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Review
. 2025 May 27:21:100594.
doi: 10.1016/j.ijcchd.2025.100594. eCollection 2025 Sep.

Pulmonary arterial hypertension treatment. A new era

Sophia Anastasia Mouratoglou  1 , Alexandra Arvanitaki  2 , George Papadopoulos  3 , Rogerio Souza  4 , George Giannakoulas  3
Affiliations
Review

Pulmonary arterial hypertension treatment. A new era

Sophia Anastasia Mouratoglou et al. Int J Cardiol Congenit Heart Dis. .

Abstract

Pulmonary arterial hypertension (PAH) is a progressive and life-threatening condition characterized by elevated pulmonary vascular resistance, ultimately leading to right heart failure and death. Advances in the understanding of PAH pathophysiology have driven the development of targeted therapies that improve functional capacity, quality of life and survival. The current treatment paradigm emphasizes early and aggressive therapy, with combination therapy now considered standard of care, offering superior outcomes compared to monotherapy. Herewith, we examine the underlying pathophysiological mechanisms of PAH and explore the modes of action and clinical efficacy of major therapeutic classes, providing a comprehensive overview of contemporary treatment strategies.

Keywords: Endothelin; Nitric oxide; Prostacyclin; Pulmonary arterial hypertension; Risk stratification; Sotatercept; Treatment.

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Conflict of interest statement

The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Sophia Anastasia Mouratoglou reports a relationship with Galenica Pharmaceuticals Inc that includes: travel reimbursement. Sophia Anastasia Mouratoglou reports a relationship with MSD that includes: speaking and lecture fees and travel reimbursement. Sophia Anastasia Mouratoglou reports a relationship with Janssen Pharmaceuticals Inc that includes: speaking and lecture fees. George Giannakoulas reports a relationship with Elpen Pharmaceuticals that includes: consulting or advisory and speaking and lecture fees. George Giannakoulas reports a relationship with Galenica Pharmaceuticals Inc that includes: consulting or advisory and speaking and lecture fees. George Giannakoulas reports a relationship with 10.13039/100004330GlaxoSmithKline; Janssen Pharmaceutical that includes: consulting or advisory and speaking and lecture fees. George Giannakoulas reports a relationship with 10.13039/100005205Janssen Pharmaceutical Companies of Johnson & Johnson that includes: consulting or advisory and speaking and lecture fees. George Giannakoulas reports a relationship with MSD that includes: speaking and lecture fees. Rogerio Souza reports a relationship with Bayer Corporation that includes: consulting or advisory. Rogerio Souza reports a relationship with Janssen Pharmaceuticals Inc that includes: consulting or advisory. Rogerio Souza reports a relationship with Pulmovant Inc that includes: consulting or advisory. Rogerio Souza reports a relationship with MSD that includes: consulting or advisory. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1
Fig. 1
Pathophysiology of pulmonary arterial hypertension. PAH: pulmonary arterial hypertension.
Fig. 2
Fig. 2
The effect of pharmacotherapy on the pathophysiology of pulmonary arterial hypertension.
Fig. 3
Fig. 3
Risk assessment and treatment algorithm of pulmonary arterial hypertension. FC: functional class, 6MWD: 6 min walk distance, ERA: endothelin receptor antagonist, mPAP: mean pulmonary arterial pressure, PAH: pulmonary arterial hypertension, PDE5i: phosphodiesterase 5 inhibitor, PPA: prostacyclin pathway agents, PVR: pulmonary vascular resistance.
See this image and copyright information in PMC

References

    1. Farmakis I.T., Vrana E., Mouratoglou S.A., Zafeiropoulos S., Zanos S., Giannakoulas G. Haemodynamic effects of initial combination therapy in pulmonary arterial hypertension: a systematic review and meta-analysis. ERJ Open Res. 2022;8(4) - PMC - PubMed
    1. Gabler N.B., French B., Strom B.L., Palevsky H.I., Taichman D.B., Kawut S.M., et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation (New York, N Y) 2012;126(3):349–356. - PMC - PubMed
    1. Savarese G., Paolillo S., Costanzo P., D'Amore C., Cecere M., Losco T., et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials. J Am Coll Cardiol. 2012;60(13):1192–1201. - PubMed
    1. Humbert M., Farber H.W., Ghofrani H.-A., Benza R.L., Busse D., Meier C., et al. Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eur Respir J. 2019;53(6) - PMC - PubMed
    1. Humbert M., Sitbon O., Guignabert C., Savale L., Boucly A., Gallant-Dewavrin M., et al. Treatment of pulmonary arterial hypertension: recent progress and a look to the future. Lancet Respir Med. 2023;11(9):804–819. - PubMed

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