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Prostacyclin Therapy for Pulmonary Arterial Hypertension

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Diagnosis and Management of Pulmonary Hypertension

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

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Abstract

Prostacyclins are the first class of agents that showed efficacy in the treatment of pulmonary arterial hypertension (PAH) in a clinical trial and, for a long time, the only treatment specifically approved for this devastating disease. As new therapies have emerged for PAH in recent years, the class of prostacyclins has also evolved and several formulations of different prostacyclin analogs, using various routes of delivery, have been tested and shown to be efficacious in PAH. This chapter reviews the pharmacology of prostacyclins and the results of pivotal clinical trials testing various formulations. Lastly, it examines practical aspects of dosing, management of side effects, and expectations of treatment goals.

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Abbreviations

6MWD:

6 min walk distance

cAMP:

Cyclic AMP

HETE:

Hydroxyeicotetraenoic acid

HIV:

Human immunodeficiency virus

IPAH:

Idiopathic pulmonary arterial hypertension

LOX:

Lipoxygenase

mPAP:

Mean pulmonary artery pressure

NIH:

National Institutes of Health

NYHA:

New York Heart Association

PAH:

Pulmonary arterial hypertension

PG:

Prostaglandin

PGI2 :

Prostacyclin

PH:

Pulmonary hypertension

PVR:

Pulmonary vascular resistance

TXA2 :

Thromboxane A2

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Correspondence to Ioana R. Preston M.D. .

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Preston, I.R. (2015). Prostacyclin Therapy for Pulmonary Arterial Hypertension. 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_13

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  • DOI: https://doi.org/10.1007/978-1-4939-2636-7_13

  • Publisher Name: Humana Press, New York, NY

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