{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T10:09:26Z","timestamp":1753870166941,"version":"3.41.2"},"reference-count":15,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T00:00:00Z","timestamp":1732492800000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology Case Reports"],"published-print":{"date-parts":[[2024,11]]},"abstract":"Abstract<\/jats:title>Pulmonary veno\u2010occlusive disease (PVOD) is a rare form of pulmonary vascular disease that is difficult to distinguish clinically from pulmonary arterial hypertension (PAH). Multiple genes have been implicated in disease pathogenesis in PAH and PVOD and the diseases are thought to be genetically distinct. In this report we present a case of first\u2010degree relatives with pathological evidence of PVOD and PAH. The index patient was diagnosed with PAH at age 42, was treated with escalating pulmonary vasodilator therapy, but eventually succumbed to her disease. On autopsy, her pathology was consistent with PAH. Her son was diagnosed with PAH at age 16, did well on pulmonary vasodilator therapy for over 10\u2009years, but ultimately developed refractory right ventricular failure and received a heart and lung transplantation. Pathology of his explanted lung was consistent with PVOD, and genetic testing was negative for recognized variants that cause PAH or PVOD.<\/jats:p>","DOI":"10.1002\/rcr2.70064","type":"journal-article","created":{"date-parts":[[2024,11,25]],"date-time":"2024-11-25T23:19:39Z","timestamp":1732576779000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Case report: A finding of PVOD<\/scp> and PAH<\/scp> in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases"],"prefix":"10.1002","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4750-0745","authenticated-orcid":false,"given":"Roger","family":"Winters","sequence":"first","affiliation":[{"name":"Internal Medicine Residency Program, Department of Medicine University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lindsay M.","family":"Forbes","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dunbar","family":"Ivy","sequence":"additional","affiliation":[{"name":"Division of Pediatric Cardiology University of Colorado Children's Hospital Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlyne","family":"Cool","sequence":"additional","affiliation":[{"name":"Division of Pathology University of Colorado Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bryan D.","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Hountras","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Badesch","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sue","family":"Gu","sequence":"additional","affiliation":[{"name":"Division of Pulmonary Sciences & Critical Care Medicine, Pulmonary Vascular Disease Center University of Colorado Aurora Colorado USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edda","family":"Spiekerkoetter","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Allergy & 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