Brugada syndrome, also known as sudden unexplained nocturnal death syndrome (SUNDS), is a rare heart disease characterized by irregular heartbeat. Other symptoms may include fainting, seizures, breathing problems, nightmares, disrupted sleep, and sudden death. These problems often occur when a person is either sleeping or at rest. There are four types of Brugada syndrome.
Brugada syndrome most often affects people in their thirties. However, symptoms can appear at any age. The average age of sudden death among people with Brugada syndrome is about 40 for all types of the disease.
Brugada syndrome can be classified as type 1, type 2, type 3, or type 4. Type 1, the most studied type, is sometimes caused by mutations or defects in the SCN5A gene. The SCN5A gene provides instructions for making the cardiac sodium channel, which is essential to the normal electrical functioning and rhythm of the heart. Only about one-third of people with Brugada syndrome have been identified as having a mutated copy of this gene. Most SCN5A mutations cause arrhythmias that produce channels that continue to operate late in the cardiac cycle when normal channels are silent; such late activity creates an environment for chaotic rhythms and sudden death. Some researchers believe that some cases of sudden infant death syndrome (SIDS) are also caused by a mutation in the SCN5A gene. In those individuals without a mutated SCN5A gene, the cause of type 1 Brugada syndrome is often unknown. In some cases, a nongenetic form of Brugada syndrome may be caused by certain drugs. Drugs used to treat some forms of arrhythmia (irregular heart beat), angina (chest pain), high blood pressure, and depression and other mental illnesses can cause an altered heart rhythm.
When Brugada syndrome is inherited, or passed down among family members, it follows an autosomal dominant pattern of inheritance. This means that only one copy of the defective gene is needed for the disease to occur.
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