Διαχείρηση Ασυμπτωματικού ασθενούς με ΗΚΓ τύπου Brugada

Transcription

1 Διαχείρηση Ασυμπτωματικού ασθενούς με ΗΚΓ τύπου Brugada Άννα Κωστοπούλου Επιμελήτρια Α Ωνάσειο Καρδιοχειρουργικό Κέντρο Τμήμα Ηλεκτροφυσιολογίας και Βηματοδότησης

2 BrS: Diagnosis 5:10000 First described in 1992 by Brugada brothers genetic basis established in 1998 coved elevation incomplete RBBB in right leads neg T which causes SD manifests in adults mainly men y (*8-10) Possibly 20% of SD in pts w normal hearts 4.6% SCD as the first symptom

3 BrS: Diagnosis Spontaneous Type I BrS ECG Type I BrS ECG after provocative test + 1: documented VF/VT syncope probably arrhythmic F History of SCD at 45 years old with negative autopsy coved-type in family members nocturnal agonal respiration EP Inducibility of VT/VF with 1 or 2 extra Marked variation triggered by vagal tone, fever, alcohol large meal and medication Antzelevich et al. J-Wave syndromes expert consensus conference report. Europace. 2017

4 Differential diagnosis- conditions that can mimic brugada -ECG phenocopy Antzelevich et al. J-Wave syndromes expert consensus conference report. Europace. 2016

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6 Arrhythmic events in asymptomatic BrS patients mean age 40y: 0.5% to 1.2% annual incidence 12% malignant ventricular arrhythmia rate at 10 y Fever induced type has intermediate risk true incidence unknown asymptomatics may be never diagnosed Delice et al Europace 2017

7 Risk stratification

8 EP& Genetics Letsas et al Jafib 2016

9 EPS Circulation. 2003;108:

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11 308 consecutive individuals (247 men, 80%; median age 44 years (18-72) EPS at enrollment, FU every 6 months. Median follow-up of 34 months J Am Coll Cardiol 2012;59:37 45

12 (A) entire PRELUDE cohort B) inducible with 1 or 2 extrastimuli

13 EP has been questioned Typical ventricular arrhythmia in Brugada syndrome is a polymorphic ventricular tachycardia, that can evolve into ventricular fibrillation Mechanism: phase 2 reentry Closely coupled extra systoles from RVOT trigger VT/VF- Ca K mutations (Repolarization theory) Both Multifactorial substrate cardiomyopathy- Monomorphic ventricular tachycardia is rarely seen. Atrial fibrillation occurs more frequently in patients with the Brugada ECG pattern than in the general population. reentry slow conduction in RV- Na mutations (Depolarization theory) Wilde et al J Mo Cell Cardiol 2010

14 J Am Coll Cardiol 2015;65: spontaneous or druginduced Brugada type 1 electrocardiographic with ICDs 176 pts FU months

15 Circulation 2016 risk Syncope spontaneous type 1 and positive EPS

16 Gene guided indications- risk stratification controversial 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 multifactorial background Europace (2011) 13,

17 Children Problems - higher risk of complications Indications in children challenging no universal consensus individualise Probst et al Circulation. 2007;115: Conte et al J Am Coll Cardiol 2014;63: Gonzales et al. Cardiol Young Aug;26(6):

18 Brugada elderly years Lower risk of SD Conte et al J Cardiovasc Electrophysiology 2014

19 Treatment

20 ACC/AHA/ HRS 2017

21 aggressive protocol 38% side effects 60% compliance 90% efficacy in preventing VF during EP

22 Quinidine 1912 Wenckebach-the opium of the heart normalizes the ECG pattern in patients with Brugada syndrome but may also do the oppposite AAA IA, antimuscarinic, a blocker &anti malaria effect blocks the calcium-independent transient outward potassium current (Ito) and Na+ Side effects vomiting diarrhea proarrhythmia- myasthenia thrombocytopenia-cinchonism psychosis TdP QTc prolongation No control group Circ Res Aug;73(2): Circulation. 2004;110: HeartRhythm Case Rep Nov; 2(6):

23 Haïssaguerre et al first ablated VF- purkinje triggering PVCs in RVOT Nademanne 9 pts epicardial ablation prolonged fragmented ventricular potentials Haissaguerre et al Circulation. 2003; 108: Morita et al Heart Rhythm. 2009; 6: Nademanee et al Circulation. 2011; 123: symptomatic pts, dissapear of ECG pattern and no events except 2 and non inducibility

24 Lets go to a case

25 Case Asymptomatic 42y old male amateur pilot pt examined for insurance screening & referred for further evaluation No episodes of syncope or sustained tachycardia No use of drugs reported Family history negative for SD Normal labs & cardiac echo

26 Stress test recovery vagal reaction w/o sync

27 Holter recording No VEs

28 Questions asymptomatic Type 1 BrS 1) Do Nothing -no other tests required 2) Do Nothing FU regularly 3) Do an EP study consider implanting an ICD if positive 4) Implant an ICD directly 5) Quinidine 6) Epicardial ablation

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30 discussed about an ICD w pt

31 Conclusion Asymptomatic BrS pts have a risk for SD Exact risk is debatable Stratification is still a challenge EP is a tool Future may have a completely different prospective Personalize & discuss w pt

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