{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T11:48:36Z","timestamp":1753876116409,"version":"3.41.2"},"reference-count":5,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2022,3,28]],"date-time":"2022-03-28T00:00:00Z","timestamp":1648425600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Instituto Carlos III"},{"DOI":"10.13039\/501100003329","name":"Ministerio de Econom\u00eda y Competitividad","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,5,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension (PAH) where vascular remodelling affects mainly the post-capillary vessels. It is characterized by a particularly worse prognosis and by the risk of developing life-threatening pulmonary oedema, especially after PAH-targeted therapy. Therefore, suspicion of PVOD is crucial to guide the patient\u2019s management. In the absence of specific genetic or histological findings, diagnosis has traditionally relied on the recognition of non-invasive indicators associated with a high likelihood of PVOD. The cardiopulmonary exercise testing (CPET) arises as a promising additional tool both to identify these patients and to guide their management.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>We report the case of a young female patient with dyspnoea and clinical suspicion of PVOD. The diagnostic workup is thoroughly described stressing the valuable and readily accessible information that CPET can provide, in addition to the data of radiological and lung function tests. Once diagnosed, she was started on PAH-targeted therapy with subsequent improvement. The patient underwent a complete reassessment with satisfactory findings, including those of the CPET.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>Pulmonary veno-occlusive disease diagnosis is still one of the most difficult tasks that pulmonary hypertension physicians have to deal with. An accurate and timely PVOD diagnosis can be challenging, as it is to decide the most appropriate timing of referal to the lung transplant team, and CPET may serve these purposes. Through this case, we would like to review one of the typical clinical courses that PVOD may present and how to analyse the information provided by the diagnostic tests.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytac138","type":"journal-article","created":{"date-parts":[[2022,3,29]],"date-time":"2022-03-29T16:16:23Z","timestamp":1648570583000},"source":"Crossref","is-referenced-by-count":0,"title":["The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0269-5574","authenticated-orcid":false,"given":"Teresa","family":"Segura de la Cal","sequence":"first","affiliation":[{"name":"Department of Cardiology, Hospital Universitario 12 de Octubre , Madrid, Spain"},{"name":"Ciber-CV Centro de investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Cardiovasculares , Madrid, Spain"},{"name":"Centro de Referencia Nacional de Hipertensi\u00f3n Pulmonar Compleja , Madrid, Spain"},{"name":"ERN-Lung-Pulmonary Hypertension Referal Center , Madrid, Spain"},{"name":"Centro de Referencia nacional de Cardiopat\u00edas Cong\u00e9nitas del Adulto , Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital Universitario 12 de Octubre (Imas12), Red SAMID , Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmen","family":"P\u00e9rez-Olivares","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Universitario 12 de Octubre , Madrid, Spain"},{"name":"Ciber-CV Centro de investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Cardiovasculares , Madrid, Spain"},{"name":"Centro de Referencia Nacional de Hipertensi\u00f3n Pulmonar Compleja , Madrid, Spain"},{"name":"ERN-Lung-Pulmonary Hypertension Referal Center , Madrid, Spain"},{"name":"Centro de Referencia nacional de Cardiopat\u00edas Cong\u00e9nitas del Adulto , Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda Jos\u00e9","family":"Cristo Ropero","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital de Torrej\u00f3n , Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raquel","family":"Luna L\u00f3pez","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Universitario 12 de Octubre , Madrid, Spain"},{"name":"Ciber-CV Centro de investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Cardiovasculares , Madrid, Spain"},{"name":"Centro de Referencia Nacional de Hipertensi\u00f3n Pulmonar Compleja , Madrid, Spain"},{"name":"ERN-Lung-Pulmonary Hypertension Referal Center , Madrid, Spain"},{"name":"Centro de Referencia nacional de Cardiopat\u00edas Cong\u00e9nitas del Adulto , Madrid, Spain"},{"name":"Instituto de Investigaci\u00f3n Sanitaria del Hospital Universitario 12 de Octubre (Imas12), Red SAMID , Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pilar","family":"Escribano-Sub\u00edas","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Hospital Universitario 12 de Octubre , Madrid, Spain"},{"name":"Ciber-CV Centro de investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Cardiovasculares , Madrid, Spain"},{"name":"Centro de Referencia Nacional de Hipertensi\u00f3n Pulmonar Compleja , Madrid, Spain"},{"name":"ERN-Lung-Pulmonary Hypertension Referal Center , Madrid, Spain"},{"name":"Centro de Referencia nacional de Cardiopat\u00edas Cong\u00e9nitas del Adulto , Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2022,3,28]]},"reference":[{"key":"2022071812331892000_ytac138-B1","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","year":"2008","journal-title":"Medicine"},{"key":"2022071812331892000_ytac138-B2","doi-asserted-by":"crossref","first-page":"1518","DOI":"10.1183\/13993003.00026-2016","article-title":"Pulmonary veno-occlusive disease","volume":"47","author":"Montani","year":"2016","journal-title":"Eur Respir J"},{"key":"2022071812331892000_ytac138-B3","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1016\/j.recesp.2017.03.030","article-title":"Variable expressivity of a founder mutation in the EIF2AK4 gene in hereditary pulmonary veno-occlusive disease and its impact on survival","volume":"71","author":"Navas Tejedor","year":"2018","journal-title":"Rev Esp Cardiol (Engl Ed)"},{"key":"2022071812331892000_ytac138-B4","doi-asserted-by":"crossref","first-page":"2100115","DOI":"10.1183\/13993003.00115-2021","article-title":"The role of cardiopulmonary exercise test in identifying pulmonary 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