Open Access

Brugada syndrome in a young patient with type 1 myotonic dystrophy requiring an implantable cardioverter defibrillator for primary prevention: a case report

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Date: 17 June 2019
Journal: European Heart Journal - Case Reports , Volume 3 , Issue 2
Authors: P. Korantzopoulos , A. Bechlioulis , L. Lakkas , K. Naka , B. Dinov , K. Iliodromitis , R. Schönbauer , A. Vamvakidou , P. Patel

ESC Journals

AbstractBackground 

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.

Case summary 

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.

Discussion 

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.

About the contributors

Panagiotis Korantzopoulos

Ioannina (University of Ioannina Medical School)

Role: Author

Aris Bechlioulis

Role: Author

Lampros Lakkas

Role: Author