Review articleReaddressing the entity of exercise pulmonary arterial hypertension

•EPAH is a real entity despite the controversy surrounding its definition and course.•EPAH likely shares a common pathomechanism with resting PAH.•Some cases of EPAH will progress to resting PAH with resultant morbidity and mortality.•The prevalence of EPAH in scleroderma can be as high as 50%.•PAH therapy improves exercise tolerance and quality of life in some cases of EPAH.

Exercise pulmonary hypertension (EPH) indicates an abnormally elevated pulmonary artery pressure (PAP) during exercise. The physiological range of PAP during exercise remains poorly defined and, therefore, a universally accepted definition of EPH remains elusive. Nevertheless, previous data concerning the distribution of PAP in normal populations and more recent retrospective clinical data enhanced our ability to define EPH. EPH can impair exercise capacity and cause dyspnea. The underlying pathophysiology of the arterial form of EPH (EPAH) appears to be similar to that seen in resting pulmonary arterial hypertension (PAH), and EPAH individuals are at risk of developing resting PAH. Patients with collagen vascular disease, especially scleroderma, are at risk for EPAH and its presence indicates a relatively poor prognosis. The prevalence of EPAH in scleroderma may be as high as 50%. The utility of pulmonary vasodilator therapy for EPAH is not well defined; however, a sizable subgroup of EPAH patients will achieve an improvement in symptoms.