{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T04:32:19Z","timestamp":1772253139527,"version":"3.50.1"},"reference-count":26,"publisher":"Sociedade Brasileira de Cardiologia","issue":"4","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Bras. Cardiol."],"abstract":"<jats:p>Resumo A doen\u00e7a veno-oclusiva pulmonar (DVOP) e a hemangiomatose capilar pulmonar s\u00e3o tipos raros de substratos histopatol\u00f3gicos dentro do espectro da hipertens\u00e3o arterial pulmonar (HAP) com progn\u00f3stico muito ruim. Caracterizam-se por um processo fibroproliferativo generalizado das veias e\/ou capilares de pequeno calibre com preserva\u00e7\u00e3o das veias maiores, resultando em um fen\u00f3tipo de hipertens\u00e3o pulmonar pr\u00e9-capilar. A apresenta\u00e7\u00e3o cl\u00ednica \u00e9 inespec\u00edfica e semelhante a outras etiologias de HAP. O diagn\u00f3stico definitivo \u00e9 obtido por meio de an\u00e1lise histol\u00f3gica, embora a bi\u00f3psia pulmonar n\u00e3o seja aconselhada devido ao maior risco de complica\u00e7\u00f5es. No entanto, alguns achados adicionais podem permitir um diagn\u00f3stico cl\u00ednico presuntivo de DVOP, especialmente hist\u00f3ria de tabagismo, uso de drogas quimioter\u00e1picas, exposi\u00e7\u00e3o a solventes org\u00e2nicos (particularmente tricloroetileno), baixa capacidade de difus\u00e3o do mon\u00f3xido de carbono (DLCO), dessatura\u00e7\u00e3o ao esfor\u00e7o e evid\u00eancias de doen\u00e7a venosa sem doen\u00e7a card\u00edaca esquerda no exame de imagem, manifestada por uma tr\u00edade cl\u00e1ssica de opacidades em vidro fosco, linhas septais, e linfadenopatias. O transplante pulmonar \u00e9 o \u00fanico tratamento eficaz e os pacientes devem ser encaminhados no momento do diagn\u00f3stico, devido \u00e0 r\u00e1pida progress\u00e3o da doen\u00e7a e ao progn\u00f3stico ruim. Apresentamos o caso de um homem de 58 anos com HAP com caracter\u00edsticas de envolvimento venoso\/capilar em que a suspeita cl\u00ednica, o pronto diagn\u00f3stico e o encaminhamento precoce para transplante pulmonar foram determinantes para um bom desfecho.<\/jats:p>","DOI":"10.36660\/abc.20230565","type":"journal-article","created":{"date-parts":[[2024,6,5]],"date-time":"2024-06-05T14:02:35Z","timestamp":1717596155000},"source":"Crossref","is-referenced-by-count":0,"title":["Hipertens\u00e3o Arterial Pulmonar com Caracter\u00edsticas de Envolvimento Venoso: Um Trabalho Investigativo"],"prefix":"10.36660","volume":"121","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-0277-7398","authenticated-orcid":false,"given":"Daniel In\u00e1cio","family":"Cazeiro","sequence":"first","affiliation":[{"name":"Universidade de Lisboa,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4164-5481","authenticated-orcid":false,"given":"Rui","family":"Pl\u00e1cido","sequence":"additional","affiliation":[{"name":"Universidade de Lisboa,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1744-8843","authenticated-orcid":false,"given":"Miguel Azeredo","family":"Raposo","sequence":"additional","affiliation":[{"name":"Universidade de Lisboa,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joana","family":"Brito","sequence":"additional","affiliation":[{"name":"Universidade de Lisboa,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandra","family":"Borba","sequence":"additional","affiliation":[{"name":"Centro Hospitalar Universit\u00e1rio Lisboa Central,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatiana","family":"Guimar\u00e3es","sequence":"additional","affiliation":[{"name":"Universidade de Lisboa,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eug\u00e9nia","family":"Pinto","sequence":"additional","affiliation":[{"name":"Centro Hospitalar Universit\u00e1rio Lisboa Central,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9968-477X","authenticated-orcid":false,"given":"Pedro","family":"Freitas","sequence":"additional","affiliation":[{"name":"Centro Hospitalar Universit\u00e1rio Lisboa Ocidental,  Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8034-4529","authenticated-orcid":false,"given":"Fausto J.","family":"Pinto","sequence":"additional","affiliation":[{"name":"Universidade de Lisboa,  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Med"},{"issue":"16","key":"ref4","doi-asserted-by":"crossref","first-page":"1627","DOI":"10.1161\/CIRCULATIONAHA.116.019171","article-title":"Scintigraphy Leading to the Misdiagnosis of Chronic Thromboembolic Disease in a Patient With Pulmonary Veno-Occlusive Disease","volume":"133","author":"Balke L","year":"2016","journal-title":"Circulation"},{"issue":"113","key":"ref5","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1183\/09059180.00003909","article-title":"Pulmonary veno-occlusive disease misdiagnosed as idiopathic pulmonary arterial hypertension","volume":"18","author":"Palazzini M","year":"2009","journal-title":"Eur Respir Rev"},{"issue":"4","key":"ref6","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1097\/MD.0b013e31818193bb","article-title":"Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology","volume":"87","author":"Montani 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