Catecholaminergic Polymorphic Ventricular Tachycardia | Heart Rhythm Disorder | MedStar Health

A genetic condition that causes an abnormal heartbeat during physical activity and stress

Certain proteins in our heart operate ion channels, which control the flow of sodium, potassium, and calcium through the heart’s cells. These molecules help regulate your heart’s electrical activity.

This is a genetic condition that mutates these proteins, leading to an irregular or abnormal heart rhythm (arrhythmia) during physical activity or emotional stress. It’s also known as familial polymorphic ventricular tachycardia (FPVT).

These ventricular arrhythmias may return to a normal heartbeat on their own, but sometimes they can worsen, causing a more dangerous heart rhythm called ventricular fibrillation. Without immediate medical treatment, this condition can lead to sudden cardiac death.

This heart rhythm disturbance is suspected to cause a significant number of unexplained sudden cardiac deaths in children and young adults without other heart abnormalities. If a family member is diagnosed with CPVT, you may want to consider genetic testing to determine your risk of having the disorder or passing it on to your children.

Symptoms

While this disorder usually starts in childhood, it can develop during adulthood.

Symptoms usually appear during physical activity or emotional stress and can include:

  • Dizziness or lightheadedness

  • Fainting

  • Palpitations or sensations of a racing heart or fluttering in your chest

Tests

A standard electrocardiogram (ECG) generally will not detect this heart rhythm disturbance/disorder. If your doctor suspects the disease based on family history or symptoms, you may need further testing.

Angiogram (angiography)

An angiogram is a special X-ray taken as a special dye is injected through a thin, flexible tube called a catheter to detect blockages or aneurysms in blood vessels.

Chest X-ray

Chest X-rays use a small dose of radiation to create pictures of the structures inside the chest, including the lungs, heart, and chest wall.

Computerized Tomography (CT) Scan

The cardiac computed tomography scan, or cardiac CT, uses X-rays to create three-dimensional images of your heart and blood vessels.

Echocardiogram

An echocardiogram uses high-frequency sound waves to create images of your heart.

Electrocardiogram (ECG)

An electrocardiogram, also known as an ECG, measures the heart’s electrical activity.

Electrophysiology testing

Electrophysiology testing is used to evaluate the cause and location of an abnormal heartbeat (known as an arrhythmia).

Event monitors

An event monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but where an electrocardiogram takes place over a few minutes, an event monitor measures heart rhythms over a much longer time.

Holter monitors

A Holter monitor is a small device that records the heart’s electrical activity. It’s similar to an electrocardiogram, but whereas an electrocardiogram records over a few minutes, a Holter monitor records over the course of a day or two.

Loop recorder

 A loop recorder is a device that’s implanted underneath the skin of your chest to record your heart rhythm for up to 3 years.

Stress tests

Stress tests are used to assess how your heart works during physical activity. There are several types of stress tests, including treadmill or bike stress tests, nuclear stress tests, stress echocardiograms, and chemically induced stress tests.

Treatments

Treatment options include lifestyle modifications, such as avoiding strenuous activity, medications to regulate heart contractions, or implanting a defibrillator to correct dangerous heart arrhythmias with electric shocks. 

Cardiac ablation

Cardiac ablation uses heat or cold to destroy heart tissue causing abnormal heart rhythms known as arrhythmias.

Implantable Cardioverter Defibrillator (ICD)

An implantable cardioverter defibrillator (ICD) is a device implanted below your collarbone that monitors your heart’s rhythm. When it detects an abnormal rhythm, it delivers an electrical impulse or shock to the heart to correct it.

Additional information

Genetic counseling and testing for heart conditions

Our cardiogenetics experts work to improve early detection and treatment for patients at risk for certain genetic heart conditions.

Read our Cardiovascular Performance & Outcomes Booklet

Ask MHVI

Have questions for our heart and vascular program? Email us at AskMHVI@medstar.net.