Diagnosis of pulmonary arterial hypertension

Accurate diagnosis of pulmonary arterial hypertension can be challenging and often requires a high index of clinical suspicion. Use of a variety of noninvasive tests can help define the population of patients in whom invasive cardiac catheterization should be pursued. An understanding of the historical, physical exam, electrocardiographic, radiographic, and echocardiographic clues in the diagnosis is important. A ventilation-perfusion scan and careful assessment for left-to-right shunting are mandatory to avoid missing reasons for pulmonary hypertension that may require nonpharmacologic management. Right heart, and sometimes concomitant left heart, catheterization is required to establish the diagnosis and distinguish pulmonary arterial from pulmonary venous hypertension.