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J波综合征：良性还是恶性？

J wave syndrome: Benign or malignant?

作者信息

Sherafati Alborz, Eslami Masoud, Mollazadeh Reza

机构信息

Assistant Professor, Department of Cardiology, School of Medicine, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

Associate Professor, Department of Cardiology, School of Medicine, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran.

出版信息

ARYA Atheroscler. 2021 Jul;17(4):1-9. doi: 10.22122/arya.v17i0.2259.

DOI:10.22122/arya.v17i0.2259
PMID:35685231
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9137236/
Abstract

J wave syndrome is an electrical disease of the heart due to pathologic early repolarization. It encompasses a clinical spectrum from aborted sudden cardiac death due to ventricular arrhythmia (VA) usually in young affected patients to self-terminating ventricular ectopies, and finally, asymptomatic relatives of probands detected during electrocardiography acquisition (early repolarization pattern). This syndrome consists of 2 phenotypes, early repolarization and Brugada syndrome. Herein, we first describe 2 patients with early repolarization and Brugada syndrome, then, discuss their definition, epidemiology, genetics, cellular mechanism, diagnosis, risk stratification, and finally, therapeutic challenges and options one by one in detail.

摘要

J波综合征是一种因病理性早期复极所致的心脏电疾病。它涵盖了一系列临床情况，从通常在年轻患病患者中因室性心律失常（VA）导致的心脏骤停猝死，到自行终止的室性早搏，最后是在心电图检查时检测出的先证者无症状亲属（早期复极模式）。该综合征包括两种表型，即早期复极和Brugada综合征。在此，我们首先描述2例早期复极和Brugada综合征患者，然后详细逐一讨论它们的定义、流行病学、遗传学、细胞机制、诊断、风险分层，以及最后的治疗挑战和选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/91a56a7ae523/ARYA-17-6-2259f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/f6fdf972ad12/ARYA-17-6-2259f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/71e0168ebdea/ARYA-17-6-2259f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/f5a6736b77c7/ARYA-17-6-2259f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/91a56a7ae523/ARYA-17-6-2259f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/f6fdf972ad12/ARYA-17-6-2259f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/71e0168ebdea/ARYA-17-6-2259f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/f5a6736b77c7/ARYA-17-6-2259f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dadd/9137236/91a56a7ae523/ARYA-17-6-2259f4.jpg

相似文献

1
J wave syndrome: Benign or malignant?J波综合征：良性还是恶性？
ARYA Atheroscler. 2021 Jul;17(4):1-9. doi: 10.22122/arya.v17i0.2259.
2
[Doubts of the cardiologist regarding an electrocardiogram presenting QRS V1-V2 complexes with positive terminal wave and ST segment elevation. Consensus Conference promoted by the Italian Cardiology Society].[心脏病专家对一份心电图的疑问：该心电图显示V1 - V2导联QRS波群终末波正向且ST段抬高。意大利心脏病学会组织的共识会议]
G Ital Cardiol (Rome). 2010 Nov;11(11 Suppl 2):3S-22S.
3
J Wave Syndrome: Clinical Diagnosis, Risk Stratification and Treatment.J波综合征：临床诊断、风险分层与治疗
J Atr Fibrillation. 2014 Dec 31;7(4):1173. doi: 10.4022/jafib.1173. eCollection 2014 Dec.
4
Transmural dispersion of repolarization and arrhythmogenicity: the Brugada syndrome versus the long QT syndrome.复极跨壁离散度与致心律失常性：Brugada综合征与长QT综合征
J Electrocardiol. 1999;32 Suppl:158-65. doi: 10.1016/s0022-0736(99)90074-2.
5
Early repolarization syndrome: electrocardiographic signs and clinical implications.早期复极综合征：心电图表现及临床意义。
Ann Noninvasive Electrocardiol. 2014 Jan;19(1):15-22. doi: 10.1111/anec.12113. Epub 2013 Sep 30.
6
J-wave syndromes. from cell to bedside.J波综合征：从细胞到床边
J Electrocardiol. 2011 Nov-Dec;44(6):656-61. doi: 10.1016/j.jelectrocard.2011.07.026. Epub 2011 Sep 9.
7
High prevalence of early repolarization in the paediatric relatives of sudden arrhythmic death syndrome victims and in normal controls.早复极在儿童心律失常性猝死综合征患者亲属和正常对照中的高发生率。
Europace. 2017 Aug 1;19(8):1385-1391. doi: 10.1093/europace/euw248.
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Prevalence of early repolarization pattern in inferolateral leads in patients with Brugada syndrome.Brugada综合征患者下侧壁导联早期复极模式的患病率。
Heart Rhythm. 2008 Dec;5(12):1685-9. doi: 10.1016/j.hrthm.2008.09.021. Epub 2008 Sep 23.
9
The relationship between repolarization parameters and serum electrolyte levels in patients with J wave syndromes.J波综合征患者复极参数与血清电解质水平的关系。
Magnes Res. 2015 Jan-Mar;28(1):1-13. doi: 10.1684/mrh.2015.0379.
10
Prognostic significance of early repolarization in inferolateral leads in Brugada patients with documented ventricular fibrillation: a novel risk factor for Brugada syndrome with ventricular fibrillation.下壁导联早期复极在伴有室颤记录的 Brugada 患者中的预后意义：Brugada 综合征伴室颤的新危险因素。
Heart Rhythm. 2013 Aug;10(8):1161-8. doi: 10.1016/j.hrthm.2013.04.009. Epub 2013 Apr 12.

