Pulmonary arterial hypertension (PAH) results from continuously high blood pressure (BP) in the pulmonary artery. In patients with PAH, the BP is usually greater than 25 mm Hg at rest and above 30 mm Hg with exercise.
The cause of pulmonary hypertension may be idiopathic or familial in nature, but it may also be congenital or related to collagen vascular disease, human immunodeficiency virus (HIV), portal hypertension, and the use of recreational or therapeutic drugs (e.g., amphetamines, appetite suppressants, and cocaine).
Idiopathic PAH is a form of primary pulmonary hypertension (PPH). A patient with PAH may experience fatigue, breathlessness, cyanosis of the lips, palpitations, chest pain, and syncope. Ultimately, lower-extremity edema is present around the ankles.
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Right-sided congestion, consisting of increased jugular venous pressure, ascites, and hepatomegaly, may occur, leading to right ventricular heart failure. If PAH remains untreated, the risk of mortality is increased.
The incidence of PPH ranges from one to two cases per million people in the general population.