Publicação
Pulmonary arterial hypertension with features of venous involvement: a detective's task
| Resumo: | Pulmonary 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. |
|---|---|
| Autores principais: | Cazeiro, Daniel |
| Outros Autores: | Plácido, Rui; Azaredo Raposo, Miguel; Brito, Joana; Borba, Alexandra; Guimarães, Tatiana; Pinto, Eugénia; Freitas, Pedro; Pinto, Fausto J. |
| Assunto: | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| Ano: | 2024 |
| País: | Portugal |
| Tipo de documento: | artigo |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Universidade de Lisboa |
| Idioma: | inglês |
| Origem: | Repositório da Universidade de Lisboa |
| _version_ | 1866810501765267456 |
|---|---|
| author | Cazeiro, Daniel |
| 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 |
| 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 |
| contributor_name_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| country_str | PT |
| creators_json_txt | [{\"Person.name\":\"Cazeiro, Daniel\",\"Person.identifier.orcid\":\"0009-0004-0277-7398\"},{\"Person.name\":\"Plácido, Rui\",\"Person.identifier.orcid\":\"0000-0003-4164-5481\"},{\"Person.name\":\"Azaredo Raposo, Miguel\",\"Person.identifier.orcid\":\"0000-0002-1744-8843\"},{\"Person.name\":\"Brito, Joana\",\"Person.identifier.orcid\":\"0000-0001-6672-7542\"},{\"Person.name\":\"Borba, Alexandra\"},{\"Person.name\":\"Guimarães, Tatiana\",\"Person.identifier.orcid\":\"0000-0001-7054-0465\"},{\"Person.name\":\"Pinto, Eugénia\"},{\"Person.name\":\"Freitas, Pedro\"},{\"Person.name\":\"Pinto, Fausto J.\",\"Person.identifier.orcid\":\"0000-0002-8034-4529\"}] |
| datacite.contributors.contributor.contributorName.fl_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| datacite.creators.creator.creatorName.fl_str_mv | Cazeiro, Daniel Plácido, Rui Azaredo Raposo, Miguel Brito, Joana Borba, Alexandra Guimarães, Tatiana Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. |
| datacite.date.Accepted.fl_str_mv | 2024-01-01T00:00:00Z |
| datacite.date.available.fl_str_mv | 2024-06-12T14:48:24Z |
| datacite.date.embargoed.fl_str_mv | 2024-06-12T14:48:24Z |
| datacite.rights.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| datacite.subjects.subject.fl_str_mv | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| datacite.titles.title.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 |
| dc.contributor.none.fl_str_mv | Repositório Científico de Acesso Aberto da ULisboa |
| dc.creator.none.fl_str_mv | Cazeiro, Daniel Plácido, Rui Azaredo Raposo, Miguel Brito, Joana Borba, Alexandra Guimarães, Tatiana Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. |
| dc.date.Accepted.fl_str_mv | 2024-01-01T00:00:00Z |
| dc.date.available.fl_str_mv | 2024-06-12T14:48:24Z |
| dc.date.embargoed.fl_str_mv | 2024-06-12T14:48:24Z |
| dc.format.none.fl_str_mv | application/pdf |
| dc.identifier.none.fl_str_mv | http://hdl.handle.net/10451/65034 |
| dc.language.none.fl_str_mv | eng |
| dc.publisher.none.fl_str_mv | Sociedade Brasileira de Cardiologia - SBC |
| dc.rights.cclincense.fl_str_mv | http://creativecommons.org/licenses/by/4.0/ |
| dc.rights.none.fl_str_mv | http://purl.org/coar/access_right/c_abf2 |
| dc.subject.none.fl_str_mv | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| dc.title.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 |
| dc.type.none.fl_str_mv | http://purl.org/coar/resource_type/c_6501 |
| description | Pulmonary 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. |
| dirty | 0 |
| eu_rights_str_mv | openAccess |
| format | article |
| fulltext.url.fl_str_mv | https://repositorio.ulisboa.pt/bitstreams/a94d8bae-e133-454d-979b-3a0fc4ccfc9f/download |
| id | ul_e4c1dc90bc452e5cbe4dfca37cf71d3b |
| identifier.url.fl_str_mv | http://hdl.handle.net/10451/65034 |
| instacron_str | ul |
| institution | Universidade de Lisboa |
| instname_str | Universidade de Lisboa |
| language | eng |
| network_acronym_str | ul |
| network_name_str | Repositório da Universidade de Lisboa |
| oai_identifier_str | oai:repositorio.ulisboa.pt:10451/65034 |
| organization_str_mv | urn:organizationAcronym:ul |
