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Endothelin Receptor Antagonists

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Book cover Diagnosis and Management of Pulmonary Hypertension

Part of the book series: Respiratory Medicine ((RM,volume 12))

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Abstract

Endothelin-1 (ET-1) is a potent vasoconstrictor and mitogen that is secreted by the endothelium. Pulmonary vascular expression of endothelin is increased in pulmonary hypertension and plasma levels correlate with the severity of disease. Several endothelin receptor antagonists (ERAs) have been developed and are available for the treatment of pulmonary arterial hypertension (PAH). These drugs have been shown to be effective at improving functional capacity, decreasing pulmonary vascular resistance and delaying the time to clinical worsening and play an important role in the management of PAH. This chapter briefly reviews how the endothelin signaling pathway modulates pulmonary vascular function and describes its role in the pathogenesis of PAH. The major clinical trials responsible for the currently available ERAs are presented along with their findings and limitations. Recently, adopted treatment guidelines for the use of ERAs in the treatment of pulmonary hypertensive disease are discussed along with potential side effects and adverse reactions associated with the use of these drugs.

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Abbreviations

6MWD:

6-min Walk distance

CTEPH:

Chronic thromboembolic pulmonary hypertension

ERA:

Endothelin receptor antagonist

ET-1:

Endothelin-1

ET-2:

Endothelin-2

ET-3:

Endothelin-3

ETA :

Endothelin receptor A

ETB :

Endothelin receptor B

IPAH:

Idiopathic pulmonary arterial hypertension

mPAP:

Mean pulmonary artery pressure

NO:

Nitric oxide

PAH:

Pulmonary arterial hypertension

PPET-1:

Preproendothelin

PVR:

Pulmonary vascular resistance

TPR:

Total pulmonary resistance

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Authors and Affiliations

  1. Pulmonary and Critical Care Division, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114-2696, USA

    Josanna Rodriguez-Lopez M.D. & Richard N. Channick M.D.

Authors
  1. Josanna Rodriguez-Lopez M.D.
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  2. Richard N. Channick M.D.
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Corresponding author

Correspondence to Richard N. Channick M.D. .

Editor information

Editors and Affiliations

  1. Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, Rhode Island, USA

    James R. Klinger

  2. Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA

    Robert P. Frantz

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Rodriguez-Lopez, J., Channick, R.N. (2015). Endothelin Receptor Antagonists. In: Klinger, J., Frantz, R. (eds) Diagnosis and Management of Pulmonary Hypertension. Respiratory Medicine, vol 12. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-2636-7_14

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  • DOI: https://doi.org/10.1007/978-1-4939-2636-7_14

  • Publisher Name: Humana Press, New York, NY

  • Print ISBN: 978-1-4939-2635-0

  • Online ISBN: 978-1-4939-2636-7

  • eBook Packages: MedicineMedicine (R0)

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