法布里病的心电图特征性分析

杨长根; 余扬; 周炳元

doi:10.13201/j.issn.1001-1439.2020.06.017

法布里病的心电图特征性分析

- 苏州大学附属第一医院心血管内科(江苏苏州, 215006)

详细信息

通讯作者: 周炳元,E-mail:zhoubinyuan@sina.com

中图分类号: R596

收稿日期: 2019-11-04

修回日期: 2020-03-31

Analysis of electrocardiogram characteristics in patients with Fabry disease

- Department of Cardiovascular Medicine, the First Affiliated Hospital of Soochow University, Suzhou, Jiangsu, 215006, China

More Information

Corresponding author: ZHOU Bingyuan, E-mail: zhoubinyuan@sina.com

Received Date: 04 November 2019

Revised Date: 31 March 2020

摘要

摘要: 回顾性分析我院随访的3家系12例法布里病（FD）患者心电图特征。根据我院3例先证者及其家系基因检测结果，从典型和非典型症状来分析12例患者的心电图特征，并复习相关文献。3家系12例患者中（男4例、女8例），ST-T段改变7例（58.3%），短PR间期6例（50%），传导阻滞4例（33.3%），心律失常4例（33.3%），永久起搏器植入1例（8.3%）；其中典型症状者，心电图主要表现为短PR间期和T波倒置；非典型症状者，心电图主要表现为T波倒置或短PR间期。FD特征性心电图表现，可能有助于对FD的早期诊断。
- 法布里病 /
- 心电图 /
- 短PR间期
Abstract: The electrocardiographic characteristics of 12 patients with Fabry disease(FD) from 3 families followed up in our hospital were analyzed retrospectively. According to the gene detection results of 3 probands and their families in our hospital, the ECG characteristics of 12 patients were retrospectively analyzed from typical symptoms and atypical symptoms, and the related literatures were reviewed. Among the 12 patients in 3 families(male 4; female 8), ST-T segment changes were found in 7 cases(58.3%), short PR interval in 6 cases(50%), conduction block in 4 cases(33.3%), arrhythmia in 4 cases(33.3%), and permanent pacemaker implantation in 1 case(8.3%);In patients with typical symptoms, the main electrocardiographic manifestations were short PR interval and T wave inversion. In patients with atypical symptoms, the main electrocardiographic manifestations were T wave inversion or short PR interval. The characteristic ECG of FD may be helpful for the early diagnosis of FD.
- Fabry disease /
- electrocardiogram /
- short PR interval

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参考文献(9)

[1]	Cianciulli TF, Saccheri MC, Rísolo MA, et al.Mechanical dispersion in Fabry disease assessed with speckle tracking echocardiography[J].Echocardiography, 2020, 37(2):293-301.
[2]	Hagège A, Réant P, Habib G, et al.Fabry disease in cardiology practice:Literature review and expert point of view[J].Arch Cardiovasc Dis, 2019, 112(4):278-287.
[3]	Yeung DF, Sirrs S, Tsang M, et al.Echocardiographic assessment of patients with Fabry disease[J].J Am Soc Echocardiogr, 2018, 31(6):639-649.e2.
[4]	Perry R, Shah R, Saiedi M, et al.The role of cardiac imaging in the diagnosis and management of Anderson-Fabry disease[J].JACC Cardiovasc Imaging, 2019, 12(7 Pt 1):1230-1242.
[5]	Simonetta I, Tuttolomondo A, Di Chiara T, et al.Genetics and gene therapy of Anderson-Fabry disease[J].Curr Gene Ther, 2018, 18(2):96-106.
[6]	Yuasa T, Takenaka T, Higuchi K, et al.Fabry disease[J].J Echocardiogr, 2017, 15(4):151-157.
[7]	Serra W, Marziliano N.Role of cardiac imaging in Anderson-Fabry cardiomyopathy[J].Cardiovasc Ultrasound, 2019, 17(1):1.
[8]	Lee SN, Kim GH, Yoo KD. A case of a 50-year-old woman with typical Fabry disease who showed serial electrocardiographic and echocardiographic changes over a 17-year period[J]. Case Rep Cardiol, 2019, 2019:9385361.
[9]	Vijapurapu R, Kozor R, Hughes DA, et al.A randomised controlled trial evaluating arrhythmia burden, risk of sudden cardiac death and stroke in patients with Fabry disease:the role of implantable loop recorders (RaILRoAD) compared with current standard practice[J].Trials, 2019, 20(1):314.