{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:52:56Z","timestamp":1782910376950,"version":"3.54.5"},"reference-count":14,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>\n                    Mutations in BMPR2 are the most common genetic cause of heritable pulmonary arterial hypertension (PAH). We report a 47-years-old male with a BMPR2 variant (c.246A &amp;gt; G) who presented with dyspnea and recurrent hemoptysis. Right heart catheterization confirmed PAH with a mean PAP of 46 mmHg, PAWP of 3 mmHg, and PVR of 10.75 WU. A pulmonary artery biopsy was performed to exclude alternative diagnoses such as vasculitis and chronic thromboembolism. Histology showed foam cell accumulation in the intima, infiltration of CD3\n                    <jats:sup>+<\/jats:sup>\n                    T cells, CD20\n                    <jats:sup>+<\/jats:sup>\n                    B cells, and CD68\n                    <jats:sup>+<\/jats:sup>\n                    histiocytes in the intima and media, and mucoid deposition. These findings suggest an active inflammatory component and dysregulation of lipid metabolism in early-stage PAH. Targeted PAH therapy improved symptoms, and resolved hemoptysis after bronchial artery embolization. Although the variant is of uncertain significance, this rare biopsy provides early pathological insights into BMPR2-associated PAH. Further studies are needed to clarify mechanisms linking BMPR2, inflammation, and lipid metabolism in vascular remodeling.\n                  <\/jats:p>","DOI":"10.3389\/fmed.2026.1848607","type":"journal-article","created":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:30:13Z","timestamp":1782909013000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Pulmonary artery biopsy findings in a patient with a BMPR2 variant-associated pulmonary arterial hypertension"],"prefix":"10.3389","volume":"13","author":[{"given":"Hongxia","family":"Wu","sequence":"first","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiya","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology, West China Hospital, Sichuan University","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Faming","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University","place":["Chengdu, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ye","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Respiratory and Critical Care Medicine, West China Hospital, Sichuan University","place":["Chengdu, 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