Brugada syndrome

Brugada syndrome (BrS) (also known as sudden unexplained [or unexpected] nocturnal death syndrome and as idiopathic ventricular fibrillation) should be suspected in any patient with a past history of any one or more of unexplained cardiac arrest, ventricular arrhythmia (e.g., documented polymorphic ventricular tachycardia or ventricular fibrillation) of unknown cause, or cardiogenic syncope. Be aware that patients may also present asymptomatically with incidental findings of Brugada pattern on ECG, or through family screening.

Diagnosis of BrS is considered probable or definite if spontaneous type 1 Brugada pattern is recorded on ECG from the 2nd to 4th intercostal spaces. Induced type 1 Brugada pattern (e.g., by fever, medications such as sodium-channel blockers, or by psychotropic drugs, alcohol, or illicit drugs) or type 2 or 3 Brugada pattern on the ECG is not diagnostic, and requires further investigation. However, diagnosis of BrS should be made by a specialist, and requires synthesis of ECG findings and features of the history (which should include relevant family history) and physical exam.

Treatment consists of conservative management and primary and secondary prevention of serious arrhythmic events. This aims to prevent serious arrhythmic events, while avoiding potential complications of any treatments. Nonconservative treatments include implantable cardioverter defibrillator (ICD), pharmacologic therapy, and radiofrequency catheter ablation.

​Brugada syndrome (BrS) (also known as sudden unexplained [or unexpected] nocturnal death syndrome and as idiopathic ventricular fibrillation) is an inherited channelopathy characterized by a typical pattern of ST-segment elevation in the precordial leads V1-V3, and is associated with increased risk of serious arrhythmic events such as ventricular arrhythmias and cardiac arrest.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com ​ BrS is typically first diagnosed in young- to middle-aged patients and is rare in children.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [2]Oe H, Takagi M, Tanaka A, et al. Prevalence and clinical course of the juveniles with Brugada-type ECG in Japanese population. Pacing Clin Electrophysiol. 2005;28(6):549-54. http://www.ncbi.nlm.nih.gov/pubmed/15955188?tool=bestpractice.com [3]​Yamakawa Y, Ishikawa T, Uchino K, et al. Prevalence of right bundle-branch block and right precordial ST-segment elevation (Brugada-type electrocardiogram) in Japanese children. Circ J. 2004;68(4):275-9. http://www.ncbi.nlm.nih.gov/pubmed/15056820?tool=bestpractice.com

The diagnosis and management of BrS in children is not covered in this topic.