Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is common in the general population and associated with increased mortality. Accordingly, physicians commonly encounter patients with
dyspnea, exercise intolerance, and/or right
heart failure who have elevated pulmonary artery systolic pressure (
PASP) on echocardiography. Although pulmonary arterial
vasodilators may often be considered in this setting, these drugs have been predominantly tested in the subset of PH patients with
pulmonary arterial hypertension (PAH). Elevated
PASP alone is not sufficient for the diagnosis of PAH, and secondary causes of
PASP elevation, most commonly left
heart disease, are far more prevalent than isolated PAH. Treatment of this more common group of patients with PH due to left
heart disease is challenging because there are few evidence-based treatment options, and pulmonary
vasodilator therapy may lead to worsening symptoms. Therefore, improving symptoms and avoiding adverse outcomes in patients with PH requires the following: (1) understanding the optimal use of echocardiography for the diagnosis of PH; (2) recognizing the utility and proper interpretation of invasive hemodynamic testing prior to starting pulmonary
vasodilator therapy; (3) differentiating PAH from pulmonary venous
hypertension due to left
heart disease; and (4) understanding the appropriate treatment strategies for PH and resultant right
heart failure.