Abstract
Pulmonary arterial hypertension is a chronic disorder of the pulmonary circulation characterized by an increased pulmonary artery pressure and an impaired cardiac function. It can present as idiopathic form or associated with several systemic diseases. Typical symptoms include progressive dyspnoea and exercise intolerance. When pulmonary arterial hypertension is suspected, echocardiography is the key investigation for noninvasive assessment of the disease. The diagnosis requires an invasive measurement of pulmonary hemodynamics by right heart catheterization. Treatment is based on general measures and application of pulmonary vasoactive compounds, such as prostanoids, endothelin receptor blockers and phosphodiesterase 5 inhibitors. These treatment options can be combined at progression of the disease. Lung transplantation might be an option in patients refractory to medical therapy.
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