From the Guidelines
Brugada syndrome patients with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, should be treated with an ICD if meaningful survival of greater than 1 year is expected. This recommendation is based on the 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death 1. The guideline suggests that in patients with Brugada syndrome, an ICD is recommended for those with a high risk of sudden cardiac death, including those with a history of cardiac arrest or syncope.
Diagnosis and Risk Stratification
Diagnosis of Brugada syndrome involves ECG testing, and a pharmacological challenge using a sodium channel blocker can be useful for diagnosis in patients with suspected Brugada syndrome in the absence of a spontaneous type 1 Brugada electrocardiographic pattern 1. Electrophysiological study with programmed ventricular stimulation may be considered for further risk stratification in asymptomatic patients with a spontaneous type 1 Brugada electrocardiographic pattern 1.
Treatment and Management
Treatment of Brugada syndrome primarily involves implantation of an ICD for high-risk patients to prevent sudden death by delivering a shock when dangerous rhythms occur. Quinidine or catheter ablation may be recommended in patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT or in those who are not candidates for or decline an ICD 1. Patients should avoid certain medications that can trigger arrhythmias, excessive alcohol consumption, and high fevers, as these can increase the risk of sudden cardiac death 1.
Lifestyle Changes and Family Screening
Lifestyle changes, including avoidance of drugs that may induce ST-segment elevation in right precordial leads, avoidance of excessive alcohol intake and large meals, and prompt treatment of any fever with antipyretic drugs, are recommended in all patients with a diagnosis of Brugada syndrome 1. Family screening is important since the condition is hereditary, following an autosomal dominant pattern of inheritance, and genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives 1.
Key points to consider in the management of Brugada syndrome include:
- ICD implantation for high-risk patients
- Quinidine or catheter ablation for patients with recurrent ICD shocks or those who are not candidates for or decline an ICD
- Lifestyle changes to reduce the risk of sudden cardiac death
- Family screening and genetic counseling to identify relatives at risk.
From the Research
Definition and Characteristics of Brugada Syndrome
- Brugada syndrome is an inherited channelopathy with an increased risk of sudden cardiac death (SCD) due to ventricular arrhythmias (VA) and an increased incidence of supraventricular arrhythmias 2.
- It is characterized by a spontaneous or Na+-channel blocker induced "type 1" Brugada electrocardiographic (ECG) pattern typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal spaces 3.
- The syndrome can manifest as syncope or sudden cardiac death in individuals without overt structural heart disease 3.
Diagnosis and Risk Stratification
- The diagnosis of Brugada syndrome is mainly based on the presence of a spontaneous or Na+-channel blocker induced "type 1" Brugada ECG pattern 3.
- Current guidelines recommend ICD implantation only in patients with spontaneous type 1 ECG pattern and either history of aborted cardiac arrest or documented sustained ventricular tachycardia (class I) or syncope of arrhythmic origin (class IIa) because they are at high risk of recurrent arrhythmias 3.
- However, the majority of BrS patients are asymptomatic when diagnosed and have generally low risk (0.5 % annually or lower) and therefore are not indicated for ICD 3.
Treatment and Management
- The implantable cardioverter-defibrillator (ICD) is used for primary or secondary prevention in Brugada syndrome, but its use remains controversial amongst low-risk asymptomatic patients 4.
- Quinidine therapy has been demonstrated to be effective in preventing VA in Brugada syndrome, especially in patients with ICD and recurrent shocks or in asymptomatic patients with inducible ventricular fibrillation (VF) at electrophysiological study 2.
- ICD therapy is effective for primary and secondary prevention of SCD in BrS, but it is associated with high rates of inappropriate shocks and device-related complications 5.
Outcomes and Prognosis
- Appropriate shocks occur more frequently in BrS patients presenting with VT/VF, but they also occur in asymptomatic patients 4.
- Type 1 Brugada pattern and the presence of other arrhythmia are independent predictors of appropriate shock delivery 4.
- ICD therapy was an effective strategy in Brugada syndrome, treating potentially lethal arrhythmias in 17% of patients during long-term follow-up 5.