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Catecholaminergic Polymorphic Ventricular Tachycardia

The Inherited Arrhythmia Clinic in Madison, Wisconsin, was established to meet the needs of individuals and families with possible inherited arrhythmia syndromes, including Catecholaminergic Polymorphic Ventricular Tachycardia, a congenital condition that occurs in patients with no structural heart problems and may cause syncope (fainting) or even sudden cardiac death.

 

About Catecholaminergic Polymorphic Ventricular Tachycardia

 

Any time we experience extreme physical activity or intense emotions, our body releases adrenaline, a type of hormone known as a catecholamine, which causes an increase in heart rate. Catecholaminergic Polymorphic Ventricular Tachycardia is caused by an abnormality in the heart's electrical system that prevents it from responding correctly to catecholamines, causing the heart to develop an abnormal rhythm (arrhythmia) that is faster than normal. This arrhythmia can become dangerous, causing the heart to beat quickly and irregularly, preventing adequate blood flow to the brain and the rest of the body. 


Catecholaminergic Polymorphic Ventricular Tachycardia is caused by an abnormality in the heart's ion channels (proteins that control the passage of important chemicals such as potassium, calcium and sodium) that is due to a genetic mutation. Catecholaminergic Polymorphic Ventricular Tachycardia affects approximately one in 10,000 people. Although both males and females can have the condition, males are more likely to have symptoms.

 

Catecholaminergic Polymorphic Ventricular Tachycardia Symptoms

 

People with Catecholaminergic Polymorphic Ventricular Tachycardia have structurally normal hearts and will generally appear to be in good health. The symptoms of Catecholaminergic Polymorphic Ventricular Tachycardia can range from dizzy spells and fainting episodes to sudden cardiac death. People with Catecholaminergic Polymorphic Ventricular Tachycardia will frequently experience these symptoms when they are exercising or experiencing emotional stress. Most often, a person will have no warning that they are going to faint. Typically, Catecholaminergic Polymorphic Ventricular Tachycardia presents in childhood (around  eight to 10 years old) but this condition can be seen in patients of all ages.

 

Diagnosing Catecholaminergic Polymorphic Ventricular Tachycardia Symptoms

 

In making the diagnosis of Catecholaminergic Polymorphic Ventricular Tachycardia, our clinic staff takes into account

 

  • Symptoms
  • Resting ECG (a test that measures the electrical signals of the heart beat)
  • Exercise stress test results
  • Holter monitor results
  • Family history
  • Results of genetic testing

 

Patients with Catecholaminergic Polymorphic Ventricular Tachycardia will typically have a normal resting ECG. For this reason, an exercise stress test can be helpful to evaluate a person's heart during exercise when the heart rate is higher.

 

Treating Catecholaminergic Polymorphic Ventricular Tachycardia Symptoms

 

Currently, there is no cure for Catecholaminergic Polymorphic Ventricular Tachycardia. Certain medications, such as beta blockers, can help reduce the risk of syncope and sudden death. However, other patients may need more medical intervention, such as the implantation of animplantable cardioverter defibrillator, which is essentially a pacemaker that can also shock the heart in the event of a dangerous arrhythmia. We also make recommendations for a person's lifestyle, such as eliminating or avoiding activities like strenuous exercise, which could trigger a dangerous heart rhythm.

 

Catecholaminergic Polymorphic Ventricular Tachycardia Symptoms and Genetics

 

The majority of genes associated with Catecholaminergic Polymorphic Ventricular Tachycardia are important for the proper formation of the ion channels in the heart. Genetic testing is available to determine if a mutation, or gene change, can be found in any one of these genes.

 

We are typically able to identify a gene mutation in about 50 to 60 percent of patients with symptoms. This means that there are still many patients who have genetic mutations who cannot be identified at present. In the event that a mutation is not detectable, we will make recommendations for screening family members who may also be at risk.

 

If a person does have a genetic mutation, we can test other family members to determine if they also this same genetic mutation. Relatives with an identified mutation have a 50 percent chance of sharing the same gene abnormality. The presence of a genetic mutation does not automatically indicate that a family member will also have symptoms or will develop symptoms of Catecholaminergic Polymorphic Ventricular Tachycardia in the future. It does, however, let us know who should be evaluated by a cardiologist and which family members do not need a cardiac evaluation for Catecholaminergic Polymorphic Ventricular Tachycardia.