Right Ventricular Myofilament Functional Differences in Humans With Systemic Sclerosis–Associated Versus Idiopathic Pulmonary Arterial Hypertension

Abstract

A primary defect in human SSc-PAH resides in depressed sarcomere function, whereas this is enhanced in IPAH. These disparities correlate with in vivo RV contractility and contractile reserve and are consistent with worse clinical outcomes in SSc-PAH. The existence of sarcomere disease before the development of resting PAH in patients with SSc-d suggests that earlier identification and intervention may prove useful.