Pharmacologic strategies for management of pulmonary arterial hypertension

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Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary artery pressure ≥ 25 mmHg at rest with a pulmonary wedge pressure 15 mmHg and a pulmonary vascular resistance > 3 Wood units measured by right cardiac catheterization. Increased awareness of the progressive nature of the disease has led to earlier evaluation, identification, and therapy. Management of PAH includes non-pharmacologic therapy as well as background and targeted medical therapy. Background therapy may include diuretics, digoxin, oxygen, and anticoagulation for specific patients. Five classes of targeted medical therapy are currently available to manage PAH, including prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase inhibitors, a soluble guanylate cyclase stimulator, and a prostacyclin IP receptor agonist. Recent evidence shows that initial combination therapy with some of these agents reduces the time to clinical failure and the incidence of PAH-related hospitalizations.

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