Pulmonary arterial hypertension (PAH) is a chronic and progressive disorder, defined as a mean pulmonary arterial pressure > 20 mm Hg and a pulmonary vascular resistance > 3 WU, with a normal capillary wedge pressure of < 15 mm Hg. PAH is characterized by an increased pulmonary resistance resulting in right ventricular overload and right heart failure.
Studies have established the molecular pathways involved in the pathophysiology of PAH, allowing the development of disease-specific medications: prostacyclin pathway, nitric oxide pathway, and endothelin pathway. There are currently 14 medications that are approved by the US Food and Drug Administration for the treatment of PAH. Although they improve symptoms and quality of life in patients with PAH, they require consistent monitoring and are not considered curative. Ultimately, PAH remains a fatal disease with a mortality of at least 50% in 7 years. The goal of treatment is to lower the risk for mortality, prevent progression, and optimize the quality of life of patients with this condition.
How familiar are you with PAH? Test your knowledge with this quick quiz.
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Cite this: Vikramjit Khangoora. Fast Five Quiz: Pulmonary Arterial Hypertension Treatment - Medscape - Jun 02, 2022.