The pulmonary vascular bed is normally a low-resistance, high-capacitance circuit capable of accommodating the entire cardiac output at pressures approximately 15%–20% of those in the systemic circulation. In pulmonary hypertension (PH), elevated pulmonary arterial pressure places a burden on the normally thin-walled right ventricle as it works to maintain normal blood flow. Without effective therapy, right heart dysfunction leads to progressive symptoms and is often fatal. Pulmonary hypertension is frequently a result of common left-sided heart or lung diseases. Less frequently, it results from a disease process intrinsic to the pulmonary vasculature itself. Differentiating among the several causes of PH requires methodical evaluation. Differentiation is essential because management varies according to the underlying cause, and misapplication of therapy can cause serious harm.