Medicines for Brugada syndrome

What it is

Brugada syndrome is a rare, inherited condition that affects the heart’s electrical activity and can cause the heart to beat in a dangerously abnormal way (a serious heart rhythm problem), which can, in some cases, be life-threatening. It is caused by genetic changes that affect the channels controlling the electrical signals in the heart muscle, so that the heart is prone to developing abnormal, fast, and chaotic rhythms, particularly from the lower chambers of the heart (ventricles). Because it is inherited, it can run in families. A key and important feature of Brugada syndrome is that many people with it have no symptoms and feel completely well, and the heart is structurally normal — the problem is with the heart’s electrical activity, not its structure. It is often diagnosed through a characteristic pattern seen on a heart tracing (an ECG, sometimes only apparent under certain conditions or with specific testing), for example when someone is investigated because of symptoms, a family history, or an ECG done for another reason. When Brugada syndrome does cause symptoms, they result from the abnormal heart rhythms and can include: fainting (blackouts), particularly if caused by an abnormal rhythm; palpitations (an awareness of an abnormal or fast heartbeat); and, in the most serious cases, a dangerous rhythm can cause a cardiac arrest (where the heart stops pumping effectively), which is life-threatening and can, tragically, be the first sign in some people, and is a cause of sudden death in some cases. The risk of dangerous rhythms varies between individuals, and certain things can trigger or increase the risk — notably a high temperature (fever), certain medicines, excessive alcohol, and some other factors — which is why avoiding these triggers is an important part of management. Brugada syndrome is managed by specialist heart (cardiology) teams, with an emphasis on assessing the individual’s risk, avoiding triggers, treating fevers promptly, and, for those at higher risk, an implantable device (ICD) that can treat a dangerous rhythm; family members are also assessed, as the condition is inherited. The key messages are that Brugada syndrome is a rare inherited condition affecting the heart’s electrical activity that can cause dangerous heart rhythms, that many people have no symptoms, and that diagnosis, avoiding triggers, and, for some, an implanted device reduce the risk.

How it is treated

Brugada syndrome is managed by specialist heart teams, focusing on assessing the individual’s risk, avoiding triggers (including promptly treating fever), and, for those at higher risk, an implantable device to treat dangerous rhythms, along with assessing family members. Diagnosis is made by cardiologists, based on the characteristic ECG pattern (which may need specific testing to reveal, as it is not always present on a standard ECG), along with the person’s history, symptoms, and family history, and, where relevant, genetic testing; because it is inherited, family members are often assessed too. A central part of management is assessing the individual’s level of risk of dangerous heart rhythms, as this varies — factors such as whether the person has had symptoms (particularly fainting due to an abnormal rhythm, or a cardiac arrest), the ECG findings, and other features help assess risk and guide management. For everyone with Brugada syndrome, avoiding triggers that can provoke dangerous rhythms is important, and includes: treating a high temperature (fever) promptly (as fever is an important trigger), for example with fever-reducing measures and medicines, and seeking advice when unwell with a fever; avoiding certain medicines that can be risky in Brugada syndrome (people are given a list of medicines to avoid, and should check with a doctor or pharmacist, and mention the condition when prescribed any medicine); avoiding excessive alcohol; and being aware of other relevant triggers. For people assessed to be at higher risk (particularly those who have had a cardiac arrest or fainting due to an abnormal rhythm), the main treatment to protect against sudden death is an implantable cardioverter defibrillator (ICD) — a small device implanted under the skin that continuously monitors the heart and can deliver a treatment (a shock) to restore a normal rhythm if a dangerous rhythm occurs; this can be life-saving. Other treatments (such as certain medicines, or, in some cases, other procedures) may be used in particular situations, guided by the specialist team. For people at lower risk without symptoms, the emphasis is on avoiding triggers and monitoring, with an ICD generally reserved for higher-risk individuals, as it has its own considerations. Because the condition is inherited, screening and assessment of close family members (with ECGs and, where relevant, genetic testing) are important, so that relatives who also have the condition can be identified and managed, and genetic counselling is offered. Anyone with Brugada syndrome should seek assessment for symptoms such as fainting or palpitations, and it is important that those around them know what to do in an emergency (including calling emergency services and starting CPR if someone collapses and is not breathing normally). The reassuring messages are that, although Brugada syndrome can cause dangerous heart rhythms, many people have no symptoms, that risk can be assessed, that avoiding triggers (especially treating fever promptly and avoiding certain medicines) reduces the risk, and that, for those at higher risk, an implantable device provides protection; so specialist assessment, avoiding triggers, and, where needed, an ICD, along with assessing family members, are the keys to managing Brugada syndrome.