Brugada syndrome treatment

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.Patients with aborted sudden cardiac death are at high risk for recurrence and should undergo AICD implantation, and do not require an electrophysiologic study to assess inducibility. Patients with symptoms (either syncope, seizures or nocturnal agonal respirations) should undergo implantation of a defibrillator if no other cause of their symptoms can be identified. Asymptomatic patients should undergo electrophysiologic testing, and if VT / VF can be induced, they should undergo implantation of an ICD. Asymptomatic patients who cannot be induced should followed-up closely. Patients who are asymptomatic with no family history of Brugada syndrome can be followed-up closely.

The Two Patient Groups

The 2005 consensus statement divides patients into two groups:

  • Higher risk patients with spontaneous Type I Brugada pattern
  • A less high risk cohort of patients who require infusion of a sodium channel blocker to induce a Type I Brugada pattern.

The management of these two groups of patients will be discussed separately.

Management of Patients with a Spontaneous Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:

Symptomatic Patients

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Asymptomatic Patients

  • Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
  • Patients with no family history of sudden cardiac death in whom VT VF can be induced on electrophysiologic testing.

In essence, if VT VF can be induced on electrophysiologic testing in these patients, a cardiac defibrillator should be implanted. It is unclear if the same recommendations apply to those patients who require that the electrodes be placed one to two intercostal spaces higher to demonstrate a Brugada type I electrocardiographic pattern.

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Management of Patients with a Sodium Channel Induced Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:

Symptomatic Patients

The flowchart below summarizes the recommendations of the 2005 consensus panel:

Asymptomatic Patients

  • Patients with a family history of sudden cardiac death that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.

The flowchart below summarizes the recommendations of the 2005 consensus panel.

Pharmacotherapy

  • Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in infants.[1]
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  • Pharmacological tests using a sodium-channel blocker drug should be used to evaluate suspected Brugada syndrome. Intravenous ajmaline and flecainide are the most widely used agents. The test is considered positive if a type 1 ECG pattern is identified during drug infusion.


Drugs with Potential Antiarrhythmic Effect

(Alphabetical order generic name)

Generic name Brand name® Class / Clinical use References Recommendation
Cilostazol e.g.
Pletal® 		Phosphodiesterase inhibitor Tsuchiya 2002
Abud 2006
Matsui 1999 		Class IIb
Isoproterenol
Isoprenaline 		e.g.
Isuprel® 		Beta-adrenergic receptor stimulation Miyazaki 1996
Suzuki 2000
Watanabe 2006
Ohgo 2007
Ganesan 2006 		Class I
Orciprenaline e.g.
Alotec®
Metaprel®
Novasmasol® 		Beta-adrenergic receptor stimulation Kyriazis 2009 Class IIa
Quinidine e.g.
Quinalan®
Chinidin® 		Antiarrhythmic Agent Suzuki 2000
Alings 2001
Belhassen 2004
Mizusawa 2006
Probst 2007
Ohgo 2007
Yan 1999 		Class I
  • Recommendation: Class I: convincing evidence/opinion; Class IIa: evidence/opinion less clear; Class IIb: conflicting evidence/opinion; Class III: very little evidence.

Treatment of VT Storm

  • VT storm has been successfully treated with isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.

Treatment of Coronary Ischemia

Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms

  • Fever in a Brugada syndrome patient should be treated with an antipyretic.
  • Brugada syndrome patients should avoid hot tubs, very hot baths or extremely hot climates.
  • Hypokalemia, hyperkalemia, and hypercalcemia should be treated aggressively.
  • Carbohydrate loading should be avoided.

Contraindicated medications

Brugada syndrome is considered an absolute contraindication to the use of the following medications:

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [8]

Recommendations for Brugada Syndrome

Class I
"1. An ICD is indicated for Brugada syndrome patients with previous cardiac arrest receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)"
Class IIa
"1. An ICD is reasonable for Brugada syndrome patients with spontaneous ST-segment elevation in V1, V2, or V3 who have had syncope with or without mutations demonstrated in the SCN5A gene and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)"
"2. Clinical monitoring for the development of a spontaneous ST-segment elevation pattern is reasonable for the management of patients with ST-segment elevation induced only with provocative pharmacological challenge with or without symptoms. (Level of Evidence: C)"
"3. An ICD is reasonable for Brugada syndrome patients with documented VT that has not resulted in cardiac arrest and who have reasonable expectation of survival with a good functional status for more than 1 y. (Level of Evidence: C)"
"4. Isoproterenol can be useful to treat an electrical storm in the Brugada syndrome. (Level of Evidence: C)"
Class IIb
"1. EP testing may be considered for risk stratification in asymptomatic Brugada syndrome patients with spontaneous ST elevation with or without a mutation in the SCN5A gene. (Level of Evidence: C)"
"2. Quinidine might be reasonable for the treatment of electrical storm in patients with Brugada syndrome.(Level of Evidence: C)"

References

  1. ↑ Al-Khatib, Sana M.; Stevenson, William G.; Ackerman, Michael J.; Bryant, William J.; Callans, David J.; Curtis, Anne B.; Deal, Barbara J.; Dickfeld, Timm; Field, Michael E.; Fonarow, Gregg C.; Gillis, Anne M.; Granger, Christopher B.; Hammill, Stephen C.; Hlatky, Mark A.; Joglar, José A.; Kay, G. Neal; Matlock, Daniel D.; Myerburg, Robert J.; Page, Richard L. (2018). "2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000549. ISSN 0009-7322.
  2. ↑ Belhassen B, Glick A, Viskin S (2004). "Efficacy of quinidine in high-risk patients with Brugada syndrome". Circulation. 110 (13): 1731–7. doi:10.1161/01.CIR.0000143159.30585.90. PMID 15381640.
  3. ↑ Tsuchiya, Takeshi; Ashikaga, Keiichi; Honda, Toshihiro; Arita, Makoto (2002). "Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 13 (7): 698–701. doi:10.1046/j.1540-8167.2002.00698.x. ISSN 1045-3873.
  4. ↑ Abud, Atilio; Bagattin, Daniel; Goyeneche, Raul; Becker, Carlos (2006). "Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 17 (2): 210–212. doi:10.1111/j.1540-8167.2005.00290.x. ISSN 1045-3873.
  5. ↑ Matsui, Kazunori; Kiyosue, Tatsuto; Wang, Jin-Cheng; Dohi, Kazuhiro; Arita, Makoto (1999). Cardiovascular Drugs and Therapy. 13 (2): 105–113. doi:10.1023/A:1007779908346. ISSN 0920-3206. Missing or empty |title= (help)
  6. ↑ Miyazaki, Toshihisa; Mitamura, Hideo; Miyoshi, Shunichiro; Soejima, Kyoko; Aizawa, Yoshifusa; Ogawa, Satoshi (1996). "Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome". Journal of the American College of Cardiology. 27 (5): 1061–1070. doi:10.1016/0735-1097(95)00613-3. ISSN 0735-1097.
  7. ↑ Suzuki, Hiroshi; Torigoe, Katsumi; Numata, Osamu; Yazaki, Satoshi (2000). "Infant Case with a Malignant Form of Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 11 (11): 1277–1280. doi:10.1046/j.1540-8167.2000.01277.x. ISSN 1045-3873.
  8. ↑ Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M; et al. (2006). "ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society". Circulation. 114 (10): e385–484. doi:10.1161/CIRCULATIONAHA.106.178233. PMID 16935995.

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