Long-term prognostic value of left and right ventricular systolic function on cardiovascular magnetic resonance imaging in systemic sclerosis.

Aims: Systemic sclerosis (SSc) is a rare autoimmune disorder associated with a high risk of cardiovascular diseases. We aimed to determine the long-term prognostic value of left and right ventricular (LV and RV) systolic dysfunction in SSc patients with clinically suspected cardiac disease.

Methods and results: We conducted a retrospective cohort study of consecutive adults with SSc who had cardiovascular magnetic resonance (CMR) imaging for suspected cardiac disease. We assessed two CMR measures of LV and RV function, ejection fraction (EF), and feature tracking-derived global longitudinal strain (GLS) and investigated their associations with the long-term incidence of a composite endpoint of death or major adverse cardiac events (MACE). In 151 patients (median age 58 years, 81% women) who had CMR at a median of 3.6 years after diagnosis, the median LVEF was 58.0%, and the median LVGLS was -15.7%. The median RVEF was 57.0%, and the median RVGLS was -16.2%. Over a median follow-up of 4.7 years, 69 patients experienced the composite endpoint of death or MACE. LVGLS was independently associated with the composite endpoint [hazard ratio (HR) 1.08 per 1% worsening; 95% confidence interval (CI) 1.01-1.15; P = 0.018], while LVEF was not. Similarly, RVGLS was independently associated with the composite endpoint (HR 1.08 per 1% worsening; 95% CI 1.01-1.15; P = 0.017), while RVEF was not.

Conclusion: In patients with SSc and clinically suspected cardiac disease, worse LVGLS and RVGLS on CMR were independently associated with death or MACE, while LVEF and RVEF were not.