Electrical and Structural Substrate of Arrhythmogenic Right Ventricular Cardiomyopathy Determined Using Noninvasive Electrocardiographic Imaging and Late Gadolinium Magnetic Resonance Imaging
Electrical and Structural Substrate of Arrhythmogenic Right Ventricular Cardiomyopathy Determined Using Noninvasive Electrocardiographic Imaging and Late Gadolinium Magnetic Resonance Imaging
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DOI：
10.1161/circep.116.005105
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发表时间：
2017-07-01
影响因子：
8.4
通讯作者：
Rudy, Yoram
中科院分区：
文献类型：
--
作者：
Andrews, Christopher M.;Srinivasan, Neil T.;Rudy, Yoram
致心律失常性右心室心肌病（ARVC）是年轻人心脏性猝死的重要原因。改进的非侵入性评估ARVC和更好地了解疾病基板是重要的，为改善患者outcomes.Methods和结果，我们研究了20个基因型ARVC患者的疾病使用心电图成像（一种方法，非侵入性心脏电生理映射）和先进的晚期钆增强心脏磁共振瘢痕成像的广谱。与20名健康对照组相比，ARVC患者的心室激动持续时间较长（中位数，52 vs 42 ms; P=0.007），平均心外膜激动恢复间期延长（局部动作电位持续时间的替代;中位数，275 vs 241 ms; P=0.014）。在这些患者中，我们观察到异常和不同的心外膜激动突破位置和区域的不均匀传导和碎裂电描记图。ARVC隐匿相早期存在不均匀传导和碎裂电位。电生理异常与晚期钆增强瘢痕共定位，表明与结构性疾病的关系。室性早搏在两个心室起始部位不同的ARVC患者中很常见。心室期前收缩率随着运动而增加，在解剖节段内，它与复极延长、疤痕电标记和晚期钆增强相关（均P < 0.001）。结论-心电图成像揭示了ARVC患者与健康对照组不同的电生理基质特性。一个新的机制发现是心室异位起源的区域存在复极异常。结果表明，心电图成像和晚期钆增强在ARVC患者的早期诊断和无创随访中具有潜在作用。
Background-Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a significant cause of sudden cardiac death in the young. Improved noninvasive assessment of ARVC and better understanding of the disease substrate are important for improving patient outcomes.Methods and Results-We studied 20 genotyped ARVC patients with a broad spectrum of disease using electrocardiographic imaging (a method for noninvasive cardiac electrophysiology mapping) and advanced late gadolinium enhancement cardiac magnetic resonance scar imaging. Compared with 20 healthy controls, ARVC patients had longer ventricular activation duration (median, 52 versus 42 ms; P=0.007) and prolonged mean epicardial activation-recovery intervals (a surrogate for local action potential duration; median, 275 versus 241 ms; P=0.014). In these patients, we observed abnormal and varied epicardial activation breakthrough locations and regions of nonuniform conduction and fractionated electrograms. Nonuniform conduction and fractionated electrograms were present in the early concealed phase of ARVC. Electrophysiological abnormalities colocalized with late gadolinium enhancement scar, indicating a relationship with structural disease. Premature ventricular contractions were common in ARVC patients with variable initiation sites in both ventricles. Premature ventricular contraction rate increased with exercise, and within anatomic segments, it correlated with prolonged repolarization, electric markers of scar, and late gadolinium enhancement (all P < 0.001).Conclusions-Electrocardiographic imaging reveals electrophysiological substrate properties that differ in ARVC patients compared with healthy controls. A novel mechanistic finding is the presence of repolarization abnormalities in regions where ventricular ectopy originates. The results suggest a potential role for electrocardiographic imaging and late gadolinium enhancement in early diagnosis and noninvasive follow-up of ARVC patients.