Catecholaminergic Polymorphic Ventricular Tachycardia

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What is Catecholaminergic Polymorphic Ventrricular Tachycardia (CPVT)?

CPVT is short for catecholaminergic polymorphic ventricular tachycardia [KAT-ik-ohl-AH-mihn-er-jik PAH-lee-mawrf-IK ven-TRIK-yuh-ler tak-i-KAHR-dee-uh].

With CPVT, physical activity or emotional stress cause an abnormal heartbeat (arrhythmia) and lead to dizziness or fainting. CPVT is most often diagnosed in children between the ages of 7 and 12.

CPVT is identified by the presence of a ventricular tachycardia. Tachycardia refers to any heartbeat that is more than 100 beats per minute in adolescents and adults, and a heartbeat that is more than 140 beats per minute in children between 2 and 12 years of age. Most experience a heartbeat of over 200 beats per minute during an episode of CPVT. CPVT is a type of ventricular fibrillation [ven-TRIK-yuh-ler fahy-bruh-LEY-shuh], which refers to an irregular heartbeat in the lower chambers of the heart.

If left untreated, these irregular, rapid heart rates can cause poor blood flow and cause the heart to stop beating (cardiac arrest). As many as 1 in 3 cases of CPVT lead to cardiac arrest and the condition may be a significant cause of death in children.

CPVT can be confused with cardiomyopathy [kahr-dee-oh-mahy-OP-uh-thee] but the two conditions are quite different. Cardiomyopathy is an enlarged or thickened heart muscle that damages the surrounding tissues. CPVT is the presence of an abnormally fast heartbeat in the absence of other heart abnormalities.

Symptoms

One of the ways to identify CPVT is the onset of symptoms after your child has engaged in lots of physical activity or had emotional stress. Some of the symptoms you may observe in your child include the following:

  • Lightheadedness
  • Dizziness
  • Fainting
  • Seizure
  • Irregular, fast heartbeat
  • Cardiac arrest

CPVT can be genetic. If a family member has the condition your child should also be tested, even if there aren’t any symptoms.

When to See a Doctor

In families where CPVT has already been diagnosed, all family members, including children, should be tested for the condition.

If your child has dizziness, fainting, seizure or irregular heartbeat after undergoing physical activity or emotional stress, you should consult your child’s healthcare provider right away. You need to find out if CPVT is the cause.

Causes

About half of all cases of CPVT are inherited from a family member who has passed down the inability to create the proteins needed to regulate the heartbeat. The absence of these proteins results in problems with keeping up calcium levels and in turn causes ventricular tachycardia. There is a 50 percent chance that a child will have CPVT if a parent has been diagnosed with the condition.

Doctors are not sure what causes other CPVT cases. One out of every 10,000 people have CPVT, and for half of those cases there is no genetic link.

Diagnosis and Tests

CPVT is usually tested with treadmill stress testing. Your child may have a normal resting heart rate, but abnormalities will appear under stress. Treadmill testing involves watching your child’s blood pressure and heartrate while they walk or run on a treadmill.

Genetic testing is also available and can be part of a broader panel of tests to decide the source of heart rhythm problems.

Treatments

There is no cure for CPVT but treatment can prevent symptoms. If your child does not show symptoms of CPVT, but has tested positive for the condition, doctors advise treatment to avoid sudden cardiac death. There are a few potential treatments, including:

  • Medicines. Beta-blockers or calcium channel-blockers may be prescribed. You’ll need to monitor your child’s dose closely on these medicines. Follow your healthcare provider’s instructions.
  • Defibrillator [dee-FIB-ruh-lay-ter]. If your child’s symptoms persist or are severe, an implantable cardioverter defibrillator [KAHR-dee-oh-VER-ter] (ICD) may be recommended.

In addition to these treatments, it is suggested that your family and your child’s school have an automatic external defibrillator on hand and understand how to use it in the event of an emergency.

Prevention

There is no way known to prevent CPVT. Your child’s CPVT can be managed with medicine, arrhythmia management techniques, and in more extreme cases, a defibrillator,.

Support and Resources

Catecholaminergic polymorphic ventricular tachycardia, or CPVT, is a condition that involves rapid heartrate triggered by physical activity or emotional stress. This causes dizziness and fainting. If left untreated, CPVT can cause the heart to stop beating (cardiac arrest) and cause sudden death.

CPVT is most often diagnosed in childhood. Half of cases are inherited, but doctors aren’t sure what causes the condition in children who don’t inherit it.