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ª 2 0 1 9 T H E A U T H O R S . P U B L I S H E D B Y E L S E V I E R O N B E H A L F O F T H E A M E R I C A N

C O L L E G E O F C A R D I O L O G Y F OU N D A T I O N . T H I S I S A N O P E N A C C E S S A R T I C L E U N D E R

T H E C C B Y - N C - N D L I C E N S E ( h t t p : / / c r e a t i v e c o mm o n s . o r g / l i c e n s e s / b y - n c - n d / 4 . 0 / ) .

IMAGING VIGNETTE

ECG CHALLENGE

Brugada Syndrome

Death Reveals its Perpetrator

Kiersten Frenchu, MD, Shane Flood, MD, Lauren Rousseau, MPAS, PA-C, CCDS, Athena Poppas, MD, Antony Chu, MD

ABSTRACT

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Rapid diagnosis of Brugada syndrome is critical to therapy, which is aimed at reversing provoking factors to

suppress/terminate malignant arrhythmias. This case highlights the diagnosis and peri-operative management of

patients with Brugada syndrome at high risk for sudden cardiac death. (Level of Difficulty: Beginner.)

(J Am Coll Cardiol Case Rep 2019;1:55–6) © 2019 The Authors. Published by Elsevier on behalf of the

American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

B rugada syndrome is an inherited channelopathy that confers an increased risk of sudden cardiac death(SCD). Approximately 25% of individuals affected with Brugada syndrome demonstrate a mutation inthe SCN5a sodium channel (1). Baseline electrocardiography (ECG) results may be electrophysiologi-

cally normal and therefore confound the diagnosis. Provoking factors may be required to unmask the BrugadaECG pattern (2). This case highlights the discovery and management of a patient with heretofore undiagnosedBrugada syndrome.

SEE PAGE 57

CASE REPORT

A 50-year-old female with a history of essential hypertension and hyperlipidemia presented with fever andabdominal pain. She denied any chest pain, palpitations, or syncope prior to admission, and acute appendicitiswas diagnosed. Her baseline ECG was unremarkable. Pre-operatively, she developed spontaneous ventricularfibrillation (VF) and was found unresponsive and required a 200-J biphasic shock for termination. Afterdefibrillation, her ECG revealed new ST-segment elevations, right bundle branch pattern, and coved, persis-tently elevated ST-segments in V1 and V2, consistent with type 1 Brugada syndrome (Figure 1).

DISCUSSION

Treatment of this patient required rapid diagnosis of Brugada syndrome by ECG findings and clinical history.This patient’s post-arrest ECG is demonstrative for type 1 Brugada, with coved ST-segment elevations of>2 mm in V1 to V2 and a negative T-wave in the setting of right bundle branch block (1,2). Importantly, the QTccomplex is normal in patients with Brugada syndrome as the most common mutations involve sodium channeldefects (as opposed to potassium channel defects that lead to QTc abnormalities).

Treatment of Brugada syndrome is aimed at prevention of SCD with an automatic implantable cardioverter-defibrillator (AICD); however, that was contraindicated in this patient due to her active infection (2). She wasmanaged acutely with quinidine, the most effective general therapy, with a plan to administer isoproterenol toprevent further episodes of arrhythmia if needed for electrical storm (2). Therapy consisting of hydration,

N 2666-0849 https://doi.org/10.1016/j.jaccas.2019.05.016

m the Department of Internal Medicine, Brown University Rhode Island Hospital, Providence, Rhode Island. The authors have

orted that they have no relationships relevant to the contents of this paper to disclose.

nuscript received April 1, 2019; revised manuscript received April 29, 2019, accepted May 2, 2019.

FIGURE 1 Type 1 Brugada Syndrome

Frenchu et al. J A C C : C A S E R E P O R T S , V O L . 1 , N O . 1 , 2 0 1 9

Brugada Syndrome: Death Reveals its Perpetrator J U N E 2 0 1 9 : 5 5 – 6

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electrolyte replenishment, and antipyretic administration was optimized. In regard to management, there arecurrently conflicting views of the use of amiodarone and lidocaine in Brugada syndrome and evidence thatprocainamide should be avoided (2).

Perioperatively, the patient received an infusion of isoproterenol. She underwent an uncomplicated lapa-roscopic appendectomy, with slow peritoneal insufflation and deflation to avoid increased vagal tone, anothertrigger for ventricular arrhythmias in the setting of Brugada syndrome.

The patient had no further arrhythmia episodes. Her echocardiogram result was normal. Prior to discharge,an AICD was placed after her infection resolved. Later, she reported that her father had died suddenly at age 45years. Genetic testing was recommended for her and her first-degree family members.

CONCLUSIONS

Rapid diagnosis of Brugada syndrome unmasked by triggers is critical to therapy designed to reverse provokingfactors and suppress or terminate lethal episodes of ventricular arrhythmia. This case outlines perioperativemanagement of patients with Brugada syndrome at high risk for SCD, particularly those with an index pre-sentation secondary to acute illness. Following is an overview of Brugada management:

� Acute management of Brugada syndrome includes optimizing hydration, correcting electrolyte abnormal-ities, and using antipyretics and quinidine.

� A secondary preventative of SCD from type 1 Brugada syndrome following VF arrest involves placement ofan AICD.

� For patients undergoing surgery, vigilant planning is required.� Genetic testing should be recommended to the patient and the patient’s first-degree family members.

ADDRESS FOR CORRESPONDENCE: Dr. Kiersten Frenchu, Brown Medicine, 245 Chapman Street, ProvidenceRhode Island 02905. E-mail: kiersten.frenchu@lifespan.org.

RE F E RENCE S

1. Juang JJ, Horie M. Genetics of Brugada syn-drome. J Arrhythm 2016;32:418–25.

2. Antzelevitch C, Fish JM. Therapy for the Bru-gada syndrome. Handb Exp Pharmacol 2006:305–30.

KEY WORDS electrocardiography, suddencardiac death