Brugada Syndrome Treatment & Management

  • Author: Jose M Dizon, MD; Chief Editor: Jeffrey N Rottman, MD   more...
 
Updated: Jan 9, 2012
 

Approach Considerations

At present, implantation of an automatic implantable cardiac defibrillator (ICD) is the only treatment proven effective in treating ventricular tachycardia and fibrillation and preventing sudden death in patients with Brugada syndrome. No pharmacologic therapy has been proven to reduce the occurrence of ventricular arrhythmias or sudden death.

Indications for ICD implantation were published in the report of the Second Consensus Conference on Brugada syndrome.[19] For patients at the 2 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.[19]

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Activity Restriction

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. Therefore, Pelliccia et al recommend that patients with a definite diagnosis of Brugada syndrome should be restricted from competitive sports.[33]

However, no direct evidence supports this recommendation. It remains unclear whether asymptomatic carriers of SCN5A mutations should also be restricted from participation in sports.

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Management of Concomitant Syncope or Cardiac Arrest

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.

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Deterrence/Prevention of Complications

When indicated, use of an ICD may prevent sudden cardiac death.[10] The patient's relatives and coworkers should be educated about Brugada syndrome and the basics of cardiopulmonary resuscitation (CPR). Genetic counseling is indicated if desired by the patient and his or her family.

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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.

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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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Contributor Information and Disclosures
Author

Jose M Dizon, MD  Associate Professor of Medicine and Surgery, Clinical Electrophysiology Laboratory, Division of Cardiology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Medicine, New York-Presbyterian Hospital, Columbia University Medical Center

Jose M Dizon, MD is a member of the following medical societies: American College of Cardiology and Heart Rhythm Society

Disclosure: Nothing to disclose.

Coauthor(s)

Tamim M Nazif, MD  Fellow, Division of Cardiology, Columbia University College of Physicians and Surgeons

Tamim M Nazif, MD, is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Specialty Editor Board

Justin D Pearlman, MD, ME, PhD, FACC, MA  Chief, Division of Cardiology, Director of Cardiology Consultative Service, Director of Cardiology Clinic Service, Director of Cardiology Non-Invasive Laboratory, Director of Cardiology Quality Program KMC, Dartmouth-Hitchcock Medical Center, Dartmouth Medical School

Justin D Pearlman, MD, ME, PhD, FACC, MA is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Federation for Medical Research, International Society for Magnetic Resonance in Medicine, and Radiological Society of North America

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald J Oudiz, MD, FACP, FACC, FCCP  Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society

Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; LungRx Clinical Trials + honoraria; Bayer Grant/research funds Consulting; Medtronic Consulting fee Consulting; Novartis Consulting fee Consulting

Chief Editor

Jeffrey N Rottman, MD  Professor of Medicine and Pharmacology, Vanderbilt University School of Medicine; Chief, Department of Cardiology, Nashville Veterans Affairs Medical Center

Jeffrey N Rottman, MD is a member of the following medical societies: American Heart Association and North American Society of Pacing and Electrophysiology (NASPE)

Disclosure: Nothing to disclose.

References

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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.
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.
Table. ECG Patterns in Brugada Syndrome
Characteristic Type 1 Type 2 Type 3
J wave amplitude≥2 mm≥2 mm≥2 mm
T waveNegativePositive or biphasicPositive
ST-T configurationCover-typeSaddlebackSaddleback
ST segment, terminal portionGradually descendingElevated by ≥1 mmElevated by < 1 mm
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