Diagnostic value of late gadolinium enhancement at cardiovascular magnetic resonance to distinguish arrhythmogenic right ventricular cardiomyopathy from differentials.

IF 4.2 1区 医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS
Lian Y Rekker, Steven A Muller, Alessio Gasperetti, Mimount Bourfiss, Marish I F J Oerlemans, Maarten J Cramer, Stefan L Zimmerman, Dennis Dooijes, Hanke Schalkx, Pim van der Harst, Cynthia A James, J Peter van Tintelen, Marco Guglielmo, Birgitta K Velthuis, Anneline S J M Te Riele
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引用次数: 0

Abstract

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.

心脏磁共振的晚期钆增强对区分心律失常性右室心肌病的诊断价值
背景:虽然晚期钆增强(LGE)被建议作为心律失常性右室心肌病(ARVC)的诊断标准,但 LGE 区分 ARVC 和鉴别诊断的潜力仍然未知。我们旨在评估 LGE 对 ARVC 诊断的诊断价值:我们纳入了 132 名接受过心脏磁共振成像和 LGE 评估的 ARVC 或 ARVC 差异型受试者(男性占 60%,47±11 岁)。ARVC根据2010年工作组标准诊断(55人)。ARVC 差异包括家族性/遗传性扩张型心肌病(25 人)、心肌炎(13 人)、肉样瘤病(20 人)和淀粉样变性(19 人)。所有鉴别诊断均基于最新的黄金标准。使用 7 段左心室模型和 17 段左心室模型评估是否存在 LGE。随后,我们根据帕多瓦标准评估了每位患者的 LGE 模式,以确定其是否符合 LV 和/或 RV-LGE,而与临床诊断(即表型)无关。我们使用敏感性和特异性分析了任何 RV-LGE、任何 LV-LGE、符合帕多瓦标准的 RV-LGE 的诊断价值,以及符合帕多瓦标准的 LV-LGE 的患病率图。结果:三分之一(38%)的 ARVC 患者患有 RV-LGE,而一半(51%)的患者患有 LV-LGE。与非 ARVC 患者相比,RV-LGE 在 ARVC 患者中的观察频率较低(38% 对 58%,P=0.034),因此鉴别潜力较差(任何 RV-LGE:灵敏度为 38%,特异性为 42%;根据帕多瓦标准观察的 RV-LGE:灵敏度为 36%,特异性为 44%)。与 ARVC 患者相比,非 ARVC 患者更常出现 LV-LGE (91% 对 51%,P 结论:LGE 常出现在 ARVC 鉴别中,在不了解 LGE 模式的情况下使用可能会导致假阳性诊断。在 ARVC 中通常可观察到中度 RV-LGE 而无前室壁和心肌中段 LV-LGE。
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来源期刊
CiteScore
10.90
自引率
12.50%
发文量
61
审稿时长
6-12 weeks
期刊介绍: Journal of Cardiovascular Magnetic Resonance (JCMR) publishes high-quality articles on all aspects of basic, translational and clinical research on the design, development, manufacture, and evaluation of cardiovascular magnetic resonance (CMR) methods applied to the cardiovascular system. Topical areas include, but are not limited to: New applications of magnetic resonance to improve the diagnostic strategies, risk stratification, characterization and management of diseases affecting the cardiovascular system. New methods to enhance or accelerate image acquisition and data analysis. Results of multicenter, or larger single-center studies that provide insight into the utility of CMR. Basic biological perceptions derived by CMR methods.
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