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September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e330

CLINICAL STATEMENTS AND GUIDELINES

7.9.1.3 Brugada Syndrome

Recommendations for Brugada Syndrome References that support the recommendations are summarized in Online Data Supplement 42 and Systematic Review Report.

COR LOE Recommendations

I B-NR 1. In asymptomatic patients with only inducible type 1 Brugada electrocardiographic pattern, observation without therapy is recommended.S7.9.1.3-1–S7.9.1.3-5

I B-NR 2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected.S7.9.1.3-4,S7.9.1.3-6

I B-NR 3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended.S7.9.1.3-7–S7.9.1.3-11

I B-NR 4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended.S7.9.1.3-7,S7.9.1.3-9–S7.9.1.3-11

IIa B-NR 5. In patients with suspected Brugada syndrome in the absence of a spontaneous type 1 Brugada electrocardiographic pattern, a pharmacological challenge using a sodium channel blocker can be useful for diagnosis.S7.9.1.3-12–S7.9.1.3-14 IIb B-NRSR 6. In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.S7.9.1.3-1,S7.9.1.3-6,S7.9.1.3-13,S7.9.1.3-15–S7.9.1.3-17

IIb C-EO 7. In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.S7.9.1.3-18–S7.9.1.3-20 SR indicated systematic review.

Synopsis

Refer to the “Systematic Review for the 2017 ACC/ AHA/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death” for the complete systematic evidence review for additional data and analyses.S7.9.1.3-15 The results from the question “For asymptomatic patients with Brugada syndrome, what is the association between an abnormal EP study and SCD and other arrhythmia endpoints? (Part 1)” and the writing committee’s review of the totality of the literature were used to frame decision-making. Recommendations that are based on a body of evidence that includes the system-

Figure 13. Exercise-induced polymorphic VT in catecholaminergic polymorphic ventricular tachycardia.

Recommendations for Brugada Syndrome (Continued)

COR LOE Recommendations

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