Management of brugada syndrome: Should all high risk patients receive an ICD?

Management of Brugada Syndrome:
Should All High Risk Patients Receive an ICD?
Ph.D. Pham Huu Van. 
Introduction
• More than 20 years ago, the Brugada brothers reported 8 Pts with recurrent 
episodes of aborted sudden death and no demonstrable heart disease having 
a peculiar ECG pattern of ST elevation in right precordial leads
• BrS, one of the most devastating causes of SCD in relatively young Pts with 
apparently normal heart, was born.
• Recent publication of several works dealing with various modes of 
management of BrS has raised questions about the optimal treatment that 
should be offered to these Pts.
• Meregalli PG. Cardiovasc Res. 2005. Brugada P, J Am Coll Cardiol. 1992
General Considerations
Importance of ECG Phenotype Ascertainment (1)
• Although there has been general initial consensus about the ECG 
definition of Brugada ECG pattern into 3 types (type 1, type 2, and type 3)
• There has been a recent effort to establish a more simplified mode of 
classification, including only 2 ECG patterns6: pattern 1 identical to the 
classic type 1 of other consensus (coved pattern) and pattern 2 that joins 
patterns 2 and 3 of previous consensus (saddle-back pattern).
• Rodríguez-Mañero M, Heart Rhythm. 2016. Olde Nordkamp LR. Heart Rhythm. 2015. Sieira J, Circ Arrhythm
Electrophysiol. 2015
General Considerations
Importance of ECG Phenotype Ascertainment (2)
• This is in agreement with the classification presently adopted 
by most specialists that differentiates Brugada ECG type 1 
versus nontype 1 patterns.
• To date, only the ECG type 1 pattern, spontaneously evident or 
induced by a provocative drug challenge, should be considered 
as BrS diagnosis and to avoid unnecessary ICD implantations.
Clinical Presentation
• There are 3 main clinical presentations of BrS: 
• (1) polymorphic ventricular tachycardia (VT)/ventricular fibrillation 
(VF) associated with cardiac arrest (CA) or less frequently 
monomorphic VT; 
• (2) syncope; and 
• (3) no symptoms.
• Definition of the syncope group did not clearly indicate that patients with 
neurocardiogenic syncope were actually included in the asymptomatic group.
Arrhythmic Risk Stratification
• The arrhythmic risk stratification of BrS has been the subject of 
many studies. 
• The topic has been extensively reviewed by Adler et al. 
• An important pooled analysis of programmed ventricular 
stimulation (PVS) for risk stratification has been recently 
reported by Sroubek et al.
• Adler A, Heart Rhythm. 2016. Sroubek J. Circulation. 2016
Clinical parameters
• The risk of arrhythmic event (AE) is highest for Pts presenting with CA, 
intermediate for Pts with syncope, and lowest for Pts with asymptoms.
• Age, sex, and genetics: gene carriers among males and females is similar,
the majority of diagnosed Pts are male. First AEs usually occur between 
35 and 55 years and very rarely in children or the elderly. 
• SCN5A mutations are found in ≈30% of BrS Pts. 
• Large registries have not found an association between the risk of VF 
and a family history of SCD or mutations in the SCN5A gene.
ECG parameters
• A spontaneous type 1 Brugada ECG is an independent predictor of VF. 
• In the largest multicenter study:
• Annual incidences of AE in asymptomatic Pts with spontaneous and drug induced 
type 1 ECG were 0.8% and 0.4%, respectively, during a mean follow-up period of 
31 months.
• Sieira J, Circ Arrhythm Electrophysiol. 2015
ECG parameters (contj)
• The presence of early repolarization
• in peripheral leads, 
• fragmented QRS complexes,
• late potentials, 
• microscopic T wave alternans,
• and wide or large S wave in lead I have also 
• Shown promising results for predicting arrhythmic risk
• Calò L, J Am Coll Cardiol. 2016
Value of PVS
• Following the initial results of the Brugada group showing high value of 
PVS in predicting arrhythmic risk in BrS Pts.
