The Effect of Hematopoietic Stem Cell Transplantation on Cardiac Mechanics in Systemic Sclerosis

Abstract

Abstract Purpose Systemic sclerosis (SSc) is an autoimmune disease that causes inflammation and fibrosis. Cardiac involvement in SSc is often subclinical and portends a worse prognosis. Autologous hematopoietic stem cell transplant (HSCT) improves survival in SSc but its effect on cardiac function is unknown. This study aimed to assess HSCT’s effect on cardiac mechanics in SSc. Methods Participants with SSc were identified from a prospective registry, and grouped according to the receipt of HSCT between 2009 and 2018. The HSCT cohort underwent comprehensive conventional and speckle-tracking echocardiography (STE) pre- and post-HSCT. The non-HSCT cohort received echocardiograms within a similar time frame. Baseline and follow-up clinical and echocardiographic variables were compared within and between groups. Results The HSCT cohort (n = 88) was older (59±6 versus 51±11 years, p = 0.002) and more female-predominant (95% vs 75%, p = 0.049) compared to the non-HSCT cohort (n = 20). HSCT recipients showed improved right ventricular (RV) strain globally (18.1±3.9% versus 20.0±4.5%, p < 0.001) and within the RV free wall (20.7±5.3% versus 23.2±5.6%, p < 0.001). While left ventricular (LV) strain did not change, left atrial (LA) reservoir strain improved (35.9±8.7% versus 47.8±11.4%, p < 0.001) and LA stiffness index (0.24±0.12 versus 0.18±0.08, p < 0.001) decreased post-HSCT. No longitudinal changes in STE measures were observed among the non-HSCT cohort. Between-group analysis demonstrated a significant association between HSCT and change in LA reservoir strain (p = 0.002) at follow-up. Conclusions RV and LA mechanics significantly improve after HSCT among patients with SSc. This suggests a favorable effect of HSCT on the underlying myocardial pathology caused by SSc.