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Review
. 2016 Nov 24:5:2755.
doi: 10.12688/f1000research.9739.1. eCollection 2016.

Recent advances in the management of pulmonary arterial hypertension

Halley Tsai #  1 , Yon K Sung #  1   2 , Vinicio de Jesus Perez  1   2
Affiliations
Review

Recent advances in the management of pulmonary arterial hypertension

Halley Tsai et al. F1000Res. .

Abstract

Over the past 20 years, there has been an explosion in the development of therapeutics to treat pulmonary arterial hypertension (PAH), a rare but life-threatening disorder associated with progressive elevation of pulmonary pressures and severe right heart failure. Recently, the field has seen the introduction of riociguat, a soluble guanylate cyclase stimulator, a new endothelin receptor antagonist (macitentan), and oral prostanoids (treprostinil and selexipag). Besides new drugs, there have been significant advances in defining the role of upfront combination therapy in treatment-naïve patients as well as proposed methods to deliver systemic prostanoids by use of implantable pumps. In this review, we will touch upon the most important developments in PAH therapeutics over the last three years and how these have changed the guidelines for the treatment of PAH. These exciting developments herald a new era in the treatment of PAH which will be punctuated by the use of more clinically relevant endpoints in clinical research trials and a novel treatment paradigm that may involve upfront double- or triple-combination therapy. We anticipate that the future will make use of these strategies to test the efficacy of upcoming new drugs that aspire to reduce disease progression and improve survival in patients afflicted with this devastating disease.

Keywords: macitentan; pulmonary arterial hypertension; riociguat; selexipag; treprostinil.

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Conflict of interest statement

The authors declare that they have no competing interests. Competing interests: Dr. Maron receives financial support from Gilead Sciences to research Pulmonary Hypertension. No competing interests were disclosed.

Figures

Figure 1.
Figure 1.. Pathways targeted in current therapies for pulmonary arterial hypertension.
Newly approved therapies are listed in blue. cAMP, cyclic adenosine monophosphate; cGMP, cyclic guanylate monophosphate; ERA, endothelin receptor antagonist; FDA, US Food and Drug Administration; INH, inhaled; IP 2, prostacyclin receptor 2; IV, intravenous; NO, nitric oxide; PAH, pulmonary arterial hypertension; PDE-5, phosphodiesterase-5; PDE-5i, phosphodiesterase-5 inhibitor; PGI 2, prostaglandin I 2; sGC, soluble guanylate cyclase; SQ, subcutaneous.
Figure 2.
Figure 2.. Treatment algorithm from the 2015 European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension.
Reproduced with permission from the European Respiratory Society and European Society of Cardiology .
See this image and copyright information in PMC

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