[Pulmonary arterial hypertension]

Pulmonary arterial hypertension is a life-threatening, progressive disorder of pulmonary blood vessels leading to an increase in pressure in pulmonary artery. Diagnosis is based on a mean pulmonary artery pressure of more than 25 mmHg at rest or more than 30 mmHg during exercise. No cure exists for it yet. However, specialized treatment can lower pulmonary pressure, reduce symptoms, increase the capacity to be active, and prolong lifespan. In this review article, we attempt to summarize the current knowledge regarding clinical classification, risk factors and associated conditions, pathology and pathogenesis of this disease, diagnostic tests and detection of it, clinical course, current therapeutic strategies for the treatment of pulmonary arterial hypertension (calcium channel blockers, prostacyclin analogues, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, etc.). Interventional procedures, combination therapy, and new strategies (selective serotonin reuptake inhibitors, antivascular endothelial growth factor agents, potassium channel openers, etc.) for the management of pulmonary arterial hypertension and prognosis of this rare disease are also discussed.