| person_str_mv | Cazeiro, Daniel Cazeiro, Daniel http://orcid.org/0009-0004-0277-7398 0009-0004-0277-7398 Plácido, Rui Plácido, Rui https://www.ciencia-id.pt/1A11-7BF5-0D37 1A11-7BF5-0D37 http://orcid.org/0000-0003-4164-5481 0000-0003-4164-5481 Azaredo Raposo, Miguel Azaredo Raposo, Miguel https://www.ciencia-id.pt/201D-88DC-5C86 201D-88DC-5C86 http://orcid.org/0000-0002-1744-8843 0000-0002-1744-8843 Brito, Joana Brito, Joana https://www.ciencia-id.pt/281C-A194-3753 281C-A194-3753 http://orcid.org/0000-0001-6672-7542 0000-0001-6672-7542 Borba, Alexandra Guimarães, Tatiana Guimarães, Tatiana http://orcid.org/0000-0001-7054-0465 0000-0001-7054-0465 Pinto, Eugénia Freitas, Pedro Pinto, Fausto J. Pinto, Fausto J. https://www.ciencia-id.pt/C311-AEDD-6DBB C311-AEDD-6DBB http://orcid.org/0000-0002-8034-4529 0000-0002-8034-4529 |
| publishDate | 2024 |
| publisher.none.fl_str_mv | Sociedade Brasileira de Cardiologia - SBC |
| reponame_str | Repositório da Universidade de Lisboa |
| repository_id_str | urn:repositoryAcronym:ul |
| service_str_mv | urn:repositoryAcronym:ul |
| spelling | engSociedade Brasileira de Cardiologia - SBCpt_PTPulmonary 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.application/pdfpt_PTPulmonary arterial hypertension with features of venous involvement: a detective's taskAlternativeTitlept_PTHipertensão arterial pulmonar com características de envolvimento venoso: um trabalho investigativoPersonalCazeiro, DanielDSpacehttp://dspace.org/items/1429ae42-ed17-4df5-8b40-b950cbbd8562DSpacehttp://dspace.org/items/1429ae42-ed17-4df5-8b40-b950cbbd8562CazeiroDanielORCIDhttp://orcid.org0009-0004-0277-7398PersonalPlácido, RuiDSpacehttp://dspace.org/items/f94a2173-a4ff-4460-b47d-194f917cb98bDSpacehttp://dspace.org/items/f94a2173-a4ff-4460-b47d-194f917cb98bPlácidoRuiCiência IDhttps://www.ciencia-id.pt1A11-7BF5-0D37ORCIDhttp://orcid.org0000-0003-4164-5481Scopus Author IDhttps://www.scopus.com18438045300PersonalAzaredo Raposo, MiguelDSpacehttp://dspace.org/items/0acbacea-532e-410d-894b-924fbabe662aDSpacehttp://dspace.org/items/0acbacea-532e-410d-894b-924fbabe662aAzaredo RaposoMiguelCiência IDhttps://www.ciencia-id.pt201D-88DC-5C86ORCIDhttp://orcid.org0000-0002-1744-8843PersonalBrito, JoanaDSpacehttp://dspace.org/items/3298f703-5e47-4fdd-9d3e-2f643355df53DSpacehttp://dspace.org/items/3298f703-5e47-4fdd-9d3e-2f643355df53BritoJoanaCiência IDhttps://www.ciencia-id.pt281C-A194-3753ORCIDhttp://orcid.org0000-0001-6672-7542Researcher IDhttps://www.researcherid.comH-8224-2016Borba, AlexandraPersonalGuimarães, TatianaDSpacehttp://dspace.org/items/bee0ac18-190d-43ca-9d70-dc16f5655515DSpacehttp://dspace.org/items/bee0ac18-190d-43ca-9d70-dc16f5655515GuimarãesTatianaORCIDhttp://orcid.org0000-0001-7054-0465Pinto, EugéniaFreitas, PedroPersonalPinto, Fausto J.DSpacehttp://dspace.org/items/5f44176f-69f5-482c-83cd-ab94425a6ec3DSpacehttp://dspace.org/items/5f44176f-69f5-482c-83cd-ab94425a6ec3PintoFausto J.Ciência IDhttps://www.ciencia-id.ptC311-AEDD-6DBBORCIDhttp://orcid.org0000-0002-8034-4529Researcher IDhttps://www.researcherid.comG-9363-2015Researcher IDhttps://www.researcherid.comGQA-4584-2022Scopus Author IDhttps://www.scopus.com7102740158HostingInstitutionOrganizationalRepositório Científico de Acesso Aberto da ULisboae-mailmailto:repositorio@reitoria.ulisboa.ptrepositorio@reitoria.ulisboa.ptISSNIsPartOf0066-782XDOIIsPartOf10.36660/abc.202305652024-06-12T14:48:24Z20242024-01-01T00:00:00ZHandlehttp://hdl.handle.net/10451/65034http://purl.org/coar/access_right/c_abf2open accessPulmonary arterial hypertensionPulmonary veno-oclusive diseaseLung transplantationHemangioma capillaryTomography-X-Ray ComputedSurgery360249 bytesliteraturehttp://purl.org/coar/resource_type/c_6501journal article2024http://creativecommons.org/licenses/by/4.0/http://purl.org/coar/access_right/c_abf2application/pdffulltexthttps://repositorio.ulisboa.pt/bitstreams/a94d8bae-e133-454d-979b-3a0fc4ccfc9f/downloadArquivos Brasileiros de Cardiologia1214 |
| 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 |
| status | SINGLETON |
| subject.fl_str_mv | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| title | 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_short | 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 |
| topic | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| topic_facet | Pulmonary arterial hypertension Pulmonary veno-oclusive disease Lung transplantation Hemangioma capillary Tomography-X-Ray Computed Surgery |
| url | http://hdl.handle.net/10451/65034 |
| visible | 1 |