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Semin Respir Crit Care Med 2005; 26(4): 394-401
DOI: 10.1055/s-2005-916154
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Prostacyclin Therapy for Pulmonary Arterial Hypertension: New Directions

Mardi Gomberg-Maitland1 , Ioana R. Preston2
  • 1Division of Cardiology, University of Chicago Hospitals, Chicago, Illinois
  • 2Pulmonary Critical Care and Sleep Division, Tufts-New England Medical Center, Boston, Massachusetts
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Publication History

Publication Date:
25 August 2005 (online)

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ABSTRACT

Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction and smooth muscle cell proliferation of the pulmonary arterioles, as well as in situ thrombosis of the small pulmonary arteries. Prostacyclin is involved in PAH vascular remodeling. It is unclear if decreased prostacyclin in the lungs is a cause or a consequence of PAH, but the relative lack of prostacyclin and its positive effects on the pulmonary vascular bed support the theory that long-term prostacyclin replacement is effective. Current therapies based on evidence-based medicine include epoprostenol, treprostinil, iloprost, and beraprost, each with limitations based on the drugs' inherent properties and administration route. Treatment of PAH by inhibiting multiple pathways concurrently may produce additive benefit. Because prostacyclin therapy is not curative and does not normalize pulmonary hemodynamics in the majority of cases, combining a prostacyclin with other PAH agents may be a promising approach.

KEYWORDS

Pulmonary arterial hypertension - prostacyclin - combination therapies

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Mardi Gomberg-MaitlandM.D. M.Sc. 

Division of Cardiology, University of Chicago Hospitals

5841 S. Maryland Ave., MC2016

Chicago, IL 60637

Email: mgomberg@medicine.bsd.uchicago.edu