Impact of targeted pulmonary arterial hypertension therapies in severe pulmonary hypertension in chronic lung diseases

Autor: Romain Naud, Julien Bermudez, Noémie Resseguier, Ana Nieves, Bérengère Coltey, Nadine Dufeu, Clarisse Gautier, Youssef Trigui, Marc Laine, Benjamin Coiffard, Martine Reynaud-Gaubert
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: ERJ Open Research, Vol 9, Iss 4 (2023)
Druh dokumentu: article
ISSN: 2312-0541
23120541
DOI: 10.1183/23120541.00027-2023
Popis: Research questions Patients with severe pulmonary hypertension associated with chronic lung disease have a poor prognosis. Targeted pulmonary arterial hypertension therapies might improve exercise capacity and outcome, but there are no guidelines on treatments which are not recommended because of an unproven benefit, with discordant results from few studies in this context. The aim of our study was to evaluate targeted pulmonary arterial hypertension therapies for severe group 3 pulmonary hypertension patients. Study design and methods We conducted an observational retrospective monocentre study on patients with severe group 3 pulmonary hypertension diagnosed on right heart catheterisation treated with targeted therapies. Primary outcome was an improvement of the distance on 6-min walk test of ≥30 m. Secondary end-points included changes in haemodynamics (pulmonary vascular resistance (PVR) and mean pulmonary arterial pressure (mPAP)) and identification of potential predictive factors of therapeutic response. Results 139 patients were enrolled. Most patients had monotherapy with phosphodiesterase 5 inhibitors (n=128; 92%). Mean change in 6-min walk distance was +1.5 m after treatment (p=0.59). Forced expiratory volume in 1 s and forced vital capacity were not predictive factors for response. We found a significant improvement of PVR and mPAP of −1.0 Wood Units (p
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