Korantzopoulos Panagiotis, Bechlioulis Aris, Lakkas Lampros, Naka Katerina K
1st Department of Cardiology, University of Ioannina, Stavros Niarchos Avenue, Ioannina, Greece.
Laboratory of Pacing and Electrophysiology, 1st and 2nd Department of Cardiology, University Hospital of Ioannina, Greece.
Eur Heart J Case Rep. 2019 Jun 1;3(2). doi: 10.1093/ehjcr/ytz086.
Cardiac electrical disturbances represent the most frequent cardiac manifestations of myotonic dystrophy Type 1 (MD1). Limited data suggest that the prevalence of Brugada syndrome in MD1 may be increased compared to the general population.
We report a case of a 22-year-old asymptomatic man with repolarization abnormalities in leads V1-V3 suggestive of Type III Brugada pattern. The patient had a family history of MD and incidents of sudden death in relatives. Drug-induced Brugada Type 1 syndrome was revealed after procainamide challenge. A ventricular stimulation study was positive since a polymorphic ventricular tachycardia was induced after two extrastimuli. The patient underwent implantation of a single chamber cardiac defibrillator (ICD). Eight months after the procedure he suffered an appropriate ICD shock due to rapid polymorphic ventricular tachycardia.
Brugada syndrome is linked with MD1. Potential life-threatening arrhythmias may develop in the adult life of MD1 patients. Electrocardiographic surveillance and tailored invasive treatment with ICDs can prevent sudden cardiac death in this setting.
心脏电紊乱是1型强直性肌营养不良（MD1）最常见的心脏表现。有限的数据表明，与普通人群相比，MD1患者中布加综合征的患病率可能更高。
我们报告一例22岁无症状男性病例，其V1-V3导联存在复极异常，提示III型布加图形。该患者有MD家族史，亲属中有猝死事件。普鲁卡因胺激发试验后发现药物性1型布加综合征。心室刺激试验呈阳性，因为在两个额外刺激后诱发了多形性室性心动过速。该患者接受了单腔心脏除颤器（ICD）植入术。术后八个月，他因快速多形性室性心动过速接受了适当的ICD电击。
布加综合征与MD1有关。MD1患者成年后可能会发生潜在的危及生命的心律失常。心电图监测和使用ICD进行有针对性的侵入性治疗可以预防这种情况下的心源性猝死。
