Imaging modalities for the diagnosis of pulmonary hypertension in systemic sclerosis

Abstract

Patients with systemic sclerosis (SSc) are at considerable risk of developing pulmonary arterial hypertension (PAH). PAH has a dramatic impact on the natural history of the disease and overall survival of the patient. Despite progress made in elucidating the pathogenesis of PAH and introduction of novel therapies, SSc-related PAH (SScPAH) remains a devastating disease that responds poorly to therapy. Although early diagnosis is of paramount importance, there are no available validated strategies for assessing SScPAH because reliable evaluation of the structure and function of the right ventricle is difficult owing to its complex geometry. Additionally, myocardial fibrosis might affect cardiac contractility and contribute to heart failure. Modern imaging modalities, such as novel echocardiographic techniques and cardiac MRI, are highly sensitive, quantitative and reproducible methods that allow noninvasive assessment of regional and global myocardial performance without relying on geometric assumptions. In this Review, we examine the imaging modalities currently available, focusing on evolving diagnostic imaging methodologies and their possible clinical implications in the SScPAH setting.