Pharmacotherapy for pulmonary arterial hypertension

Changhwan Kim; Yong Bum Park

doi:10.5124/jkma.2011.54.12.1299

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Kim and Park: Pharmacotherapy for pulmonary arterial hypertension

Pharmacotherapeutics

Journal of the Korean Medical Association

Journal of the Korean Medical Association 2011; 54(12): 1299-1305.

Published online: 13 December 2011

DOI: https://doi.org/10.5124/jkma.2011.54.12.1299

Pharmacotherapy for pulmonary arterial hypertension

Changhwan Kim, MD^1,², Yong Bum Park, MD^1,²

¹Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Hallym University Kangdong Sacred Heart Hospital, Seoul, Korea.

²Lung Research Institute, Hallym University College of Medicine, Seoul, Korea.

Corresponding author: Yong Bum Park, bfspark@medimail.co.kr

Received 10 November 2011 Accepted 20 November 2011

(open-access):

This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Although pulmonary arterial hypertension (PAH) is an orphan disease with high mortality and for which there is no cure, current treatment have led to considerable gains in the outcomes of these patients. Oral anticoagulation is proposed for most patients; diuretic treatment and supplemental oxygen are indicated in cases of fluid retention and hypoxemia. High doses of calcium-channel blockers are indicated only in the minority of patients who respond to acute vasoreactivity testing. Nonresponders to acute vaoreactivity testing or who remain in World Health Organization (WHO) functional class III, should be considered candidates for treatment with either an oral phophodiesterase-5 inhibitor or an oral endothelin-receptor antagonist. Continuous intravenous administration of epoprostenol remains the treatment of choice in WHO functional class IV patients. Combination therapy is recommended for patients treated with PAH monotherapy who remain in WHO functional class III. The pharmacologic management of PAH is rapidly evolving as newer therapeutic targets that stabilize or reverse pulmonary vascular disease and as clinical practice pattern shift in favor of earlier diagnosis and aggressive treatment. Questions about preferred first-line therapy and when to institute combination therapies remain. Future drug development targeting other molecular pathways of PAH is essential for definitively improving patient survival. The search for novel treatment continues, with promising new concepts arising from a better understanding of the pathobiology of PAH.

Keywords: Hypertension, pulmonary, Drug therapy, Prostacyclin analogues, Endothelin receptor antagonists, Phosphodiesterase 5 inhibitors

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