Ripolarizzazione precoce. Non così innocente come si pensava

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1 Brugada syndrome: tests and diagnosis. Torino, 27 ottobre 2017 Ripolarizzazione precoce. Non così innocente come si pensava Carla Giustetto Carla Giustetto Divisione di Cardiologia Divisione Università di Cardiologia Torino Ospedale S.Giovanni Battista -Università di Torino Ospedale Città della Salute e della Scienza

2 Brugada syndrome: diagnosis J point 2mm Channelopathy characterized by: Coved type ST Genetic segment mutations: elevationna + channel in 15-30% of pts; Ca ++ channel, Negative K + T channel, wave GPD1L NO structural heart disease Risk of sudden death Diagnostic ECG: typical ECG alterations (V 1 -V 2 )

3 LOOP RECORDER HIDROQUINIDINE ICD

4 Brugada Piedmont Registry 826 pts 12 diagnosed after Sudden Death total 814 pts Symptoms at presentation Asymptomatic (75%) 77 (9%) 118 (15%) asd 11 (1.4%) 1% neurally mediated Torino Asti Vercelli Orbassano Rivoli Cuneo Novara Savigliano unexplained Syncope 195 (24%)

5 A 45 years old man: traumatic syncope, which occurred after awakening at 6.30 a.m, while he was in the bathroom, with doubtful prodromes 1st ECG: sinus rhythm, normal conduction, non-significant ST-T alterations

6 Head-up tilt test (HUTT) Tilt test was negative but ST segment in V1-V2 with a type 2 Brugada pattern was recorded V1 V2

7 Pt with syncope + suspect Brugada ECG (type 2) V1 V2 Which investigations are reasonable/recommended? ECG with V1-V2 at 2 nd and 3 rd intercostal space (ICS)

8 ECG 4 th ICS ECG was recorded with V1-V2 at a higher intercostal space in this case it remained doubtful, still not diagnostic V1 2 ICS V2 2 ICS

9 Which investigations are reasonable/recommended? Pt with syncope + suspect Brugada ECG pattern (type 2) ECG with V1-V2 at 2 nd and 3 rd intercostal space type 2 Brugada ECG Drug challenge with sodium channel blockers

10 Pharmacological challenge with Na + -channel blockers was performed Basal ECG V1 Ajmaline infusion (1mg/kg in 5 min) V1 V2 V2 V3 V4 V1 - II space V1 - II space V2 - II space V2 - II space

11 Summary 45 years old man syncope of uncertain origin Drug induced type 1 Brugada ECG pattern What does literature report? What do guidelines recommend?

12 No syncope, nor type 1 Circulation 2002;105:1342 Italian Registry 200 pts Asymptomatic spontaneous type 1 Syncope + induced type 1 Syncope + spontaneous type 1 HR 6.4 for risk of cardiac arrest

13 1312 patients from 14 prospective observational studies Circulation 2016;133:

14 12-lead 24-hour Holter monitoring: intermittent spontaneous type 1 Brugada pattern V1 - II space h. 4:12 pm h. 11:44 pm h. 6:33 am h. 8:20 am h. 1:08 pm V2 - II space V1 standard V2 standard V5 V6

15 Summary 45 years old man syncope of uncertain origin drug induced type 1 Brugada ECG pattern and spontaneous type 1 documented at f-up What do guidelines recommend? What does literature report?

16 Which Brugada patients to treat: Guidelines 2015

17 Results Role of of EP-study study in in Brugada Brugada pts pts (overall (overall population) P = 0.05 Probst et al, FINGER Registry, Circulation 2010;121: 635

18 Sroubek J et al. Circulation 2016;133:

19 Annual incidence of cardiac arrest among 1312 individuals included in the analysis Sroubek J et al. Circulation 2016;133:

20 Int. J. of Cardiology2017; 241: Arrhythmic events in neurally mediated syncope versus unexplained 118 neurally-mediated vs 77 unexplained syncope Neurally mediated Unexplained 2 (0.3% person-year) f-up 62 months 6 (1.8% person-year)

21 Int. J. of Cardiology2017; 241: Role of PVS in unexplained syncope Best predictors of ventricular 7 year events mean f-upat follow-up: unexplained syncope + spontaneous type 1 + Inducibility at PVS 0 events 27% 5.2% per year

22 19 years old man, asymptomatic for syncope, no history of SD. ECG for sport eligibility: suspicious for Brugada pattern What should we do?

23 Am J Cardiol 2015; 115: Brugada Piedmont Registry 684 patients 12 Lead - 24 hour Holter 251 patients % 70% 30% Persistent type 1 Intermittent type 1 No type % spontaneous type 1 at basal ECG (group 1) % 31% 20% drug-induced type 1 (group 2) 0 group 1 group 2

24 12-lead Holter ECG: NO spontaneous type 1

25 Asymptomatic 19 years old pt evaluated for sport eligibility Basal ECG Positive ajmaline test IV space II space IV space II space

26 J Am Coll Cardiol 2010;56: Exercise test 93 Brugada pts 102 controls yes no augmentation of STsegment elevationat early recovery? 37% 63% none

27 Makimoto, J Am Coll Cardiol 2010;56:

28 pre-exercise test Peak exercise

29 Which investigations are reasonable/recommended? Asymptomatic pt + suspect Brugada ECG pattern (type 2) Negative 12-lead-Holter monitoring Type 1 Brugada ECG at exercise test programmed ventricular stimulation

30 Freedom from arrhythmic events Brugada Registry of the Piedmont region: arrhythmic events at follow-up 1 0,95 0,9 0,85 0,8 0,75 0,7 0,65 0,6 G1 vs Unexplained G2, p=0.02 syncope vs G1 vs Asympt, p=0.58 Mean follow-up of 62±48 months G2 vs Asympt, p < neurally-mediated Group1 Group2 asymptomatic neurally-mediated unexplained time (years) G G Asympt % (0.2% person-year) 2% (0.3% person-year) 9% (1.8% person-year)

31 Am J Cardiol 2015;116:98e prospective observational studies mean f-up months 3,536 asymptomatic subjects (2,820 men) 1,398 with spontaneous type 1 ECG Asymptomatic pts with spontaneous type 1 ECG exhibit an increased risk of arrhythmic events

32 Am J Cardiol 2015;116:98e103 Inducible ventricular arrhythmias at PVS were predictive of arrhythmic events

33 In summary: 1. asd ICD 2. unexplained SYNCOPE PVS + PVS - ICD loop recorder if still arrhythmic events or ICD refusal: add HQ/ablation Spontaneous type 1 ECG PVS + HQ PVS + PVS - ICD F-up YES 3. neurally mediated SYNCOPE / ASYMPTOMATIC Drug-induced type 1 12 lead Holter: Spontaneous type 1 NO F-up

34 Thank you for your attention!