本文引用的文献

1
Brugada Syndrome: Clinical Care Amidst Pathophysiological Uncertainty.Brugada 综合征：病理生理学不确定性中的临床护理。
Heart Lung Circ. 2020 Apr;29(4):538-546. doi: 10.1016/j.hlc.2019.11.016. Epub 2019 Dec 17.
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Long-term prognosis of patients withJ-wave syndrome.J 波综合征患者的长期预后。
Heart. 2020 Feb;106(4):299-306. doi: 10.1136/heartjnl-2019-315007. Epub 2019 Aug 7.
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Early Repolarization Syndrome: Diagnostic and Therapeutic Approach.早期复极综合征：诊断与治疗方法
Front Cardiovasc Med. 2018 Nov 27;5:169. doi: 10.3389/fcvm.2018.00169. eCollection 2018.
4
Concomitant SK current activation and sodium current inhibition cause J wave syndrome.同时激活 SK 电流和抑制钠电流会导致 J 波综合征。
JCI Insight. 2018 Nov 15;3(22):122329. doi: 10.1172/jci.insight.122329.
5
Depolarization versus repolarization abnormality underlying inferolateral J-wave syndromes: New concepts in sudden cardiac death with apparently normal hearts.下壁 J 波综合征的去极化与复极化异常：看似正常心脏的心脏性猝死新观念。
Heart Rhythm. 2019 May;16(5):781-790. doi: 10.1016/j.hrthm.2018.10.040. Epub 2018 Nov 2.
6
Sudden cardiac death in J wave syndrome with short QT associated to a novel mutation in Na 1.8 coding gene SCN10A: First case report for a possible pharmacogenomic role.与钠通道1.8编码基因SCN10A的新突变相关的短QT间期J波综合征患者的心脏性猝死：首例可能具有药物基因组学作用的病例报告
J Electrocardiol. 2018 Sep-Oct;51(5):809-813. doi: 10.1016/j.jelectrocard.2018.06.005. Epub 2018 Jun 9.
7
2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.2017年美国心脏协会/美国心脏病学会/心律学会室性心律失常患者管理和心脏性猝死预防指南：美国心脏病学会/美国心脏协会临床实践指南工作组和心律学会的报告
J Am Coll Cardiol. 2018 Oct 2;72(14):e91-e220. doi: 10.1016/j.jacc.2017.10.054. Epub 2018 Aug 16.
8
Electrophysiological analyses of transgenic mice overexpressing KCNJ8 with S422L mutation in cardiomyocytes.对心肌细胞中过表达带有S422L突变的KCNJ8的转基因小鼠进行电生理分析。
J Pharmacol Sci. 2017 Sep;135(1):37-43. doi: 10.1016/j.jphs.2017.08.009. Epub 2017 Sep 6.
9
Low room temperature can trigger ventricular fibrillation in J wave syndromes.低室温可引发J波综合征患者的心室颤动。
HeartRhythm Case Rep. 2016 Apr 14;2(4):347-350. doi: 10.1016/j.hrcr.2016.04.003. eCollection 2016 Jul.
10
Ankyrin-2 variants associated with idiopathic ventricular fibrillation storm in patients with intermittent early repolarization pattern.与间歇性早期复极模式患者特发性室颤风暴相关的锚蛋白-2变体
HeartRhythm Case Rep. 2015 Jun 3;1(5):337-341. doi: 10.1016/j.hrcr.2015.05.008. eCollection 2015 Sep.