Key result
Late gadolinium enhancement patterns characterized by the absence of anteroseptal and mid-myocardial left ventricular enhancement predicted ARVC with a sensitivity of 93% and specificity of 78%.
Why the study?
While LGE is proposed as a diagnostic criterion for ARVC, its potential to distinguish ARVC from differentials remains unknown.
Does cardiac magnetic resonance with late gadolinium enhancement pattern assessment distinguish arrhythmogenic right ventricular cardiomyopathy from other cardiomyopathies?
Observational (n=132)
No
Does cardiac magnetic resonance with late gadolinium enhancement pattern assessment distinguish arrhythmogenic right ventricular cardiomyopathy from other cardiomyopathies?
Answers are grounded in this paper and cite their sources.
Specific patterns of late gadolinium enhancement on cardiac MRI, such as the absence of extensive mid-myocardial left ventricular LGE, can accurately distinguish ARVC from other cardiomyopathies and reduce false positive diagnoses.
BACKGROUND: While late gadolinium enhancement (LGE) is proposed as a diagnostic criterion for arrhythmogenic right ventricular cardiomyopathy (ARVC), the potential of LGE to distinguish ARVC from differentials remains unknown. We aimed to assess the diagnostic value of LGE for ARVC diagnosis. METHODS: We included 132 subjects (60% male, 47 ± 11 years) who had undergone cardiac magnetic resonance imaging with LGE assessment for ARVC or ARVC differentials. ARVC was diagnosed as per 2010 Task Force Criteria (n = 55). ARVC differentials consisted of familial/genetic dilated cardiomyopathy (n = 25), myocarditis (n = 13), sarcoidosis (n = 20), and amyloidosis (n = 19). The diagnosis of all differentials was based on the most current standard of reference. The presence of LGE was evaluated using a 7-segment right ventricle (RV) and 17-segment left ventricle (LV) model. Subsequently, we assessed LGE patterns for every patient individually for fulfilling LV- and/or RV-LGE per Padua criteria, independent of their clinical diagnosis (i.e. phenotype). Diagnostic values were analyzed using sensitivity and specificity for any RV-LGE, any LV-LGE, RV-LGE per Padua criteria, and prevalence graphs for LV-LGE per Padua criteria. The optimal integration of LGE for ARVC diagnosis was determined using classification and regression tree analysis. RESULTS: One-third (38%) of ARVC patients had RV-LGE, while half (51%) had LV-LGE. RV-LGE was less frequently observed in ARVC vs non-ARVC patients (38% vs 58%, p = 0.034) leading to a poor discriminatory potential (any RV-LGE: sensitivity 38%, specificity 42%; RV-LGE per Padua criteria: sensitivity 36%, specificity 44%). Compared to ARVC patients, non-ARVC patients more often had LV-LGE (91% vs 51%, p < 0.001) which was also more globally distributed (median 9 [interquartile range (IQR): 3-13] vs 0 [IQR: 0-3] segments, p < 0.001). The absence of anteroseptal and absence of extensive (≥5 segments) mid-myocardial LV-LGE, and absence of moderate (≥2 segments) mid-myocardial LV-LGE predicted ARVC with good diagnostic performance (sensitivity 93%, specificity 78%). CONCLUSION: LGE is often present in ARVC differentials and may lead to false positive diagnoses when used without knowledge of LGE patterns. Moderate RV-LGE without anteroseptal and mid-myocardial LV-LGE is typically observed in ARVC.
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Rekker et al. (2024) conducted an observational in Arrhythmogenic right ventricular cardiomyopathy (ARVC) and its differentials (n=132). Late gadolinium enhancement (LGE) vs. Non-ARVC differentials was evaluated on Presence of any right ventricular late gadolinium enhancement (RV-LGE) (p=0.034). Late gadolinium enhancement patterns characterized by the absence of anteroseptal and mid-myocardial left ventricular enhancement predicted ARVC with a sensitivity of 93% and specificity of 78%.
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