Is there an overlap between Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy/dysplasia?
The Brugada syndrome is a congenital syndrome displaying an autosomal dominant mode of transmission in patients with a structurally normal heart. The disease has been linked to mutations in SCN5A , a gene located on the short arm of chromosome 3 (p21-24) that encodes for the alpha subunit of the sodium channel. The syndrome is characterized by a dynamic ST-segment elevation (accentuated J wave) in leads V 1 to V 3 of the ECG followed by negative T wave. Right bundle-branch block of varying degrees is observed in some patients. The syndrome is associated with syncope and a relatively high incidence of sudden cardiac death secondary to the development of polymorphic ventricular tachycardia that may degenerate into ventricular fibrillation. An acquired form of the Brugada syndrome is also recognized, caused by a wide variety of drugs and conditions that alter the balance of currents active during the early phases of the action potential. Among patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia, there is a subpopulation with a clinical and electrocardiographic pattern similar to that of the Brugada syndrome. These cases of arrhythmogenic right ventricular cardiomyopathy/dysplasia are thought to represent an early or concealed form of the disease. This review examines the overlap between these 2 syndromes.
Pérez Riera
Andrés Ricardo
AR
Electro-Vectorcardiographic Section, Cardiology Division, ABC's Medical School, ABC Foundation, Santo André, São Paulo, Brazil. riera@uol.com.br
Antzelevitch
Charles
C
Schapacknik
Edgardo
E
Dubner
Sergio
S
Ferreira
Celso
C
D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000503:physiopathology; D002037:Bundle-Branch Block / Q000175:diagnosis* / Q000503:physiopathology; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004562:Electrocardiography; D006801:Humans; D013575:Syncope / Q000503:physiopathology; D013577:Syndrome; D017180:Tachycardia, Ventricular / Q000503:physiopathology