KATA: Σ όλος ος ασθνίς μ σύνρομο ... - Livemedia.gr · 6 basic studies on EP • Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals

Άννα ΚωστοπούλουΑναπλ. Διευθύντρια

Ωνάσειο Καρδιοχειρουργικό ΚέντροΤμήμα Ηλεκτροφυσιολογίας και Βηματοδότησης

KATA: Σε όλους τους ασθενείς με σύνδρομο BRUGADA και θετική ηλεκτροφυσιολογική

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6 basic studies on EP

• Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest.

Circulation. 2003; 108: 3092-3096.

• Paul M et al. Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data.

Eur Heart J. 2007; 28: 2126-2133.

• Probst V et al. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry.

Circulation. 2010 Feb 9;121(5):635-43.

• Priori SG et al. Risk stratification in Brugada syndrome: results of the PRELUDE (Programmed ELectrical stimUlation preDictive valuE) registry.

J Am Coll Cardiol. 2012; 59: 37-45.

• Sieira et at. Prognostic Value of Programmed Electrical Stimulation in Brugada Syndrome 20 Years Experience Circ Arrhythm Electrophysiol. 2015;8:777-784

• Juan Sieira, MD; Giulio Conte,Sroubek J et al. Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis.

Circulation. 2016;133(7):622-30.

Circulation. 2003;108:3092-3096

EPS

FU mean of 24+33 months (1 to 160)

Patients with BS and

survived SCA show

the highest chance

for VT/VF occurrence

during follow-up.

No significant role

of PVS with regard

to arrhythmic

events during

follow-up

• 1036 patients (85%) underwent EP-53% were inducible

• highest in SCA patients (66%)• 55% of with syncope • 25% of asymptomatic pts

Specific analysis of asymptomatic pts

11 European centres, 1.029 pts median follow-up of 32 months.

Symptoms and a

spontaneous type 1 ECG

are the only independent

predictors of arrhythmic

events.

Gender, family history of

SCD, inducibility of VT/VF

and presence of a mutation

in the SCN5A gene have

no predictive value.

Event rate in asymptomatic patients was

0.5% per year.

J Am Coll Cardiol 2012;59:37–45

308 consecutive individuals 247 men (80%) median age 44 years (18 -72) EPS at enrollmentFU Median follow-up of 34 months

(A) entire PRELUDE cohort

B) inducible with 1 or 2 extrastimuli

Overall, of the 126 inducible

patients, 5.5% were induced

with 1 premature beat, 44.5%

with 2 premature beats, and

50% with 3 premature beats.

Τhe number of inducible

patients is higher when more

aggressive protocols are used.

ECG type, syncope, QRS-f VRP <200msec have higher sensitivity and specificity than EP

Asymptomatic patients:lower sensitivity but neg predictive value 98.3%

▲▲ risk Syncope spontaneous type 1 and positive EPS HR 2.7

Circulation 2016

1,312 patients with a median follow-up of 38 months and heterogeneous stimulation protocols. 53% spontaneous type 1

incidence 0.27%/per year for (asymptomatic

individuals + drug-induced type 1) 3.22% /year (syncope and spontaneous type 1

the incremental prognostic value of abnormal PES beyond solely clinical parameters, syncope, and a spontaneous type 1 ECG pattern is small.

EP reserved for patients who are not clearly stratified by clinical features.

Why are there so many differences???

Is the debate justified???

Circardian pattern:

Eur Heart J. 1999;20:465–470. Heart Rhythm, Vol 5, No 11, November 2008J Cardiovasc Electrophysiol, Vol. pp. 1-7

SD in Brugada

Typically a polymorphic ventricular tachycardia, that evolves into VF during rest -sleep

• Monogenetic????

• Genetics involved in -30-40% 30 SCN5A loss of function a subunit sodium, 5%Ca

and K channels

• No hereditary pattern is evident in most index patients-

• Complex, locus heterogeneity, incomplete penetrance, and variable expressivity

• Changes in definitions

• Complex substrate has been documented

Europace (2011) 13, 1077–1109

SD in Brugada

Mechanism of VF in BrS multifactorial

Hormones age & sex

Dynamic and autonomic changes changes

THE ENVIRONMENT

Wilde et al J Mo Cell Cardiol 2010Conte et al J Cardiovasc Electrophysiology 2014

EP

Details of the protocol Site of pacing –Drive- cycle length extra stimuli >=200msec- time between runs -number of extras –med provocationIncrease in sensitivity looses in specificityMore specific for reentry tachycardias

No usefulness in other channelopathiesand idiopathic VFIn patients with idiopathic VF yields inconsistent inducibility (50-60%).

Wellens HJ, Schuilenburg RM, Durrer D. Electrical stimulation of the heart in patients with ventricular tachycardia. Circulation. 1972; 46: 216-226.

Reasons for diversity in results

1) Different populations in the studies

• Bias in risk estimation

• First populations identified were the high risk

• True incidence of asymptomatic pts unknown

2) EP limitations

• Different protocols in the studies

• Different ECG at the time of the study

• EP looses specificity when the protocol is aggressive

3) mechanisms of VF multifactorial that cannot be predicted by EP

J Am Coll Cardiol 2015;65:879–88

spontaneous or drug-induced Brugada type 1 with ICDs 176 ptsFU 83.8 +- 57.3 months

You cannot reassure pts of being 100% safeRisk stratification in VF has no absolute standards

Personalize

Conclusion

• SCA –ICD

• Symptoms high risk clinical -ICD

• Asymptomatic BrS pts EP does not add much

• Stratification is still a challenge –

• EP is a tool for intermediate risk patients

• Individualize

Circulation 2016;133:619-621

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