What Is CPVT (Catecholaminergic Polymorphic Ventricular Tachycardia)?

Reviewed by James Beckerman, MD, FACC on March 27, 2020

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a heart rhythm problem, or arrhythmia. If you have it, your heartbeat is faster and irregular at times. When this happens, it’s harder for the heart to pump enough blood to the rest of the body.

This isn’t the same as when your heart rate naturally rises briefly during stress or activity.

Although CPVT can be dangerous if left untreated, when you make the right lifestyle choices and follow your doctor's treatment plan, you can manage this condition and lead a full life.

Causes

CPVT happens because of problems in genes, which are usually handed down through families. Scientists have found three gene problems that cause CPVT.

Most people get CPVT due to a faulty gene they inherit from their parents. The most common form of CPVT is a “dominant” genetic condition, which means that if one parent has the condition, each child has a 50-50 chance of getting it.

A rarer form of CPVT happens if you inherit an abnormal gene from both parents.

It’s not common, but occasionally CPVT happens in people who have no family history of the disease, so it's due to genetic changes that weren’t handed down.
About 1 out of every 10,000 people have CPVT. You may be more likely to have this condition if there is a history of premature death in your family, since undiagnosed CPVT can cause such sudden deaths.

Symptoms

Signs of CPVT can start as early as childhood. You may faint or feel like fainting more often than other people, especially when you exercise, get excited, or feel strong emotions. You may also feel your heart beat fast and hard on a regular basis (called palpitations).

Sometimes, CPVT may lead to seizures. If it continues untreated for long enough, your heart may stop, which can cause death.

Diagnosis

If your doctor thinks you have CPVT, you’ll likely get a test called an electrocardiogram (EKG) to record your normal heart rhythm at rest. You'll then do a supervised "exercise challenge" in a doctor’s office to see how your heartbeat changes when you’re active. You might get an irregular heart rhythm during this challenge if you have CPVT.

In some cases, your doctor may ask you to wear a Holter monitor. This is a portable device that records how your heartbeat changes over a day.

Your doctor may also recommend DNA testing to see if you carry any of the gene glitches that cause the condition. If you do, your family members should get tested for CPVT if they haven’t already.

Treatment

Your doctor will likely prescribe medication to help control your irregular heart rhythm. The first treatment is a type of medication called beta-blockers, such as nadolol or propanolol. They target a body chemical called adrenaline to help keep your heart rhythm even. You may also take a drug called flecainide, which slows down electrical signals in the heart and makes rhythm interruptions less likely.

If your condition is severe enough, or if you've had episodes of fainting or heart trouble, your doctor may recommend an implantable cardioverter defibrillator, or ICD. This device, placed on your chest wall, detects unusual heart rhythms and delivers an electrical pulse to get your heartbeat back on track when needed.

Some people with CPVT still have serious symptoms even when they take medication and have an ICD. If so, your doctor may recommend a surgical procedure called left cardiac sympathetic denervation to remove a certain chain of nerves that can speed up your heartbeat. You’ll have a much lower chance of life-threatening heart rhythm changes after this surgery.

Everyday Life

Your lifestyle is a key part of managing CPVT. Take your medication as prescribed to help prevent any sudden heart rhythm changes. To make sure your heartbeat stays controlled, your doctor may ask you to avoid strenuous exercise such as high-intensity aerobics or contact sports. (With your medical team's help, you can choose safer alternatives that help you stay fit.) You may also need to limit or avoid caffeine.

WebMD Medical Reference

Sources

SOURCES: 

Columbia University Irving Medical Center: "Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)."

Sudden Arrhythmia Death Syndromes (SADS) Foundation: "CPVT."

National Institutes of Health: "Catecholaminergic Polymorphic Ventricular Tachycardia."

American College of Cardiology: "The Athlete With Catecholaminergic Polymorphic Ventricular Tachycardia."

Saint Luke's Health System Kansas City: "Understanding Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)."

Cedars Sinai: "Catecholaminergic Polymorphic Ventricular Tachycardia."

Mayo Clinic: "Beta Blockers,” "Flecainide."

Frontiers in Cardiovascular Medicine: "Cardiac Sympathetic Denervation in Channelopathies."

University of California at San Francisco (UCSF) Cardiology: "Catecholaminergic Polymorphic Ventricular Tachycardia."

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