Cardiology Division, University of California, San Francisco, San Francisco, California.
Cardiology Division, University of California, San Francisco, San Francisco, California.
Heart Rhythm. 2020 Feb;17(2):273-281. doi: 10.1016/j.hrthm.2019.09.013. Epub 2019 Sep 12.
Early repolarization syndrome (ERS) is a rare but increasingly recognized cause of malignant ventricular arrhythmias.
The purpose of this study was to characterize the presentations and treatments of ERS at our institution.
We performed a retrospective chart review of all patients presenting to our institution between 2008 and 2019 with ERS. Exclusion criteria included Brugada syndrome, positive provocative testing with class I antiarrhythmic drugs, metabolic disturbances, or structural heart disease.
Of 10 patients identified with ERS, 8 were men with a mean age of 30 ± 17 years at diagnosis. Documented arrhythmias included ventricular fibrillation in 7 of 10, polymorphic ventricular tachycardia in 3 of 10, and monomorphic ventricular tachycardia in 3 of 10 patients. Atrial fibrillation was diagnosed in 3 of 10, and atrioventricular block was seen in 2 of 10. J waves and/or electrocardiographic early repolarization patterns were dynamic in 7 of 10. Arrhythmias occurred at rest in 8 of 10 and with exertion in 2 of 10. Only 1 patient had a family history of sudden death, and 4 of 10 patients had variants of uncertain significance on genetic testing. Quinidine effectively suppressed arrhythmias in 5 of 5 patients but required dose escalation to >1 g/d in 3 of 5 patients. Abnormal epicardial electrograms were recorded over the inferolateral left ventricle in 2 patients who underwent mapping and were successfully ablated. Premature ventricular contraction triggers were also targeted for ablation in 3 patients.
ERS is a heterogeneous condition and may be associated with both atrial and ventricular arrhythmias, atrioventricular block, dynamic electrocardiographic changes, and variable triggers. In addition to targeting premature ventricular contraction triggers, mapping and ablation of abnormal epicardial electrograms may be a potential future treatment strategy.
早期复极综合征（ERS）是一种罕见但日益被认识到的恶性室性心律失常的原因。
本研究旨在描述我们机构中 ERS 的表现和治疗方法。
我们对 2008 年至 2019 年期间在我们机构就诊的所有 ERS 患者进行了回顾性图表审查。排除标准包括 Brugada 综合征、I 类抗心律失常药物阳性激发试验、代谢紊乱或结构性心脏病。
在 10 名被诊断为 ERS 的患者中，有 8 名男性，诊断时的平均年龄为 30 ± 17 岁。记录的心律失常包括 10 例中的 7 例心室颤动、10 例中的 3 例多形性室性心动过速和 10 例中的 3 例单形性室性心动过速。10 例中诊断为心房颤动，10 例中有 2 例房室传导阻滞。10 例中有 7 例 J 波和/或心电图早期复极模式是动态的。心律失常在 10 例中的 8 例是在休息时发生的，在 10 例中的 2 例是在运动时发生的。只有 1 例患者有家族性猝死史，10 例中有 4 例患者在基因检测中有不确定意义的变异。奎尼丁在 5 例中的 5 例中有效抑制心律失常，但在 3 例中需要增加剂量至>1 g/d。在 2 例接受标测和成功消融的患者中，记录到心外膜异常电活动在左心室下外侧。还针对 3 例患者的室性早搏触发灶进行了消融。
ERS 是一种异质性疾病，可能与房性和室性心律失常、房室传导阻滞、心电图动态变化和不同的触发灶有关。除了针对室性早搏触发灶进行治疗外，标测和消融异常心外膜电活动可能是一种潜在的未来治疗策略。
