Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward

Abstract

Pulmonary arterial hypertension (PAH) can be a rapidly progressive disorder and is associated with high rate of mortality, despite medical intervention. With the availability of effective therapy, early disease detection is an important strategic objective to improve treatment outcomes. Resting echocardiography is currently the recommended screening modality for high-risk population groups. However, it is clear that derangements in resting haemodynamics (and symptoms) are late sequelae of the pathobiological processes that begin in the distal pulmonary arteries. Exercise stress may unmask early pulmonary vascular dysfunction but the definition, clinical significance, and natural history of 'exercise PAH' remain undefined. We will review the currently available and potential future strategies aimed at early disease detection, and propose that ultimately the way forward is to detect disease at a stage prior to the rise in resting pulmonary artery pressure.