New pharmacotherapy options for pulmonary arterial hypertension

Abstract

Introduction: Epoprostenol was the first targeted therapy available for the treatment of pulmonary arterial hypertension (PAH). Since then great advances in our knowledge of the disease have been made and the spectrum of therapeutic options for PAH has expanded. After an overview of current available treatments, this article describes the new pharmacotherapy options and their place in the management of PAH.

Areas covered: This paper is based on a literature search and the review of studies published on PAH pharmacotherapy using the MEDLINE database.

Expert opinion: The last decade has been particularly important in PAH management with the emergence of six new molecules, the development of novel routes of administration and improvement of pharmacokinetics. Moreover, pediatric formulations have been developed. However, further research is required to inform clinicians regarding optimal choices of combination therapies (progressive add-on therapy or upfront combination therapy, selection of associated molecules regarding the patient’s profile...), to continue to improve the quality of life of patients with new drugs and to reach the ultimate goal of curing the disease.

Keywords:

• endothelin pathway
• nitric oxide pathway
• prostacyclin pathway
• pulmonary arterial hypertension
• updated treatment recommendations

Declaration of interest

O Sitbon has relationships with Actelion, Bayer, GlaxoSmithKline, Pfizer and United Therapeutics. In addition to being an investigator involving these compounds, relationships include consulting services, fees for lectures and funds for research. L Savale is a member of scientific advisory boards and has received lecture fees from Actelion, Bayer, GlaxoSmithKline and Pfizer. X Jaïs is a member of scientific advisory boards for and has received lecture fees from Actelion, Bayer, GlaxoSmithKline and Pfizer. D Montani is a member of scientific advisory boards and has received lecture fees from Actelion, Bayer, GlaxoSmithKline and Novartis. M Humbert has relationships with Actelion, Bayer, GlaxoSmithKline, Novartis, Pfizer and Gilead. In addition to being an investigator in trials involving these compounds, he has received grants for research, lecture and consultancy fees. G Simonneau has relationships with Actelion, Bayer, GlaxoSmithKline, Novartis and Pfizer. In addition to being an investigator in trials involving these compounds, he has received consultancy fees, fees for lectures and funds for research. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Notes

This box summarizes key points contained in the article.