Conditions related to the heart's electrical activity

We treat the following conditions related to the electrical activity of the heart, also known as channelopathies:
• Long QT syndrome
• Brugada syndrome
• Catecholaminergic polymorphic ventricular tachycardia (CPVT)

This is a group of genetic disorders in which the heart is structurally normal, but its electrical activity is abnormal.

In long QT syndrome and Brugada syndrome there is a genetic defect in the proteins that regulate the electrical signaling within the heart, making the heart vulnerable to beating dangerously fast (tachycardia). In some cases there is a significant risk of collapse and sudden death.

We provide a completely integrated care plan for both individuals and families affected by these conditions. We offer a prompt and easily accessible service for those feared to be at risk or patients who have already experienced a near-fatal abnormal heart rhythm with no obvious cause.

Signs and symptoms
Sudden death in the young related to the heart is rare, but sadly many of the sudden deaths that do occur may do so in seemingly fit and healthy individuals. However, there are two important warning signs that require further investigation. The first is sudden and unexplained fainting (syncope), particularly if this occurs during physical exertion. If you or a family member experiences such an event, you need to be referred urgently to a cardiologist. Those under 40 years of age should be seen in an inherited cardiac conditions clinic such as ours. The other major warning sign is a family history of unexplained premature sudden death related to the heart in a first- or second-degree relative.

Treatment
Once detected the above conditions can be successfully treated with either drugs or, quite commonly, the implantation of a sophisticated pacemaker known as an implantable cardiac defibrillator (ICD). This is a small battery-powered device that can detect an abnormal heart rhythm and correct it by producing a small jolt of electricity.