Brugada syndrome is an uncommon, inherited abnormality of theheart’s electrical system.
It can have two notable consequences even in apparently healthy young people.
Ventricular fibrillation, and sometimes ventricular tachycardia, can cause sudden cardiac arrest or death.
Portra Images / Getty Images
These arrhythmias may also lead to syncope (passing out).
Brugada Syndrome Symptoms
The most devastating problem due to Brugada syndrome is sudden death during sleep.
Brugada syndrome has been identified as the cause of mysterious sudden unexplained nocturnal sudden death syndrome, or SUNDS.
SUNDS was first described as a condition affecting young males in Southeast Asia.
But not everyone who has the abnormal gene(s) is affected in the same way.
Furthermore, a specific gene hasn’t been found for around 70% of affected families.
The flow of ions through these channels produces the heart’s electrical signal.
One of the most important channels is the sodium channel, which allows sodium to enter cardiac cells.
This alteration leads to an electrical instability that, under some circumstances, can produce ventricular fibrillation.
The only cardiac abnormality found with this syndrome is an electrical one.
The hearts of people with Brugada syndrome are structurally normal.
In addition, people with Brugada syndrome may have a form ofdysautonomiaan imbalance betweensympathetic and parasympathetictone.
Who Gets Brugada Syndrome?
Most people diagnosed with Brugada syndrome based on symptoms are young to middle-aged adults.
Brugada syndrome is seen eight to 10 times more often in men than in women.
Some estimate that 1 in 5,000 people may have Brugada syndrome.
People with Brugada syndrome who have a high risk of sudden death should be treated aggressively.
The ability of electrophysiologic testing to accurately assess that risk is far less than perfect.
In most cases of asymptomatic Brugada, EP testing is not needed.
Furthermore, genetic testing in Brugada syndrome is quite complex and often does not yield definitive answers.
It can, however, be useful in identifying affected family members.
In general,antiarrhythmic drugsshould be avoided.
A medication called quinidine and ablation therapy have both shown some success in treating Brugada syndrome.
If the risk is high (based on symptoms or electrophysiologic testing), a defibrillator should be recommended.
But implantable defibrillators are expensive andcarry their own complications.
So if the risk of sudden death is judged to be low, these devices are not currently recommended.
This is because most arrhythmias that produce sudden death in young people are more likely to occur during exertion.
Initially, guidelines regarding exercise with Brugada syndrome were quite restrictive.
However, this absolute restriction has subsequently been recognized as being too severe.
The trick is to diagnose this condition before an irreversible event occurs.
2019 Jan;140(2):e125-e151.
2016 Jul;95(30):e4214.
2015 Nov;132(22):e326-e329.
doi:CIR.0000000000000246
Daoud E.Even a pooled analysis does not handle the debate of electrophysiology testing in Brugada syndrome.Circulation.
2018 Aug;138(13):e272-e391.
doi:10.1161/CIR.0000000000000549
Brodie OT, Michowitz Y, Belhassen B.Pharmacological therapy in Brugada syndrome.Arrhythm Electrophysiol Rev.
Journal of the American College of Cardiology.
2013;10(12):1932-1963. doi: 10.1161/CIR.0000000000000665
