Brugada syndrome treatment: Difference between revisions

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==Overview==
==Overview==
The cause of death in Brugada syndrome is [[ventricular fibrillation]].The episodes of syncope (fainting) and sudden death (aborted or not) are caused by fast polymorphic ventricular tachycardias or ventricular fibrillation. These arrhythmias appear with no warning.  While there is no exact treatment modality that reliably and totally prevents ventricular fibrillation from occurring in this syndrome, treatment lies in termination of this lethal [[arrhythmia]] before it causes death. This is done via implantation of an [[implantable cardioverter-defibrillator]] (ICD), which continuously monitors the heart rhythm and will [[defibrillation|defibrillate]] an individual if ventricular fibrillation is noted. Some recently performed studies had evaluated the role of quinidine, a Class Ia antiarrythmic drug, for decreasing VF episodes occurring in this syndrome. Quinidine was found to decrease number of VF episodes and correcting spontaneous ECG changes, possibly via inhibiting I</sup>to channels.<ref name="pmid15381640">{{cite journal |author=Belhassen B, Glick A, Viskin S |title=Efficacy of quinidine in high-risk patients with Brugada syndrome |journal=Circulation |volume=110 |issue=13 |pages=1731–7 |year=2004 |pmid=15381640 |doi=10.1161/01.CIR.0000143159.30585.90}}</ref> Those with risk factors for [[coronary artery disease]] may require an angiogram before ICD implantation.
Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.
 
Some recently performed studies had evaluated the role of quinidine, a Class Ia antiarrythmic drug, for decreasing VF episodes occurring in this syndrome. Quinidine was found to decrease number of VF episodes and correcting spontaneous ECG changes, possibly via inhibiting I</sup>to channels.<ref name="pmid15381640">{{cite journal |author=Belhassen B, Glick A, Viskin S |title=Efficacy of quinidine in high-risk patients with Brugada syndrome |journal=Circulation |volume=110 |issue=13 |pages=1731–7 |year=2004 |pmid=15381640 |doi=10.1161/01.CIR.0000143159.30585.90}}</ref> Those with risk factors for [[coronary artery disease]] may require an angiogram before ICD implantation.


* Aborted sudden death are at high risk for recurrence and should undergo AICD implantation. Patients who are symptomatic with unexplained [[syncope], [[ventricular tachycardia]] or aborted [[sudden cardiac death]] may have a symptom recurrence risk of 2% to 10% per year. In these patients an [[AICD]] implant is advisable.
* Aborted sudden death are at high risk for recurrence and should undergo AICD implantation. Patients who are symptomatic with unexplained [[syncope], [[ventricular tachycardia]] or aborted [[sudden cardiac death]] may have a symptom recurrence risk of 2% to 10% per year. In these patients an [[AICD]] implant is advisable.

Revision as of 21:46, 14 October 2012

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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.

Some recently performed studies had evaluated the role of quinidine, a Class Ia antiarrythmic drug, for decreasing VF episodes occurring in this syndrome. Quinidine was found to decrease number of VF episodes and correcting spontaneous ECG changes, possibly via inhibiting Ito channels.[1] Those with risk factors for coronary artery disease may require an angiogram before ICD implantation.

  • Aborted sudden death are at high risk for recurrence and should undergo AICD implantation. Patients who are symptomatic with unexplained [[syncope], ventricular tachycardia or aborted sudden cardiac death may have a symptom recurrence risk of 2% to 10% per year. In these patients an AICD implant is advisable.
  • VT storm has been successfully treated with Isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.
  • Asymptomatic patients require risk stratification and clinical judegement to help guide therapy
  • Quinidine (class IA sodium channel blocker) blocks the Ito current and is proven to suppress spontaneous VF
  • Cilostazol (phosphodiesterase III inhibitor that increases inward L type calcium channel current and reported to suppress spontaneous VF
  • Bepridil suppress spontaneous VF probably through blocking Ito current
  • Medical therapy alone with the above agents is currently not evaluated in randomized trials and should not be used as loan therapy.

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.

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) [2]

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. 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.
  2. 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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