Abstract

Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) predispose to ventricular arrhythmias and sudden cardiac death. The clinical and morphological features may overlap, thus there is a risk of misdiagnosis and incorrect management. We sought to assess the predictive value of major and minor electrocardiogram (ECG) repolarization abnormalities as per 2010 ARVC Task Force Criteria (TFC2010) in differentiating ARVC from CS. The baseline ECG of patients with definite ARVC diagnosis by TFC2010 (n=107) were compared to a cohort with CS patients with either primary cardiac localisation (n=37) or cardiac involvement in otherwise primary non-CS (n=22) as per imaging and/or biopsy data. Diagnostic ECG was automatically processed and Bazett-corrected QT interval (QTc), major (MaRC) and minor (MiRC) repolarization criteria as well as terminal activation duration in lead V1 (TAD-V1) were calculated. Logistic regression analysis adjusted for age and gender was used to test the association of ECG-based TFC2010 with ARVC and sensitivity (Se), specificity (Sp), positive (PPV) and negative (NPV) predictive values were calculated. ARVC patients were younger at diagnosis compared to CS patients (45±16 vs 60±13 years, p<0.001) and both showed a male predominance (66% vs. 53%, p=0.09). MaRC was evident in 35 (33%) ARVC patients compared to 2 (3.4%) CS patients (ORadj=17, 95%CI 3.61-87, p<0.001, Se 33%, Sp 97%, PPV 95%, NPV 44%). MiRC was present in 55 (51%) ARVC patients compared to 16 (27%) CS patients (ORadj=3.67, 95%CI 1.65-8.11, p=0.001, Se 51%, Sp 73%, PPV 78%, NPV 45%). Among CS patients, MaRC was only observed in those who had primary cardiac localisation. TAD-V1 > 55 ms was present in 30 (28%) ARVC patients and 8 (21%) patients with CS (p=0.26). QTc was shorter in ARVC patients compared to CS (424±39 vs 449±34 ms; ORadj=0.98 95%CI 0.97-0.99, p=0.004), though only 6 (5.8%) ARVC patients and 9 (15%) CS patients had QTc> 470 ms (p=0.014). The ECG-based TFC2010 criteria have a high clinical utility for discriminating between ARVC and CS. Despite a lower prevalence of ARVC TFC2010 repolarization criteria among patients with CS, the latter had longer QTc, thus suggesting differences in the electrophysiological properties of ventricular myocardium.

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