Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease that has a high impact on patients’ quality of life, morbidity and mortality. PAH is characterized by extensive pulmonary vascular remodeling that results in an increase in pulmonary vascular resistance and right ventricular afterload, and can lead to right heart failure. Patients with PAH exhibit inefficient ventilation, high dead space ventilation, dynamic hyperinflation, and ventricular-arterial uncoupling, which can contribute to high dyspnea and low exercise tolerance. Cardiopulmonary exercise testing can help to diagnose PAH, define prognosis and treatment response in PAH, as well as discriminate between different pulmonary vascular diseases.