Brugada Syndrome is a rare genetic disorder characterized by heart rhythm abnormalities. Brugada syndrome if untreated can result in sudden death. The condition may be inherited in some cases. It is often characterized by sudden death and the ECG pattern is characterized by incomplete right bundle branch block and ST elevations in the anterior precordial leads.
The typical case of Brugada syndrome has been associated with alterations in the SCN5A gene. This disease is more prevalent in men when compared to women. Although Brugada syndrome is a genetic disorder, it most commonly affects men aged between 30-50 years.
Symptoms
- There are a few common symptoms of the Brugada Syndrome. They are:
- Ventricular arrhythmia
- Abnormal heart rhythm
- Fainting
- Seizures
- Breathing difficulty
- Ventricular arrhythmia
- Sudden infant death
- Cardiac arrest
However, there are even cases, wherein some patients remain asymptomatic.
Diagnosis
In this syndrome , the diagnosis is mostly based on the history of aborted sudden death with the typical electrocardiographic pattern of ST segment elevation in leads V1-V3, with or without right bundle branch block [1] (Figure 1). In some cases, however, the diagnosis is different because some individuals present with an abnormal electrocardiogram but are completely asymptomatic or there is a history of sudden death in the family and the electrocardiographic criteria are observed.
Treatment
Though the Brugada syndrome poses a serious threat to life, at present, implantation of an automatic implantable cardiac defibrillator (ICD) is the only treatment proven effective in treating these patients. However, this has proven to be very effective in preventing the sudden death of the patients. No pharmacologic therapy has been proven to reduce the occurrence of the disorder.
However, there are two cases to be considered before the implantation of the ICD to patients suffering from Brugada Syndrome. Asymptomatic patients with no family history of sudden cardiac death can be managed conservatively with close follow-up, and ICD implantation is not recommended. On the other hand, patients with Brugada syndrome and a history of cardiac arrest must be treated with an ICD. Also, when it comes to surgical treatment for Brugada Syndrome, ICD placement is the only go.
Unfortunately, so far, no drug therapy for Brugada syndrome is recommended because clinical trials have proved non-effective.
Brugada Syndrome in Children
According to a recent study for Brugada Syndrome in children, the following findings were noted. The affected age group ranged from infancy to 16 years. In children with Brugada syndrome, clinical manifestations and arrhythmic events are uncommon, but arrhythmias may be more likely during febrile episodes. Potential reasons for delayed presentation include the impact of hormones and the progressive structural degradation of myocytes .But because the data is limited, the optimal approach to the management of children with BS is not known.
