Abstract

Introduction: Risk stratification in cardiac sarcoidosis (CS) remains challenging, especially in those with preserved left ventricular ejection fraction (LVEF). Myocardial strain parameters are more sensitive than LVEF for detecting early sub-clinical myocardial dysfunction, but their prognostic value in CS is not known. Methods: We investigated consecutive newly diagnosed CS patients between 2015 and 2018. All patients underwent 2D echocardiography including global longitudinal strain (GLS) and global circumferential strain (GCS) measurement, cardiac magnetic resonance (CMR) for late gadolinium enhancement (LGE) and FDG-PET imaging to derive the maximum standard uptake value (SUVmax). The LGE score was quantified as the number of myocardial segments with LGE. Major adverse cardiac events (MACE) were all-cause death, sustained ventricular arrhythmia (VA), device implantation and new onset heart failure (HF) admission. Results: A total of 120 patients were included with age of 55±12 years; 87% were Caucasian, 43% had hypertension and 13% had diabetes. Mean LVEF was 55±12.3%, GLS was -13.4±4.2%, GCS was -16.0±5.6% and regional wall motion abnormalities (RWMA) occurred in 36% of cases. During median follow-up of 4.3 years, 43 patients experienced MACE consisting of 15 deaths, 19 VAs, 7 devices and 2 HF admissions. In multivariate analysis syncope, ethnicity, echo-derived left atrial volume and RWMA were independent predictors of MACE along with CMR-derived right ventricular EF and LGE score. Although GLS and GCS were lower in those with MACE, neither had independent prognostic value. SUVmax was also not predictive of events. In a subgroup analysis of the 86 patients with LVEF ≥50%, 20 patients experienced MACE. Multivariate analysis showed non-Caucasian ethnicity (HR 3.18 [95% CI 1.02-9.88]; P = 0.04), RWMA (HR 3.76 [95% CI 1.29-10.98]; P = 0.02), LGE-score (HR 1.26 95% CI 1.05-1.51]; P = 0.01 and GCS (HR 1.18 [95% CI 1.04-1.36]; P = 0.01) were independent predictors of MACE. A GCS value above the optimal cut-off of -16.4% was associated with a worse outcome (log-rank P = 0.005). Conclusion Ethnicity, RWMA and LGE-score are robust markers of outcome in newly diagnosed CS. Reduced GCS provides additional prognostic information in those with preserved LVEF.

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