Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
Europace. 2023 Jul 4;25(7). doi: 10.1093/europace/euad197.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive inherited cardiac disease. Early detection of disease and risk stratification remain challenging due to heterogeneous phenotypic expression. The standard configuration of the 12 lead electrocardiogram (ECG) might be insensitive to identify subtle ECG abnormalities. We hypothesized that body surface potential mapping (BSPM) may be more sensitive to detect subtle ECG abnormalities.
We obtained 67 electrode BSPM in plakophilin-2 (PKP2)-pathogenic variant carriers and control subjects. Subject-specific computed tomography/magnetic resonance imaging based models of the heart/torso and electrode positions were created. Cardiac activation and recovery patterns were visualized with QRS- and STT-isopotential map series on subject-specific geometries to relate QRS-/STT-patterns to cardiac anatomy and electrode positions. To detect early signs of functional/structural heart disease, we also obtained right ventricular (RV) echocardiographic deformation imaging. Body surface potential mapping was obtained in 25 controls and 42 PKP2-pathogenic variant carriers. We identified five distinct abnormal QRS-patterns and four distinct abnormal STT-patterns in the isopotential map series of 31/42 variant carriers. Of these 31 variant carriers, 17 showed no depolarization or repolarization abnormalities in the 12 lead ECG. Of the 19 pre-clinical variant carriers, 12 had normal RV-deformation patterns, while 7/12 showed abnormal QRS- and/or STT-patterns.
Assessing depolarization and repolarization by BSPM may help in the quest for early detection of disease in variant carriers since abnormal QRS- and/or STT-patterns were found in variant carriers with a normal 12 lead ECG. Because electrical abnormalities were observed in subjects with normal RV-deformation patterns, we hypothesize that electrical abnormalities develop prior to functional/structural abnormalities in ARVC.
致心律失常性右室心肌病（ARVC）是一种进行性遗传性心脏病。由于表型表达的异质性，疾病的早期发现和风险分层仍然具有挑战性。标准的 12 导联心电图（ECG）配置可能对识别细微的 ECG 异常不敏感。我们假设体表电位图（BSPM）可能更敏感地检测到细微的 ECG 异常。
我们在桥粒蛋白-2（PKP2）-致病性变异携带者和对照受试者中获得了 67 个电极 BSPM。创建了基于心脏/胸体的特定于个体的计算机断层扫描/磁共振成像模型和电极位置。使用基于个体几何形状的 QRS 和 STT 等电位图系列可视化心脏激活和恢复模式，将 QRS/STT 模式与心脏解剖和电极位置相关联。为了检测早期功能性/结构性心脏病的迹象，我们还获得了右心室（RV）超声心动图变形成像。我们在 25 名对照者和 42 名 PKP2-致病性变异携带者中获得了体表电位图。我们在 31/42 名变异携带者的等电位图系列中识别出 5 种不同的异常 QRS 模式和 4 种不同的异常 STT 模式。在这 31 名变异携带者中，17 名在 12 导联 ECG 中没有显示去极化或复极化异常。在 19 名临床前变异携带者中，12 名 RV 变形模式正常，而 7/12 名显示异常 QRS 和/或 STT 模式。
通过 BSPM 评估去极化和复极化可能有助于在变异携带者中寻找疾病的早期发现，因为在具有正常 12 导联 ECG 的变异携带者中发现了异常的 QRS 和/或 STT 模式。由于在 RV 变形模式正常的受试者中观察到电异常，我们假设 ARVC 中的电异常先于功能/结构异常发展。
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