Treatment for Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) (Child)

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic condition. It can cause a very serious abnormal heart rhythm sometimes triggered by emotional stress or exercise. It can result in fainting or sudden death. Some children with CPVT have no symptoms. They are diagnosed only through genetic testing. Other children may have signs that include palpitations, near fainting, seizure-like brain activity, or cardiac arrest.

Types of treatment

A cardiologist will manage your child’s CPVT. Treatment is aimed at preventing and treating the abnormal heart rhythm. Treatment may include:

• Lifestyle changes. A child with CPVT should not do rigorous exercise or play competitive sports. These may cause episodes of CPVT.

• Medicine. Most people with CPVT take a type of medicine called a beta-blocker. This medicine slows the heart rate and reduces the risk of abnormal heart rhythm. This works well for most people with CPVT. Other heart rhythm medicines may also be given.

• Implantable cardioverter-defibrillator (ICD). An ICD is a small device implanted within the body to treat dangerous heart rhythms. It sends an energy shock if the dangerous rhythm is detected. This can restore normal heart rhythm within seconds.

• Catheter ablation. This is a procedure where the doctor inserts a flexible thin tube (catheter) through the blood vessels into the heart to stop (ablate) abnormal electrical channels (heartbeats).

• Sympathetic denervation. Some children with continued symptoms may need surgery to remove the nerves that signal the heart to beat faster.

Living with CPVT

• Your child will need to see their healthcare provider at least every 6 to 12 months. Your child’s heart will be checked with ECG and heart tests.

• Talk with the healthcare provider about what kind of activity is safe for your child.

• Tell all of your child’s healthcare providers that they have CPVT.

• Tell family members to be checked for CPVT.

• Talk with your healthcare provider about genetic counseling if you plan to get pregnant.

Keeping your child safe

Make sure that family, friends, and school staff know that your child has CPVT. They will need to know what to do if your child faints or can’t respond. They should know:

• When to call 911

• How to do CPR (cardiopulmonary resuscitation)

• How to use an AED (automated external defibrillator)

An AED can be used even if your child has an ICD. The American Red Cross and the American Heart Association offer CPR classes that include the use of an AED.

When to call the healthcare provider

Call the healthcare provider right away if you notice a change in your child’s symptoms.