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Review
. 2019 Sep;11(Suppl 14):S1767-S1781.
doi: 10.21037/jtd.2019.09.14.

Pharmacotherapy for pulmonary arterial hypertension

Vishal Parikh  1 , Anju Bhardwaj  2 , Ajith Nair  3
Affiliations
Review

Pharmacotherapy for pulmonary arterial hypertension

Vishal Parikh et al. J Thorac Dis. 2019 Sep.

Abstract

Pulmonary arterial hypertension (PAH) is a condition associated with substantial morbidity and mortality. Over the last 25 years there has been a significant evolution in the therapies to treat PAH. These therapies are effective for patients with group I PAH and group IV PH [chronic thromboembolic pulmonary hypertension (CTEPH)]. PAH is characterized by an imbalance of nitric oxide, prostacyclin and endothelin levels, and current pharmacotherapy involves these three pathways. Earlier clinical trials involving PAH-specific therapies evaluated improvements in 6-minute walk time as a primary improvement whereas contemporary trials have been larger and focused on morbidity and mortality reductions. While there may be a role for monotherapy in disease management, most patients should be considered for dual or triple therapy.

Keywords: Pulmonary arterial hypertension (PAH); ambrisentan; bosentan; endothelin; epoprostenol; macitentan; nitric oxide; prostacyclin; riociguat; selexipag; sildenafil; tadalafil; treprostinil.

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Conflict of interest statement

Conflicts of Interest: The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Pharmacotherapy in PAH. Adapted from (2). PAH, pulmonary arterial hypertension; PDE5, phosphodiesterase-5.
Figure 2
Figure 2
Riociguat-mechanism of action. sGC, soluble guanylate cyclase; cGMP, cyclic guanosine monophosphate.
Figure 3
Figure 3
Selexipag as monotherapy of sequential therapy to PDE5 inhibitors and/or ERA. Adapted from (40). PDE5, phosphodiesterase-5.
Figure 4
Figure 4
AMBITION trial. Combination therapy (ambrisentan and tadalafil) vs. Pooled monotherapy. Adapted from (44).
Figure 5
Figure 5
Treatment algorithm in PAH. Adapted from (6). PAH, pulmonary arterial hypertension.
See this image and copyright information in PMC

References

    1. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 2019. doi: .10.1183/13993003.01913-2018 - DOI - PMC - PubMed
    1. Humbert M, Ghofrani HA. The molecular targets of approved treatments for pulmonary arterial hypertension. Thorax 2016;71:73-83. 10.1136/thoraxjnl-2015-207170 - DOI - PMC - PubMed
    1. McLaughlin VV, Shah SJ, Souza R, et al. Management of pulmonary arterial hypertension. J Am Coll Cardiol 2015;65:1976-97. 10.1016/j.jacc.2015.03.540 - DOI - PubMed
    1. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015;46:903-75. 10.1183/13993003.01032-2015 - DOI - PubMed
    1. Frost A, Badesch D, Gibbs JSR, et al. Diagnosis of pulmonary hypertension. Eur Respir J 2019. doi: .10.1183/13993003.01904-2018 - DOI - PMC - PubMed