Diagnosis and evaluation of pulmonary hypertension

The diagnosis of pulmonary hypertension (PH) relies on a high index of suspicion. In patients with symptoms or chest radiographic findings suggestive of PH, a detailed history and physical examination followed by early assessment with a transthoracic echocardiogram, ventilation-perfusion scanning, chest computed tomography, pulmonary function testing, and nocturnal oximetry screening can provide valuable information about etiology and severity. Right heart catheterization should follow in patients who are symptomatic or who demonstrate moderate to severe PH by echocardiography and are candidates for treatment. Patients at risk for developing PH should undergo serial echocardiography and pulmonary function testing to assess for disease development and progression. Genetic testing is not currently recommended in the routine evaluation of patients with a diagnosis of primary pulmonary hypertension.