J Korean Med Assoc.  2011 Dec;54(12):1299-1305. 10.5124/jkma.2011.54.12.1299.

Pharmacotherapy for pulmonary arterial hypertension

• Kim C
• Park YB

• KMID: 2064781
• DOI: http://doi.org/10.5124/jkma.2011.54.12.1299

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.

Keyword

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

MeSH Terms

Administration, Intravenous
Anoxia
Epoprostenol
Humans
Hypertension
Hypertension, Pulmonary
Oxygen
Phosphodiesterase 5 Inhibitors
Physician's Practice Patterns
Rare Diseases
Retention (Psychology)
Vascular Diseases
World Health Organization
Epoprostenol
Hypertension, Pulmonary
Oxygen
Phosphodiesterase 5 Inhibitors

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