Brugada syndrome
Heart conduction disease / From Wikipedia, the free encyclopedia
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Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy.[2] It increases the risk of abnormal heart rhythms and sudden cardiac death.[2] Those affected may have episodes of syncope.[2] The abnormal heart rhythms seen in those with Brugada syndrome often occur at rest.[1][5] They may be triggered by a fever.[1][5]
Brugada syndrome | |
---|---|
Other names | Sudden unexplained nocturnal death syndrome, bangungut, pokkuri death syndrome[1] |
Typical type 1 ECG changes seen in Brugada syndrome | |
Specialty | Cardiology |
Symptoms | Passing out, sudden cardiac death[2] |
Usual onset | Adulthood[2] |
Causes | Genetics, certain medication[2] |
Risk factors | Family history, Asian descent, male[1][2] |
Diagnostic method | Electrocardiogram (ECG), genetic testing[2][3] |
Differential diagnosis | Romano-Ward syndrome, arrhythmogenic cardiomyopathy, Duchenne muscular dystrophy[3] |
Treatment | Watchful waiting, implantable cardioverter defibrillator (ICD)[3][4] |
Frequency | 1 per 2000[1] |
Deaths | 8% of sudden cardiac death[2] |
About a quarter of those with Brugada syndrome have a family member who also has the condition.[2] Some cases may be due to a new genetic mutation or certain medications.[1] The most commonly involved gene is SCN5A which encodes the cardiac sodium channel.[6] Diagnosis is typically by electrocardiogram (ECG), however, the abnormalities may not be consistently present.[2] Medications such as ajmaline may be used to reveal the ECG changes.[2] Similar ECG patterns may be seen in certain electrolyte disturbances[7] or when the blood supply to the heart has been reduced.[8]
There is no cure for Brugada syndrome.[3] Those at higher risk of sudden cardiac death may be treated using an implantable cardioverter defibrillator (ICD).[4] In those without symptoms the risk of death is much lower, and how to treat this group is less clear.[3][9] Isoproterenol may be used in the short term for those who have frequent life-threatening abnormal heart rhythms, while quinidine may be used longer term.[3][10] Testing people's family members may be recommended.[3]
The condition affects between 1 and 30 per 10,000 people.[2] It is more common in males than females and in those of Asian descent.[1][2] The onset of symptoms is usually in adulthood.[2] It was firstly described by Andrea Nava and Bortolo Martini in Padova in 1989[11] but it is named after the Spanish cardiologists Pedro and Josep Brugada who described the condition in 1992.[3][12] Chen first described the genetic abnormality of SCN5A channels.[13]