Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is the most characteristic group of pulmonary hypertensive states. Among other conditions, PAH includes the idiopathic type and forms associated with connective tissue diseases (CTDs), particularly systemic sclerosis, CREST syndrome, and mixed connective tissue disease. Symptoms and signs of PAH include shortness of breath, fatigue, syncope, chest pain, and swollen ankles. Pulmonary hypertension can be detected by transthoracic Doppler echocardiography. This procedure should be performed routinely once a year in patients with CTD with a high risk of developing PAH. The diagnosis of pulmonary hypertension needs to be confirmed by right heart catheterization. Pharmacological treatment is based on drugs that modulate endothelial function.