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 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

• Patients with aborted sudden cardiac death
• Patients with syncope, seizures or nocturnal agonal respirations who have no other identifiable cause for their symptoms

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

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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

• Patients with aborted sudden cardiac death
• Patients with syncope, seizures or nocturnal agonal respirations who have no other identifiable cause for their symptoms

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. Quinidine reduces the number of VF episodes and corrects spontaneous ECG changes, possibly via inhibiting Ito channels. No drug has demonstrated long term efficacy in the prevention of sudden cardiac death.

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

Patients with risk factors for coronary artery disease may require an angiogram before ICD implantation.

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.

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)"