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Title: [Analysis of 30 cases of inherited cardiac arrhythmia syndrome in children].
Author: Li ZL, Zeng SY, Liang DP, Liu T, Wang SS, Zhang ZW.
Journal: Zhonghua Er Ke Za Zhi; 2019 Sep 02; 57(9):700-704. PubMed ID: 31530356.
Abstract:
Objective: To analyze and summarize the diagnosis and treatment experience of common inherited cardiac arrhythmia syndrome in pediatric patients, and explore the most appropriate therapy. Methods: A retrospective review identified 30 pediatric cases (19 males, 11 females) diagnosed with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), arrhythmogenc right ventricular cardiomyopathy (ARVC) from January 2008 to December 2018 in the Pediatric Cardiology Department, Guangdong Provincial People's Hospital. Data obtained included the diagnosis, treatment and follow-up outcome. Results: The most common inherited cardiac arrhythmia syndromes were LQTS (n=14) including 1 case with epilepsy, CPVT (n=5), HCM (n=7), ARVC (n=1), and BrS (n=3). Twenty-seven cases were admitted to hospital due to syncope, whereas the remaining 3 cases of BrS had not presented with syncope before admission. The average onset age of inherited arrhythmia was (10.0±3.3) years. Genetic testing was performed on 20 patients. The median follow-up time was 40 months. Among 15 patients who underwent implantable cardioverter defibrillator (ICD) and survived, 2 patients had frequent ICD discharge. One patient underwent radiofrequency ablation, and the other one received left cardiac sympathetic denervation and an increased ICD defibrillation threshold, and the number of ICD discharge was significantly reduced. Among 10 patients who received drug therapy, 4 patients including two patients who discontinued treatment without advices died. Two patients whose parents refused treatment died, 1 case diagnosed with unexplained sudden cerebral death, and the remaining 2 cases without indication for drug therapy survived without any treatment. Conclusions: Mortality rate is high in pediatric patients with inherited cardiac arrhythmia and syncope. The therapeutic effect of drugs are not satisfactory, ICD implantation is the most effective treatment to prevent sudden cardiac death currently, but the postoperative frequent discharge should be brought to the forefront and handled in time. 目的： 分析儿童常见遗传性心律失常的诊治经验。 方法： 回顾性分析广东省人民医院心儿科2008年1月至2018年12月确诊为长QT综合征（LQTS）、Brugada综合征（BrS）、儿茶酚胺敏感性多形性室性心动过速（CPVT）、肥厚型心肌病（HCM）和致心律失常性右室心肌病（ARVC）的30例患儿（男19例、女11例）的诊治以及随访结果。 结果： 30例患儿中，诊断为LQTS 14例（其中1例合并癫痫）、CPVT 5例、HCM 7例、ARVC 1例、BrS 3例。3例BrS患儿入院前未发生过晕厥，余27例患儿均因晕厥入院。初次发病年龄为（10.0±3.3）岁。20例有基因检测，随访时间为3.3（0.3，10.7）年。植入心律转复除颤器（ICD）15例全部存活，其中2例出现ICD频繁放电，1例行射频消融术，1例行左心交感神经切除术并提高ICD起搏频率后ICD放电次数明显减少；药物治疗10例死亡4例，其中2例自行停药后死亡；家属放弃治疗2例均死亡；1例诊断未明突发脑死亡，2例无药物治疗指征，未行特殊治疗存活。 结论： 遗传性心律失常伴晕厥的患儿猝死率高，药物治疗效果多不理想，植入ICD是预防心源性猝死最有效的方法，但术后的频繁放电需及时处理。.

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