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Pathways and Drugs in Pulmonary Arterial Hypertension – Focus on the Role of Endothelin Receptor Antagonists

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Abstract

Pulmonary arterial hypertension (PAH) is a group of diseases characterized by a progressive increase of pulmonary vascular resistance (PVR), initially due to abnormal pulmonary vasoconstriction in response to endothelial injury. Recent studies have here confirmed the prominent role of endothelin (ET)-1 in vasoconstriction and remodelling of the pulmonary microcirculation. In patients who are acute-vasoreactive, classical treatments for PAH are calcium channels blockers (CCBs), while drugs targeting the prostacyclin, nitric oxide and endothelin pathways, i.e., prostanoids, phosphodiesterase (PDE)-5 inhibitors and endothelin receptor antagonists (ERAs), respectively, are indicated in non-vasoreactive patients or in vasoreactive patients not responding to initial CCB therapy. Randomised, placebo-controlled trials have shown that ERAs improve pulmonary haemodynamics, exercise capacity, functional status and clinical outcome in patients with PAH. Here we provide an overview of the currently recommended diagnostic and therapeutic work-up in PAH, with special emphasis on ERAs.

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Acknowledgments

The authors thank all patients afferent to the Center for the Study of Pulmonary Hypertension at “SS. Annunziata” Hospital, Chieti–Italy, and their families, for inspiring this review work.

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The authors declare no conflicts of interest.

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Authors and Affiliations

  1. Institute of Cardiology, Center of Excellence on Aging, “G. d’Annunzio” University, Chieti, Italy

    Rosalinda Madonna & Raffaele De Caterina

  2. Department “Cuore e Grossi Vasi Attilio Reale”, “Sapienza” University, Policlinico Umberto I, Rome, Italy

    Nino Cocco

  3. Institute of Cardiology, “G. d’Annunzio” University – Chieti, C/o Centro Scienze dell’Invecchiamento CESI, Via dei Vestini, 66013, Chieti, Italy

    Raffaele De Caterina

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Madonna, R., Cocco, N. & De Caterina, R. Pathways and Drugs in Pulmonary Arterial Hypertension – Focus on the Role of Endothelin Receptor Antagonists. Cardiovasc Drugs Ther 29, 469–479 (2015). https://doi.org/10.1007/s10557-015-6605-6

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