The majority of patients with pulmonary arterial hypertension (PAH) present in advanced stages of the disease, when therapeutic interventions may potentially be less effective. Early diagnosis is associated with improved outcomes, supporting best practice recommendations that asymptomatic patients in high-risk populations such as systemic sclerosis or carriers of a PAH-causing mutation should be offered periodic screening for PAH. An evidence-based algorithm for screening systemic sclerosis-associated PAH is now available based on the DETECT study. A multimodal approach using Doppler echocardiography, lung function testing, and B-type natriuretic peptides improves screening accuracy but at the expense of higher health care costs. Patients with abnormal hemodynamics but who do not meet definition for diagnosis of pulmonary hypertension (such as mean pulmonary artery pressure between 21 and 24 mm Hg) should be closely followed up given their increased risk of progression to PAH. This review summarizes the current approach to screening for PAH and discusses areas of uncertainties that require further research.