What is the management for a patient with Brugada pattern type 1 (Brugada syndrome)?

From the Guidelines

The management of Brugada syndrome type 1 should focus on preventing life-threatening arrhythmias and sudden cardiac death, with the cornerstone of treatment being implantation of an implantable cardioverter-defibrillator (ICD) for high-risk patients, particularly those with previous cardiac arrest, documented ventricular arrhythmias, or syncope, as recommended by the 2017 AHA/ACC/HRS guideline 1.

Key Recommendations

• ICD implantation is recommended for patients with Brugada syndrome and a history of cardiac arrest, sustained ventricular arrhythmias, or syncope, as it has been shown to reduce mortality in symptomatic patients 1.
• Patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern may be considered for an electrophysiological study with programmed ventricular stimulation for further risk stratification 1.
• Quinidine or catheter ablation may be considered for patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, or for those who are not candidates for or decline an ICD 1.
• Lifestyle modifications, such as avoiding excessive alcohol, maintaining electrolyte balance, and avoiding extreme physical exertion, are also recommended for patients with Brugada syndrome 1.

Risk Stratification

• Risk stratification is essential in managing Brugada syndrome, as not all patients require an ICD 1.
• Patients with a spontaneous type 1 Brugada electrocardiographic pattern and a history of syncope or cardiac arrest are at the highest risk for potentially lethal ventricular arrhythmias 1.

Additional Considerations

• Patients with Brugada syndrome should avoid medications that can exacerbate the condition, including certain antiarrhythmics, psychotropics, and anesthetics 1.
• Fever should be promptly treated with antipyretics, as it can trigger arrhythmias by affecting sodium channel function 1.
• First-degree relatives of patients with Brugada syndrome should undergo screening with ECG and possibly genetic testing, as the condition has an autosomal dominant inheritance pattern with variable penetrance 1.

From the Research

Management of Brugada Pattern Type 1

The management of Brugada syndrome (BrS) with a type 1 electrocardiogram (ECG) pattern involves several strategies to prevent sudden cardiac death (SCD).

• Implantable cardioverter-defibrillator (ICD) implantation is recommended for patients with a history of resuscitated cardiac arrest or syncope of presumed cardiac origin 2.
• The European Society of Cardiology (ESC) guidelines suggest ICD implantation for patients with a Brugada type 1 ECG pattern and a history of resuscitated cardiac arrest (class I recommendation) or syncope of presumed cardiac origin (class IIa recommendation) 2.
• However, the use of ICDs in asymptomatic patients with BrS is still controversial, and the decision to implant an ICD should be made on a case-by-case basis, considering the individual patient's risk factors and clinical presentation 3, 4.

Risk Stratification

Risk stratification is essential in managing patients with BrS, as it helps identify those at high risk of SCD who may benefit from ICD implantation.

• Recognized risk factors for SCD in BrS include spontaneous type 1 ECG pattern, syncope of presumed arrhythmic origin, and a family history of SCD 2.
• A multi-parametric approach that considers the contemporary presence of multiple risk factors may be useful in identifying patients at high risk of SCD 2.
• The use of electrophysiological studies (EPS) to guide ICD implantation is still controversial, and its prognostic value is not well established 2, 3.

Alternative Therapies

Alternative therapies, such as catheter ablation, may be considered for patients with BrS who are at high risk of SCD or have experienced ventricular arrhythmias.

• Epicardial radiofrequency substrate ablation of the right ventricular outflow tract (RVOT) has emerged as a promising tool for the management of BrS 5.
• The use of sodium channel blockers (SCB) may guide epicardial ablation of abnormal potential areas in patients with BrS 5.

Complications and Considerations

ICD implantation in patients with BrS is associated with a significant risk of complications, including inappropriate shocks and device-related issues.

• The complication rate for ICD implantation in patients with BrS can be as high as 28%, with inappropriate shocks occurring in up to 20% of patients 3.
• The use of subcutaneous ICDs (S-ICDs) may be considered as an alternative to transvenous ICDs, but careful patient selection and screening are essential to minimize complications 6.