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Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP \u226415\u2005mmHg, PVR &gt;2\u2005Wood Units (WU)), isolated post-capillary PH (PAWP &gt;15\u2005mmHg, PVR \u22642\u2005WU) and combined post- and pre-capillary PH (PAWP &gt;15\u2005mmHg, PVR &gt;2\u2005WU). Exercise PH is a haemodynamic condition describing a normal mPAP at rest with an abnormal increase of mPAP during exercise, defined as a mPAP\/cardiac output slope &gt;3\u2005mmHg\/L\/min between rest and exercise. The core structure of the clinical classification of PH has been retained, including the five major groups. However, some changes are presented herewith, such as the re-introduction of \u201clong-term responders to calcium channel blockers\u201d as a subgroup of idiopathic pulmonary arterial hypertension, the addition of subgroups in group 2 PH and the differentiation of group 3 PH subgroups based on pulmonary diseases instead of functional abnormalities. Mitomycin-C and carfilzomib have been added to the list of drugs with \u201cdefinite association\u201d with PAH. For diagnosis of PH, we propose a stepwise approach with the main aim of discerning those patients who need to be referred to a PH centre and who should undergo invasive haemodynamic assessment. In case of high probability of severe pulmonary vascular disease, especially if there are signs of right heart failure, a fast-track referral to a PH centre is recommended at any point during the clinical workup.<\/jats:p>","DOI":"10.1183\/13993003.01324-2024","type":"journal-article","created":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T05:28:12Z","timestamp":1724909292000},"page":"2401324","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":494,"title":["Definition, classification and diagnosis of pulmonary hypertension"],"prefix":"10.1183","volume":"64","author":[{"given":"Gabor","family":"Kovacs","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonja","family":"Bartolome","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3975-8938","authenticated-orcid":false,"given":"Christopher P.","family":"Denton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Gatzoulis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sue","family":"Gu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh","family":"Khanna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Badesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9358-6922","authenticated-orcid":false,"given":"David","family":"Montani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2024,8,29]]},"reference":[{"key":"2024112623054952000_64.4.2401324.1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00879-2022"},{"key":"2024112623054952000_64.4.2401324.2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehac237"},{"key":"2024112623054952000_64.4.2401324.3","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00145608"},{"key":"2024112623054952000_64.4.2401324.4","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01913-2018"},{"key":"2024112623054952000_64.4.2401324.5","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00008611"},{"key":"2024112623054952000_64.4.2401324.6","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.115.020207"},{"key":"2024112623054952000_64.4.2401324.7","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201706-1215OC"},{"key":"2024112623054952000_64.4.2401324.8","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(20)30317-9"},{"key":"2024112623054952000_64.4.2401324.9","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehad532"},{"key":"2024112623054952000_64.4.2401324.10","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00107-2022"},{"key":"2024112623054952000_64.4.2401324.11","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-022-02205-4"},{"key":"2024112623054952000_64.4.2401324.12","doi-asserted-by":"crossref","unstructured":"G\u00fcder G , Reiter T , Fette G , et al. 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