Brugada syndrome treatment

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

Medical Therapy

Treatment

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 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 Ito channels. 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 receive an ICD
• 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.

Lithium Treatment and Brugada Syndrome

Administration of Lithium can result in EKG manifestations of the Brugada syndrome. . Syncope and sudden cardiac death have been observed in these patients. The putative role of lithium has been suggested in so far as withdrawal of lithium results in either 1) normalization of the ECG or 2) conversion of the Brugada pattern to type 2 or 3. The appearance of Brugada type EKG patterns does not require toxic lithium levels.

Sodium Challenge

• Drugs that can be used
  • Ajmaline 1 mg/kg/5 min IV
  • Flecainide 2 mg/kg/10 min IV or 400 mg PO
  • Procainamide 10 mg/kg/10 min IV
  • Pilsicainide 1 mg/kg/10 min IV
• The sodium challenge should be terminated when

1. Diagnostic Type 1 ST-segment elevation or Brugada ECG, develops
2. ST segment in Type 2 increases by ≥2 mm
3. Premature ventricular beats or other arrhythmias develop
4. QRS widens to ≥130% of baseline

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)

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)"