Pulmonary arterial hypertension with features of venous involvement: a detective's task
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2024 |
| Outros Autores: | , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
| Texto Completo: | http://hdl.handle.net/10451/65034 |
Resumo: | © Sociedade Brasileira de Cardiologia - SBC. This is an open-access article distributed under the terms of the Creative Commons Attribution License |
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Pulmonary arterial hypertension with features of venous involvement: a detective's taskHipertensão arterial pulmonar com características de envolvimento venoso: um trabalho investigativoPulmonary arterial hypertensionPulmonary veno-oclusive diseaseLung transplantationHemangioma capillaryTomography-X-Ray ComputedSurgery© Sociedade Brasileira de Cardiologia - SBC. This is an open-access article distributed under the terms of the Creative Commons Attribution LicensePulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.Sociedade Brasileira de Cardiologia - SBCRepositório da Universidade de LisboaCazeiro, DanielPlácido, RuiAzaredo Raposo, MiguelBrito, JoanaBorba, AlexandraGuimarães, TatianaPinto, EugéniaFreitas, PedroPinto, Fausto J.2024-06-12T14:48:24Z20242024-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10451/65034engArq Bras Cardiol. 2024 Apr 29;121(4):e202305650066-782X10.36660/abc.202305651678-4170info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-11-20T18:30:40Zoai:repositorio.ul.pt:10451/65034Portal AgregadorONGhttps://www.rcaap.pt/oai/openairemluisa.alvim@gmail.comopendoar:71602024-11-20T18:30:40Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
| dc.title.none.fl_str_mv |
Pulmonary arterial hypertension with features of venous involvement: a detective's task Hipertensão arterial pulmonar com características de envolvimento venoso: um trabalho investigativo |
| title |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| spellingShingle |
Pulmonary arterial hypertension with features of venous involvement: a detective's task Cazeiro, Daniel Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| title_short |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| title_full |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| title_fullStr |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| title_full_unstemmed |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| title_sort |
Pulmonary arterial hypertension with features of venous involvement: a detective's task |
| author |
Cazeiro, Daniel |
| author_facet |
Cazeiro, Daniel Plácido, Rui Azaredo Raposo, Miguel Brito, Joana Borba, Alexandra Guimarães, Tatiana Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. |
| author_role |
author |
| author2 |
Plácido, Rui Azaredo Raposo, Miguel Brito, Joana Borba, Alexandra Guimarães, Tatiana Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. |
| author2_role |
author author author author author author author author |
| dc.contributor.none.fl_str_mv |
Repositório da Universidade de Lisboa |
| dc.contributor.author.fl_str_mv |
Cazeiro, Daniel Plácido, Rui Azaredo Raposo, Miguel Brito, Joana Borba, Alexandra Guimarães, Tatiana Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. |
| dc.subject.por.fl_str_mv |
Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| topic |
Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| description |
© Sociedade Brasileira de Cardiologia - SBC. This is an open-access article distributed under the terms of the Creative Commons Attribution License |
| publishDate |
2024 |
| dc.date.none.fl_str_mv |
2024-06-12T14:48:24Z 2024 2024-01-01T00:00:00Z |
| dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
| dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
| format |
article |
| status_str |
publishedVersion |
| dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10451/65034 |
| url |
http://hdl.handle.net/10451/65034 |
| dc.language.iso.fl_str_mv |
eng |
| language |
eng |
| dc.relation.none.fl_str_mv |
Arq Bras Cardiol. 2024 Apr 29;121(4):e20230565 0066-782X 10.36660/abc.20230565 1678-4170 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
| dc.publisher.none.fl_str_mv |
Sociedade Brasileira de Cardiologia - SBC |
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Sociedade Brasileira de Cardiologia - SBC |
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reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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mluisa.alvim@gmail.com |
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1817549295374565376 |