{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T11:54:21Z","timestamp":1792410861785,"version":"4.4.0"},"reference-count":8,"publisher":"BMJ","issue":"2","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2024,1,23]]},"published-print":{"date-parts":[[2024,2]]},"abstract":"<jats:p>Unlike acute pulmonary embolism (PE), the resolution of thrombi is ineffective in chronic thromboembolic pulmonary hypertension (CTEPH), leading to reorganisation and fibrotic changes within the pulmonary arteries. The authors report the case of a man in his 60s with polycythemia vera, under warfarin, following an acute PE. He was admitted a year later with right heart failure and haemodynamic instability. Acute over chronic PE caused this severe presentation, confirmed by right heart catheterisation and pulmonary scintigraphy. The challenging diagnosis and management involved transfer to a centre specialised in pulmonary vascular disease. Normalisation of functional and haemodynamic parameters, sustained in 10-year follow-up, was achieved with anticoagulation and triple therapy with prostanoids as a bridge to pulmonary thromboendarterectomy. Targeted medical therapy, not standard at that time, was crucial to recovering conditions for transfer. An individualised approach, integrating multidisciplinary pulmonary hypertension expertise, provides the basis for the best care for CTEPH.<\/jats:p>","DOI":"10.1136\/bcr-2023-257942","type":"journal-article","created":{"date-parts":[[2024,2,6]],"date-time":"2024-02-06T12:49:52Z","timestamp":1707223792000},"page":"e257942","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["10-year success story in CTEPH treatment: breaking the myth of rareness"],"prefix":"10.1136","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9832-6348","authenticated-orcid":false,"given":"Sofia","family":"Moura de Azevedo","sequence":"first","affiliation":[{"name":"Internal Medicine Department, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3985-0718","authenticated-orcid":false,"given":"In\u00eas","family":"Furtado","sequence":"additional","affiliation":[{"name":"Internal Medicine Department; Pulmonary Vascular Disease Unit, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lu\u00edsa","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Internal Medicine Department; Pulmonary Vascular Disease Unit, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ab\u00edlio","family":"Reis","sequence":"additional","affiliation":[{"name":"Pulmonary Vascular Disease Unit, Centro Hospitalar Universitario de Santo Antonio, Porto, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2024,2,6]]},"reference":[{"key":"2025082713030495000_17.2.e257942.1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"2025082713030495000_17.2.e257942.2","doi-asserted-by":"crossref","unstructured":"Kim NH , Delcroix M , Jais X , et al . Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019;53. doi:10.1183\/13993003.01915-2018","DOI":"10.1183\/13993003.01915-2018"},{"key":"2025082713030495000_17.2.e257942.3","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1183\/16000617.0108-2016","article-title":"Diagnosis of chronic thromboembolic pulmonary hypertension","volume":"26","author":"Gopalan","year":"2017","journal-title":"Eur Respir Rev"},{"key":"2025082713030495000_17.2.e257942.4","doi-asserted-by":"crossref","DOI":"10.3390\/diagnostics12020235","article-title":"Chronic thromboembolic pulmonary hypertension: An update","volume":"12","author":"Ruaro","year":"2022","journal-title":"Diagnostics (Basel)"},{"key":"2025082713030495000_17.2.e257942.5","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1016\/j.repc.2018.02.009","article-title":"Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal","volume":"37","author":"Santos","year":"2018","journal-title":"Rev Port Cardiol (Engl Ed)"},{"key":"2025082713030495000_17.2.e257942.6","doi-asserted-by":"crossref","unstructured":"Kulkarni SK , Bhairappa S , Bishnoi A , et al . Chronic thromboembolic pulmonary hypertension in a patient with polycythemia vera. BMJ Case Rep 2018. doi:10.1136\/bcr-2018-225764","DOI":"10.1136\/bcr-2018-225764"},{"key":"2025082713030495000_17.2.e257942.7","doi-asserted-by":"crossref","unstructured":"Boucly A , Weatherald J , Savale L , et al . External validation of a refined four-stratum risk assessment score from the French pulmonary hypertension registry. Eur Respir J 2022;59. doi:10.1183\/13993003.02419-2021","DOI":"10.1183\/13993003.02419-2021"},{"key":"2025082713030495000_17.2.e257942.8","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.019470"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2023-257942","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T20:03:13Z","timestamp":1756324993000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2023-257942"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,2]]},"references-count":8,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2024,2,6]]},"published-print":{"date-parts":[[2024,2]]}},"alternative-id":["10.1136\/bcr-2023-257942"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2023-257942","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,2]]}}}