Key result
Pulmonary arterial hypertension is a devastating disease affecting mostly women with a mean age of 50, characterized by elevated pulmonary artery pressures and treated with PAH-specific medications.
This overview highlights the epidemiology, symptoms, pathobiology, diagnosis, and common treatments for pulmonary arterial hypertension.
Provides PAH synthesis for clinicians; leaves open need for updated prospective research on therapies.
Pulmonary arterial hypertension (PAH) is a devastating disease characterized by elevated mean pulmonary artery pressures (PAP) that ultimately leads to right heart failure and death. Approximately 100 000 patients are living with PAH worldwide. Life expectancy is 6–7 years with treatment. PAH affects mostly women (80%) with a mean age of diagnosis of 50 years. Common symptoms of PAH include dyspnea at rest, peripheral oedema, chest pain/discomfort, dizziness/lightheadedness, syncope, cough and abdominal distention. Pathobiological findings include vasoconstriction, remodelling and in situ thrombosis. Diagnosis is confirmed by elevated mean PAPs determined by right heart catheterization. Common treatments comprise oxygen, diuretics, and anticoagulation along with PAHspecific medications: calcium channel antagonists, prostanoids, endothelin antagonists, and phosphodiesterase inhibitors.
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Lea Ann Matura (2011) conducted a review in Pulmonary arterial hypertension. Pulmonary arterial hypertension is a devastating disease affecting mostly women with a mean age of 50, characterized by elevated pulmonary artery pressures and treated with PAH-specific medications.
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