Abstract

Ventricular arrhythmias (VA) are a leading cause of death in patients with cardiac sarcoidosis (CS). Right ventricular (RV) dysfunction and late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) have been linked to incidence of VAs. However, detection of LGE of the RV is limited by image resolution. Global longitudinal RV strain (RVS) correlates to RV scar on electroanatomical mapping, and is a valid measure of regional RV function. We evaluated the association between RVS on CMR and incidence of VAs, cardiovascular (CV) death, and heart transplantation (HT) (combined primary end-point) in patients with CS. In this retrospective study, 71 patients with probable or definite CS were included. RVS and RV LGE on MRI were compared to variables known to predict arrhythmic and mortality outcomes in CS. Outcomes were obtained by review of electronic medical records and cardiac implantable electronic device interrogations over a median [IQR] follow-up period of 3.7[1.7, 6.3) years. Cox proportional hazard models were used to evaluate the probability of event-free survival. Harrell’s C statistic was used to compare variables in risk prediction models. 59% of patients were male, with median age [IQR] of 52.3 [42.4, 62.2] years, and left ventricular ejection fraction (LVEF) of 54.9%[41.4, 68.4]. During the follow-up period, 13 patients developed the primary endpoint and were more likely to have a history of Vas (HR[95% CI] 6.80[1.86,24.8],p=0.004) and higher LV LGE burden (1.07[1.01,1.12], p=0.02). In a multivariate analysis adjusted for age, race, and history of VA, decreased RVS was a significant predictor of the primary outcome (0.84[0.71, 0.98], p=0.03), whereas RV LGE, RV end-diastolic volume (EDV) and RV EF were not. Risk prediction models including RVS (Harrell’s C statistic 0.87) were superior to those including only RV LGE (0.79) or LV LGE burden (0.82). Global RVS on CMR was the best predictor of VAs or CV death in patients with CS.

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