Brugada syndrome, is an inherited but rare disorder related to cardiovascular system, where the electrical system of heart is affected by various disturbances. It has an autosomal dominant inheritance with an adult onset around 40 years. The main symptom of this includes, irregular heart beat followed by breathlessness, black outs and loss of consciousness which can even lead to sudden death in cases with no timely treatments. Unfortunately there can be patients who may face sudden death without any preceding symptoms Severity of the condition varies from person to person and known to be aggrevated often by fever and sodium blocking drugs. The most specific presentation of the condition is, sudden unexpected nocturnal death syndrome which is mostly prevalent in Southeast Asia where young individuals die during sleep with no apparent reason. Sudden infant death syndrome which is another specific presentation occurs during the first year of life of a baby which will cause sudden death without any identifiable cause.
HOW IS BRUGADA SYNDROME DIAGNOSED?
Diagnosis of Brugada syndrome is made by a cardiologist who will get a proper personal and family history of the patients along with a thorough physical evaluation. A family history of a sudden cardiac death is highly significant. Also, a specialized test known as electrocardiogram ( ECG of EKG) is done to identify the abnormal electrical impulses of the heart. Cardiologist may also use drugs like sodium channel blockers to stimulate the characeristic abnormalities on EKG. Molecular genetic DNA test is also used to confirm the diagnosis eventhough only 30-35% of affected patients have an revealable mutated gene. An electrophysiologic test will also be used to identify the risk of any sudden cardiac arrest.
HOW IS BRUGADA SYNDROME TREATED?
Eventhough there is no cure for Berguda syndrome, implantable cardioverter defibrillator (ICD) is used in high risk patients. This device automatically identifies the abnormal electrical impulses of the heart and correct them, replacing a normal heart beat. Isoproterenol, is a drug which is used to respond to electrical storms. Genetic couselling is also provided for affected individuals and their families. Other management options include, symptomatic and supportive therapies.
DID YOU KNOW?
The prevalence of Brugada ECG is more in Asia than in the US and Europe.