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A Contemporary Approach to Pulmonary Arterial Hypertension

  • Clinical Trials and Their Interpretations (J. Kizer, Section Editor)
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Abstract

In recent years, there have been major changes in the landscape of pulmonary arterial hypertension therapy with the introduction of novel agents and innovative treatment strategies for this progressive disease. The aim of this review is to discuss the evolution in trial design in this field and highlight the salient features of recently published studies. We also summarize our approach to therapy selection in this chronic disease and identify areas for future exploration. The therapeutic armamentarium now includes 13 approved therapies. While most of these agents have been studied in small, short-term trials using the 6-min walk distance as a primary endpoint, there has been a shift in recent years toward larger, long-term, event-driven trials that utilize combined morbidity and mortality endpoints. The SERAPHIN and GRIPHON trials were two such studies, which led to the approval of the dual endothelin-receptor antagonist macitentan and the selective prostacyclin receptor antagonist selexipag, respectively. Other event-driven trials, like AMBITION and COMPASS-2, have provided valuable insight into the use of combined oral therapies in symptomatic patients. In conclusion, despite being a more manageable disease in the modern treatment era, pulmonary hypertension is still associated with considerable morbidity and much more work remains to be done in this field. Important questions remain about the most optimal way to manage patients and conduct trials going forward.

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Authors and Affiliations

  1. Division of Cardiology, Department of Internal Medicine, Weill Cornell Medical College, New York Presbyterian Hospital, 520 East 70th Street, New York, NY, 10021, USA

    Udhay Krishnan & Evelyn M. Horn

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  1. Udhay Krishnan
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  2. Evelyn M. Horn
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Correspondence to Udhay Krishnan.

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Conflict of Interest

Udhay Krishnan declares no conflict of interest.

Evelyn M. Horn declares grant and research support from Abbott Laboratories, Inc., Actelion Pharmaceuticals, Inc., Mallinckrodt Pharmaceuticals, and Reata Pharmaceuticals. She also declares to be on the advisory boards for Abbott Laboratories, Inc., Janssen Pharmaceutical Company, Actelion Pharmaceuticals, Inc., and Bayer Healthcare Pharmaceuticals.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is a part of the Topical Collection on Clinical Trials and Their Interpretations

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Krishnan, U., Horn, E.M. A Contemporary Approach to Pulmonary Arterial Hypertension. Curr Atheroscler Rep 18, 58 (2016). https://doi.org/10.1007/s11883-016-0608-z

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