{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T17:30:48Z","timestamp":1780075848069,"version":"3.54.0"},"reference-count":7,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T00:00:00Z","timestamp":1698883200000},"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. Cardiovasc. Med."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>This study investigates the pulmonary arterial histopathology in patients with idiopathic pulmonary arterial hypertension (IPAH) and acute vasoreactive phenotype, who demonstrated long-term survival (&amp;gt;30 years) and incidental death from causes other than PAH progression. The pathological changes observed in these patients were compared with those in patients with bone morphogenetic protein receptor type 2 (BMPR2) mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>We present two cases of patients with pulmonary arterial hypertension (PAH) who died incidentally from causes unrelated to PAH progression. We report compares pulmonary arterial histopathology in long-term survivors of CCB-responsive PAH patient and a hereditary PAH patient with a BMPR2 mutation. Lung specimens were analyzed using the Heath and Edwards (HE) classification and percentage muscular wall thickness (%MWT) of pulmonary arterioles. A significant difference in the severity of grading (<jats:italic>p<\/jats:italic>\u2009=\u20090.0001) and distribution between grades 1-2, 4 (<jats:italic>p<\/jats:italic>\u2009=\u20090.001), and 5 (<jats:italic>p<\/jats:italic>\u2009=\u20090.014) was observed between both patients. These findings suggest differential vascular pathology between the two cases, with CCB responders displaying more mild illness lesions compared to BMPR2 mutant patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The study revealed that CCB responders exhibit more mild illness vascular lesions than BMPR2 mutant patients despite their long-term survival, suggesting a difference in vascular pathology between the two phenotypes.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fcvm.2023.1295718","type":"journal-article","created":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T10:29:51Z","timestamp":1698920991000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Case report: Pathological differences in pulmonary arterial hypertension in long-term responders to calcium channel blockers"],"prefix":"10.3389","volume":"10","author":[{"given":"Yuichi","family":"Tamura","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sayamaa","family":"Lkhagvadorj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yudai","family":"Tamura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asuka","family":"Furukawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shinsuke","family":"Aida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hirotoshi","family":"Ebinuma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takayuki","family":"Shiomi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,11,2]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"3105","DOI":"10.1161\/CIRCULATIONAHA.104.488486","article-title":"Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension","volume":"111","author":"Sitbon","year":"2005","journal-title":"Circulation"},{"key":"B2","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1161\/01.CIR.18.4.533","article-title":"The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects","volume":"18","author":"Heath","year":"1958","journal-title":"Circulation"},{"key":"B3","doi-asserted-by":"publisher","first-page":"e1","DOI":"10.1016\/j.ajog.2007.06.061","article-title":"Pulmonary arteriole muscularization in lambs with diaphragmatic hernia after combined tracheal occlusion\/glucocorticoid therapy","volume":"197","author":"Davey","year":"2007","journal-title":"Am J Obstet Gynecol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"464","DOI":"10.1164\/rccm.201508-1678OC","article-title":"Critical genomic networks and vasoreactive variants in idiopathic pulmonary arterial hypertension","volume":"194","author":"Hemnes","year":"2016","journal-title":"Am J Respir Crit Care Med"},{"key":"B5","doi-asserted-by":"publisher","first-page":"2002341","DOI":"10.1183\/13993003.02341-2020","article-title":"Targeting transforming growth factor-\u03b2 receptors in pulmonary hypertension","volume":"57","author":"Guignabert","year":"2021","journal-title":"Eur Respir J"},{"key":"B6","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1161\/01.CIR.40.4.583","article-title":"Primary pulmonary hypertension in a child. Response to pharmacologic agents","volume":"40","author":"Satyanarayana Rao","year":"1969","journal-title":"Circulation"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1207","DOI":"10.1161\/01.CIR.80.5.1207","article-title":"Primary pulmonary hypertension. Vascular structure, morphometry, and responsiveness to vasodilator agents","volume":"80","author":"Palevsky","year":"1989","journal-title":"Circulation"}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2023.1295718\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,2]],"date-time":"2023-11-02T10:29:54Z","timestamp":1698920994000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2023.1295718\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,2]]},"references-count":7,"alternative-id":["10.3389\/fcvm.2023.1295718"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2023.1295718","relation":{},"ISSN":["2297-055X"],"issn-type":[{"value":"2297-055X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,2]]},"article-number":"1295718"}}