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J Ding R Rahmat S A Cha

Abstract

INTRODUCTION


Brugada syndrome is a cardiac sodium channelopathy associated with one of several ECG patterns characterized by incomplete right bundle-branch block and ST elevations in the anterior precordial leads. Autosomal dominant inheritance is found in 50% of case. It affects young males with structurally normal hearts who are prone to develop ventricular tachyarrhythmias leading to syncope, cardiac arrest, or sudden cardiac death. 


CASE REPORT


An apparently heathy 35 yearold man suddenly collapses after complaining of dyspnoea. There was no prior history of chest pain, palpitations or syncope. He arrived in the emergency department with asystole. He was resuscitated as per ALS guidelines, ventilated, started on chest compressions and boluses of IV adrenaline. He then develop ventricular fibrillation, was defibrillated multiple times and given IV amiodarone. A return of spontaneous circulation was achieved after 40 mins. An ECG done showed ST elevation in aVR and widespread ST depression and the cause of his cardiac arrest was attributed to acute coronary syndrome. However an ECG done 2 hours later revealed a coved ST elevation V1-V2 followed by negative T wave, rearing the “ugly head” of Brugada syndrome. The further episodes of ventricular tachyarrhythmias and hemodynamic instability precluded transfer to a tertiary center. He succumbed the following day.


DISCUSSION


Brugada syndrome is diagnosed by both ECG and clinical criteria. There are 3 types of ECG abnormality but only type I (Brugada sign) is potentially diagnostic as in this case. One clinical criterion must also be fulfilled: Documented VF or VT, Family history of sudden cardiac death at <45 years old, Coved-type ECGs in family members, Inducibility of VT with programmed electrical stimulation, syncope or nocturnal agonal respiration. The only proven therapy is an implantable cardioverter-defibrillator. Quinidine is a possible alternative.


CONCLUSION


Diagnosis and early intervention of Brugada syndrome is key to preventing sudden death in affected young adults.

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