Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration.
Atrial Standstill in the Pediatric Population: A Multi-Institution Collaboration.
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儿科人群的心房停搏：多机构合作。
DOI：
10.1016/j.jacep.2022.08.022
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发表时间：
2023
期刊：
影响因子：
--
通讯作者：
Ga
中科院分区：
文献类型：
--
作者：
Howard,TaylorS;Chiang,DavidY;Ceresnak,ScottR;Ladouceur,VirginieBeausejour;Whitehill,RobertD;Czosek,RichardJ;Knilans,TimothyK;Ahnfeldt,AgnetheM;Borresen,MaleneLando;Jaeggi,Edgar;Udupa,Sharmila;Gow,Robert;Moore,JeremyP;Ga
背景：心房静止（AS）是一种罕见的情况，其特征是心房内没有电活动。迄今为止的研究有限。目的作者试图描述AS患者的临床特征、遗传学和预后。方法：本研究是一项回顾性多中心研究，研究对象为诊断为AS时年龄<18岁的患者，定义为在电生理学研究、器械放置或无创心律描记中记录的心房活动缺失，并由超声心动图证实。排除了获得性障碍患者。记录和分析临床细节和基因变异。结果20例患者中位年龄为6.6岁（IQR: 2.9 ~ 10.8岁）。心律失常包括16例（80%）心房/室上性心律失常，8例（40%）室性心动过速，包括4例心脏骤停。1型Brugada模式在文献4中被记录。18例（90%）植入了起搏器。虽然15例患者尝试心房导联，但只有4例在植入时实现起搏。在中位随访6.9年（IQR: 1.2-13.3年）期间，7例（35%）发生血栓栓塞事件。其中，没有人进行心房起搏，6人未使用抗凝剂，1人使用阿司匹林。基因检测在13例（65%）患者中发现了scn5变异。分析表明scn5功能缺失可能是导致AS的一种机制。室性心律失常和心脏骤停在双等位基因scn5变异患者中更为常见。结论sa可能与scn5变异的功能丧失有关。患者表现出心房和室性心律失常，并可能在装置放置期间提出挑战。没有心房起搏能力的患者有血栓栓塞事件的风险，需要抗凝治疗。
BackgroundAtrial standstill (AS) is a rare condition characterized by absence of electrical activity within the atria. Studies to date have been limited.ObjectivesThe authors sought to describe the clinical characteristics, genetics, and outcomes of patients with AS.MethodsThis was a retrospective multicenter study of patients <18 years at AS diagnosis, defined as absence of atrial activity documented during an electrophysiology study, device placement, or noninvasive rhythm tracings and confirmed by echocardiogram. Patients with acquired disorders were excluded. Clinical details and genetic variants were recorded and analyzed.ResultsTwenty patients were diagnosed at a median age of 6.6 years (IQR: 2.9-10.8 years). Arrhythmias included 16 (80%) with atrial/supraventricular arrhythmias and 8 (40%) with ventricular tachycardia, including 4 with cardiac arrests. A type 1 Brugada pattern was documented in 4. Pacemakers were implanted in 18 (90%). Although atrial leads were attempted in 15, only 4 achieved pacing at implantation. During a median follow-up of 6.9 years (IQR: 1.2-13.3 years), 7 (35%) had thromboembolic events. Of these, none had atrial pacing, 6 were not on anticoagulation, and 1 was on aspirin. Genetic testing identifiedSCN5Avariants in 13 patients (65%). Analyses suggestSCN5Aloss-of-function may be one mechanism driving AS. Ventricular arrhythmias and cardiac arrest were more commonly seen in patients with biallelicSCN5Avariants.ConclusionsAS may be associated with loss-of-functionSCN5Avariants. Patients demonstrate atrial and ventricular arrhythmias, and may present challenges during device placement. Patients without the capacity for atrial pacing are at risk for thromboembolic events and warrant anticoagulation.
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