eprintid: 10197075 rev_number: 9 eprint_status: archive userid: 699 dir: disk0/10/19/70/75 datestamp: 2024-09-17 15:46:39 lastmod: 2024-09-17 15:46:39 status_changed: 2024-09-17 15:46:39 type: article metadata_visibility: show sword_depositor: 699 creators_name: Kovacs, Gabor creators_name: Bartolome, Sonja creators_name: Denton, Christopher P creators_name: Gatzoulis, Michael A creators_name: Gu, Sue creators_name: Khanna, Dinesh creators_name: Badesch, David creators_name: Montani, David title: Definition, classification and diagnosis of pulmonary hypertension ispublished: pub divisions: UCL divisions: B02 divisions: C10 divisions: D17 divisions: G90 note: Copyright ©The authors 2024. This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions@ersnet.org abstract: Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) >20 mmHg, assessed by right heart catheterisation. Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP ≤15 mmHg, PVR >2 Wood Units (WU)), isolated post-capillary PH (PAWP >15 mmHg, PVR ≤2 WU) and combined post- and pre-capillary PH (PAWP >15 mmHg, PVR >2 WU). 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 >3 mmHg/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 "long-term responders to calcium channel blockers" 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 "definite association" 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. date: 2024-08-29 date_type: published publisher: European Respiratory Society (ERS) official_url: http://dx.doi.org/10.1183/13993003.01324-2024 oa_status: green full_text_type: pub language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 2309617 doi: 10.1183/13993003.01324-2024 medium: Print-Electronic pii: 13993003.01324-2024 lyricists_name: Denton, Christopher lyricists_id: CPDEN87 actors_name: Denton, Christopher actors_id: CPDEN87 actors_role: owner full_text_status: public publication: European Respiratory Journal volume: 64 number: 3 event_location: England issn: 0903-1936 citation: Kovacs, Gabor; Bartolome, Sonja; Denton, Christopher P; Gatzoulis, Michael A; Gu, Sue; Khanna, Dinesh; Badesch, David; Kovacs, Gabor; Bartolome, Sonja; Denton, Christopher P; Gatzoulis, Michael A; Gu, Sue; Khanna, Dinesh; Badesch, David; Montani, David; - view fewer <#> (2024) Definition, classification and diagnosis of pulmonary hypertension. European Respiratory Journal , 64 (3) 10.1183/13993003.01324-2024 <https://doi.org/10.1183/13993003.01324-2024>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10197075/1/Denton_13993003.01324-2024.full.pdf