Διαχείρηση Ασυμπτωματικού ασθενούς με ΗΚΓ τύπου Brugada Άννα Κωστοπούλου Επιμελήτρια Α Ωνάσειο Καρδιοχειρουργικό Κέντρο Τμήμα Ηλεκτροφυσιολογίας και Βηματοδότησης
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 41+-15y (*8-10) Possibly 20% of SD in pts w normal hearts 4.6% SCD as the first symptom
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
Differential diagnosis- conditions that can mimic brugada -ECG phenocopy Antzelevich et al. J-Wave syndromes expert consensus conference report. Europace. 2016
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
Risk stratification
EP& Genetics Letsas et al Jafib 2016
EPS Circulation. 2003;108:3092-3096
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
(A) entire PRELUDE cohort B) inducible with 1 or 2 extrastimuli
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
J Am Coll Cardiol 2015;65:879 88 spontaneous or druginduced Brugada type 1 electrocardiographic with ICDs 176 pts FU 83.8 +- 57.3 months
Circulation 2016 risk Syncope spontaneous type 1 and positive EPS
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, 1077 1109
Children Problems - higher risk of complications Indications in children challenging no universal consensus individualise Probst et al Circulation. 2007;115:2042-2048 Conte et al J Am Coll Cardiol 2014;63:2272 9 Gonzales et al. Cardiol Young. 2016 Aug;26(6):1044-55
Brugada elderly 67+-6 years Lower risk of SD Conte et al J Cardiovasc Electrophysiology 2014
Treatment
ACC/AHA/ HRS 2017
aggressive protocol 38% side effects 60% compliance 90% efficacy in preventing VF during EP
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. 1993 Aug;73(2):351-9. Circulation. 2004;110:1731-1737 HeartRhythm Case Rep. 2016 Nov; 2(6): 486 490
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: 925 928 Morita et al Heart Rhythm. 2009; 6: 665 671 Nademanee et al Circulation. 2011; 123: 1270 1279 135 symptomatic pts, dissapear of ECG pattern and no events except 2 and non inducibility
Lets go to a case
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
Stress test recovery vagal reaction w/o sync
Holter recording No VEs
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
discussed about an ICD w pt
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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