Pulmonary hypertension (PH) is a spectrum disorder with multiple causes of the elevation of pressure in the lungs. It often is difficult to diagnose because it mimics many commonly reported symptoms (eg, dyspnea, exercise intolerance, chest pain). Diagnosis is made via right heart catheterization; however, transthoracic echocardiography may show evidence of elevated pulmonary pressure as the first clue to the diagnosis. Diagnostic tests to consider include a liver panel, complete blood count, and thyroid function test; electrocardiogram; chest x-ray; pulmonary function testing; and possibly lung imaging via computed tomography scan or ventilation-perfusion scan. PH is grouped into several broad categories: group 1 is pulmonary artery hypertension, group 2 is PH due to left heart disease, group 3 is PH due to lung disease, group 4 is chronic thromboembolic PH, and group 5 is PH due to unclear or multifactorial mechanisms. Therapy targets the underlying etiology and may include physical activity and pulmonary rehabilitation; drugs such as diuretics, vasodilators, and anticoagulants; oxygen therapy; and lung transplantation. Significant PH can result in increased mortality risk. Because of its complex and heterogeneous nature, PH is best managed by subspecialists with expertise in the condition.
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