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To date, comprehensive investigational analysis of the intestinal microbiota in human subjects is still limited. To address this, we performed a pilot study of the intestinal microbiome in 20 PAH and 20 non\u2010PAH healthy control subjects, recruited from a single center, with each PAH subject recruited simultaneously with a cohabitating non\u2010PAH control subject. Shotgun metagenomic sequencing was used to analyze the microbiome profiles. There were no differences between PAH and non\u2010PAH subjects across several measures of microbial abundance and diversity (Alpha Diversity, Beta Diversity, F\/B ratio). The relative abundance of <jats:italic>Lachnospiraceae bacterium GAM79<\/jats:italic> was lower in PAH stool samples as compared to non\u2010PAH control subject' stool. There was no strong or reproducible association between PAH disease severity and global microbial abundance, but several bacterial species (a relative abundance of <jats:italic>Anaerostipes rhamnosivorans<\/jats:italic> and a relative deficiency of <jats:italic>Amedibacterium intestinale<\/jats:italic>, <jats:italic>Ruminococcus bicirculans<\/jats:italic>, and <jats:italic>Ruminococcus albus<\/jats:italic> species were associated with disease severity (most proximal right heart catheterization hemodynamics and six\u2010minute walk test distance) in PAH subjects. Our results support further investigation into the presence, significance, and potential physiologic effects of a PAH\u2010specific intestinal microbiome.<\/jats:p>","DOI":"10.1002\/pul2.12051","type":"journal-article","created":{"date-parts":[[2022,2,11]],"date-time":"2022-02-11T12:44:28Z","timestamp":1644583468000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["A unique gut microbiota signature in pulmonary arterial hypertension: A pilot study"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7109-2692","authenticated-orcid":false,"given":"Arun","family":"Jose","sequence":"first","affiliation":[{"name":"Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine University of Cincinnati Cincinnati Ohio 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