO), and dysregulated tryptophan metabolism, contributed to pulmonary vascular remodeling. This review systematically compares gut microbiota and metabolites across PAH murine models (including chronic hypoxia, SU5416\/hypoxia [SuHx], monocrotaline [MCT], and non\u2010classical models) and patients (adults and children). The analysis aims to identify disease\u2010specific microbial and metabolic signatures. It is also discussed how the microbiota and their metabolites may influence inflammation around the pulmonary vasculature. Furthermore, the potential of probiotic therapy, fecal microbiota transplantation (FMT), and mesenchymal stem cells (MSCs) therapies as novel treatment strategies for PAH is discussed.<\/jats:p>","DOI":"10.1002\/cph4.70094","type":"journal-article","created":{"date-parts":[[2026,1,6]],"date-time":"2026-01-06T12:30:36Z","timestamp":1767702636000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Gut Microbiota in Pulmonary Arterial Hypertension: Murine Models and Human Microbial Signatures, Pathogenic Mechanisms, and Emerging Therapeutic Avenues"],"prefix":"10.1002","volume":"16","author":[{"given":"Yudan","family":"Qiu","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology and Immunology, West China Second University Hospital Sichuan University  Chengdu China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University) Ministry of Education  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