• Subsequent several large studies, including FINGER, PRELUDE, and 2 
meta-analysis failed to confirm these results
• Belhassen B,. Circ Arrhythm Electrophysiol. 2015
Value of PVS (contj)
• It is only recently, after the latest meta-analysis by Fauchier et al: VF 
inducibility presented a hazard ratio for AE of 8.3 (CI: 95% 3.6–19.4), P 
< 0.01.
• This analysis showed that arrhythmia induction during PVS was 
associated with a 2.7-fold increased risk of AE over a median follow-
up of 38 months. 
• The risk was greatest among individuals who had their arrhythmias 
induced with single or double extrastimuli.
Latest Consensus Statement
• In addition to lifestyle changes (avoidance of drugs that may induce or aggravate 
ST-segment elevation in right precordial leads)
• Avoidance of excessive alcohol intake,
• Immediate treatment of fever with antipyretic drugs, 
• these guidelines recommend the following:
Latest Consensus Statement (contj)
• These guidelines recommend the following:
• ICD implantation: as class I indication for BrS Pts with CA survivors or have 
documented spontaneous sustained VT with or without syncope. 
• Also considered useful in Pts with a spontaneous diagnostic type 1 ECG who have 
a history of syncope judged to be likely caused by VA (class IIa).
• ICD may be considered in Pts with a diagnosis of BrS who develop VF during PVS 
(class IIb).
• In contrast, ICD is not indicated in asymptomatic BrS Pts with a drug-induced type 1 
ECG and on the basis of a family history of SCD alone.
Latest Consensus Statement (contj)
• Quinidine: this medication can be useful in Pts with a diagnosis of BrS and 
history of arrhythmic storms. 
• This drug can also be useful in Pts with a diagnosis of BrS who qualify for an ICD 
but present a contraindication to the ICD or refuse it and have a history of 
documented SVT that require treatment (class IIa). 
• Finally, it may be considered in asymptomatic Pts with a diagnosis of BrS with a 
spontaneous type 1 ECG (class IIb)
Latest Consensus Statement (contj)
• Catheter ablation: this procedure may be considered in 
patients with a diagnosis of BrS and history of arrhythmic 
storms or repeated appropriate ICD shocks.
Presently Available Modalities of Treatment
• There are presently 5 modalities of treatment of BrS patients:
• (1) no treatment; 
• (2) ICD implantation; 
• (3) EP-guided class 1A antiarrhythmic therapy (mainly quinidine); 
• (4) empirical quinidine therapy; and 
• (5) epicardial ablation. 
• As stated earlier, these modalities of treatment only concern the Pts
with the Brugada ECG type 1 (spontaneous or drug-induced).
• We will not deal here with the issue of the management of
• arrhythmic storms.
Annual Incidence Rate of Arrhythmic Events in Asymptomatic Pts Found to 
Have No Inducible Arrhythmias, According to Brugada ECG Pattern and 
PVS Protocol
Strategy of management of patients with Brugada syndrome who are cardiac arrest survivors or have 
syncope suspected to have an arrhythmic origin. CA indicates cardiac
arrest; EPS, electrophysiological study; ICD, implantable cardioverter defibrillator; PVS, programmed 
ventricular stimulation; QND, quinidine; and Tx, therapy.
• Strategy of management of patients with Brugada syndrome who are asymptomatic or have neurocardiogenic
syncope. EPS indicates electrophysiological study; ICD, implantable cardioverter defibrillator; PVS, programmed 
ventricular stimulation; QND, quinidine; and Tx, therapy.
Conclusions
• From the experience accumulated to date, it seems ICDs no longer warrant the 
quote: “only therapy with proven efficacy for the management of Pts with BrS”. 
• It would be more appropriate to state: there are presently several possible 
modes of management of the BrS, each of them presenting advantages and 
disadvantages.
• A multicenter study performed in institutions where all modes of therapy are 
available is certainly warranted: most research efforts will focus on comparison 
of ICD therapy and epicardial ablation.
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