Approach Considerations
To date, the only treatment that has proven effective in treating ventricular tachycardia and fibrillation and preventing sudden death in patients with Brugada syndrome is implantation of an automatic implantable cardiac defibrillator (ICD). [18] Radiofrequency catheter ablation has been recently reported as an effective new treatment. [3, 4, 5, 6, 7] No pharmacologic therapy has been proved to reduce the occurrence of ventricular arrhythmias or sudden death.
Placement of Implantable Cardioverter-Defibrillator
At present, implantation of an automatic implantable cardioverter-defibrillator (ICD) is the only treatment proved effective in treating ventricular tachycardia and fibrillation and preventing sudden death [18] in patients with Brugada syndrome.
Indications for ICD implantation were published in the report of the Second Consensus Conference on Brugada syndrome. [28] For patients at the two extremes of risk stratification, the decision to implant or not to implant an ICD is relatively straightforward.
Patients with Brugada syndrome and a history of cardiac arrest must be treated with an ICD. In contrast, asymptomatic patients with no family history of sudden cardiac death can be managed conservatively with close follow-up, and ICD implantation is not recommended. Patients with intermediate clinical characteristics present the greatest challenge. For details about risk stratification and indications for ICD implantation, readers are referred to the Second Consensus Conference report. [28]
Patients with syncope or cardiac arrest and suspected or diagnosed Brugada syndrome must be hospitalized. Continuous cardiac monitoring is necessary until definitive treatment (ie, ICD placement) can be provided.
Activity
Because regular physical activity may increase vagal tone, sport may eventually enhance the propensity of athletes with Brugada syndrome to have ventricular fibrillation and sudden cardiac death at rest or during recovery after exercise. Accordingly, Pelliccia et al recommended that patients with a definite diagnosis of Brugada syndrome be restricted from participation competitive sports. [42] However, no direct evidence supported this recommendation, and it was unclear whether asymptomatic carriers of SCN5A mutations should also be so restricted.
A scientific statement published in 2015 by the American Heart Association and the American College of Cardiology on athletic competition by persons with known or suspected cardiac channelopathies included the following recommendations related to Brugada syndrome [43] :
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That a heart rhythm specialist or genetic cardiologist experienced in cardiac channelopathies conduct a thorough evaluation of an athlete in whom such a disorder has been diagnosed or is suspected
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That asymptomatic athletes who are genotype-positive/phenotype-negative for long-QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, early repolarization syndrome, short-QT syndrome, or idiopathic ventricular fibrillation be allowed to take part in all competitive sports if precautionary measures, such as the avoidance of drugs that exacerbate Brugada syndrome, are undertaken
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That in an athlete with previously symptomatic or electrocardiographically evident Brugada syndrome, early repolarization syndrome, or short-QT syndrome, participation in competitive sports be considered if precautionary measures have been taken and disease-specific treatments are being administered and if the athlete has been asymptomatic on therapy for at least 3 months
Consultations
A board-certified cardiologist who specializes in cardiac arrhythmic disorders (ie, a clinical electrophysiologist) should evaluate patients with suspected Brugada syndrome. Consultation with a genetic counselor is indicated for genetic screening and counseling of patients and their relatives.
Long-Term Monitoring
A board-certified electrophysiologist should closely follow patients with Brugada syndrome. Taking a careful history is important, as not all syncope is necessarily arrhythmic in Brugada syndrome. For example, a clear prodrome suggesting vasovagal syncope does not suggest an adverse prognosis in an otherwise asymptomatic patient with a Brugada ECG pattern.
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Schematics show the 3 types of action potentials in the right ventricle: endocardial (End), mid myocardial (M), and epicardial (Epi). A, Normal situation on V2 ECG generated by transmural voltage gradients during the depolarization and repolarization phases of the action potential. B-E, Different alterations of the epicardial action potential that produce the ECG changes observed in patients with Brugada syndrome. Adapted from Antzelevitch, 2005.
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Three types of ST-segment elevation in Brugada syndrome, as shown in the precordial leads on ECG in the same patient at different times. Left panel shows a type 1 ECG pattern with pronounced elevation of the J point (arrow), a coved-type ST segment, and an inverted T wave in V1 and V2. The middle panel illustrates a type 2 pattern with a saddleback ST-segment elevated by >1 mm. The right panel shows a type 3 pattern in which the ST segment is elevated < 1 mm. According to a consensus report (Antzelevitch, 2005), the type 1 ECG pattern is diagnostic of Brugada syndrome. Modified from Wilde, 2002. Image courtesy of Richard Nunez, MD, and EMedHome.com (http://www.emedhome.com/).
Tables
Characteristic |
Type 1 |
Type 2 |
Type 3 |
J wave amplitude |
≥2 mm |
≥2 mm |
≥2 mm |
T wave |
Negative |
Positive or biphasic |
Positive |
ST-T configuration |
Cove-type |
Saddleback |
Saddleback |
ST segment, terminal portion |
Gradually descending |
Elevated by ≥1 mm |
Elevated by < 1 mm |
