Διαχείρηση Ασυμπτωματικού ασθενούς με ΗΚΓ τύπου Brugada
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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
5
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:
10
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
29
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
32 Ευχαριστώ για την προσοχή σας
ΤΙ ΠΡΕΠΕΙ ΝΑ ΓΝΩΡΙΖΕΙ ΟΓΕΝΙΚΟΣ ΚΑΡΔΙΟΛΟΓΟΣ ΓΙΑ ΤΙΣ ΔΙΑΥΛΟΠΑΘΕΙΕΣ
ΤΙ ΠΡΕΠΕΙ ΝΑ ΓΝΩΡΙΖΕΙ ΟΓΕΝΙΚΟΣ ΚΑΡΔΙΟΛΟΓΟΣ ΓΙΑ ΤΙΣ ΔΙΑΥΛΟΠΑΘΕΙΕΣ ΣΤΕΛΙΟΣ ΠΑΡΑΣΚΕΥΑÏΔΗΣ ΔΙΕΥΘΥΝΤΗΣ ΕΣΥ Α Καρδιολογική Κλινική ΑΠΘ, Νοσοκομείο ΑΧΕΠΑ, Θεσσαλονίκη NO CONFLICT OF INTEREST Sudden Cardiac Death
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