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Catecholaminergic polymorphic ventricular tachycardia, or CPVT, is a rare genetic condition. It causes an irregular heart rhythm that can be life threatening. It often shows up in childhood. The first sign is often fainting or near fainting during exercise or strong emotion.
Your heart has 4 chambers. The 2 upper chambers are called atria. The 2 lower chambers are called ventricles. Normally, the signal to start your heartbeat comes from a group of specialized cells in the heart called the sinoatrial node. This node is in the upper right chamber of the heart (right atrium).
People with CPVT can develop a sudden, irregular and rapid heart rhythm from the ventricles. This is called ventricular tachycardia (VT). With this condition, the heart beats so quickly that it does not have enough time to fill between beats. As a result, not enough blood gets pumped forward to the body. This abnormal heart rhythm can cause severe symptoms, such as loss of consciousness, collapse, and even death. If untreated, it is very dangerous.
CPVT results from an abnormality in one of a few different genes. Genes are part of your DNA, the material passed down from parents to children. An abnormal gene leads to the risk of VT. This means it is an inherited problem.
In some cases, CPVT is autosomal dominant. This means you need an abnormal gene from only one of your parents to have it. In other cases, CPVT is autosomal recessive. This means you need an abnormal gene from both of your parents to get the disease.
Researchers are still trying to understand other factors that may increase the chances of having symptoms of the disease. Stress and exercise can trigger episodes. Caffeine may make the episodes worse. Certain medicines, such as catecholamines, can also make it worse.
Having a relative with CPVT puts you at risk for the disease. If someone in your family has CPVT, you may need to see a healthcare provider to get checked. You may need a genetic test.
There isn’t anything you can do to decrease your risk of CPVT, since it is inherited. But you can do certain things to reduce the chance of having episodes. This includes things such as avoiding triggers and taking certain medicines. Your healthcare provider may also recommend an implantable cardioverter-defibrillator (ICD). An ICD can detect a problem heart rhythm and give a shock to help stop it.
The symptoms typically start with VT. You may faint or feel lightheaded. You may have an unpleasant awareness of your heartbeat. These symptoms tend to occur when you are physically active. It may also happen when you feel emotionally stressed. The VT may cause you to lose consciousness. This is commonly the first sign of the disease. Sometimes the VT goes away, and symptoms stop.
Other times, VT can turn into ventricular fibrillation (VF). This is more dangerous. It causes the ventricles quiver. They are unable to pump blood. This sudden loss of heart function leads to a cardiac arrest. This can cause sudden cardiac death. A person stops breathing and becomes unresponsive. Unfortunately, cardiac arrest is sometimes the first symptom of CPVT.
Diagnosis of CPVT is often hard. Your healthcare provider will ask about your medical history. He or she will give you a physical exam. You may also have tests such as:
Healthcare providers use a variety of treatments for CPVT such as:
Some people may also need an implantable cardioverter-defibrillator, or ICD. An ICD is a small device put under the skin near the chest. It can detect a problem heart rhythm and give a shock to help stop it. In some circumstances, a catheter ablation may be recommended.
If you still have symptoms, your healthcare provider may advise sympathetic denervation surgery. This is a surgery to remove the adrenaline nerves that can signal the heart to beat faster.
If you are in ventricular tachycardia, you may need a shock to your heart. This helps restore a normal rhythm. VF is a medical emergency. It needs treatment right away. This includes a shock to the heart to help restore normal heart rhythm.
Your healthcare provider may give you more instructions about how to manage your CPVT, such as:
See a healthcare provider right away if your symptoms get worse or happen more often.
CPVT is a rare genetic condition. It can cause VT. This is an abnormal heart rhythm. This rhythm sometimes goes away with minimal symptoms. Sometimes it turns into ventricular fibrillation. This causes cardiac arrest.
Tips to help you get the most from a visit to your healthcare provider:
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