Wolff-Parkinson-White (WPW) Syndrome

What Is Wolff-Parkinson-White (WPW) Syndrome?

Wolff-Parkinson-White (WPW) syndrome is a rare heart condition that occurs when a child has an extra electrical pathway, or “accessory pathway,” between the heart’s upper and lower chambers. Signals reach the heart’s lower chambers (ventricles) too quickly, causing them to contract earlier than they should. This response is known as preexcitation. 

WPW syndrome is named for the three doctors — Louis Wolff, John Parkinson and Paul Dudley White — who first described the condition in 1930. It’s also called preexcitation syndrome. 

How Does WPW Syndrome Affect the Heart?

The heart’s electrical system sends signals or impulses that control heart rate and rhythm. Usually, the signals travel from the upper heart chambers (atria) to the lower chambers (ventricles) via the atrioventricular (AV) node. When a child has an accessory pathway, the electrical signals can:

• Bypass the AV node 
• Travel from the lower chambers to the upper chambers (opposite of the normal direction) 

Both accessory pathways can increase a child’s risk of an irregular heart rhythm (cardiac arrhythmia).

What Are the Complications of WPW Syndrome?

WPW syndrome is one of the most common causes of abnormally fast heart rates in children. The most common type of arrhythmia in children with WPW syndrome is supraventricular tachycardia (SVT).

Rarely, children and adults with WPW syndrome can also develop an arrhythmia called atrial fibrillation (A-fib). When A-fib occurs, the upper heart chambers have a very irregular, and at times very fast, heart rate. Because people with WPW syndrome have an extra electrical pathway, electrical signals can travel to the lower chamber rapidly and skip the AV node. This leads to a life-threatening arrhythmia called ventricular fibrillation (V-fib). 

V-fib may cause sudden cardiac arrest in seemingly healthy children and teens. Fortunately, sudden death in patients with WPW syndrome is very rare. Treatments can further lower or eliminate this risk. 

What Causes WPW Syndrome?

The extra electrical pathway is a congenital heart condition, meaning it’s present at birth. It occurs in approximately three out of every 1,000 children. For most children, the cause is unknown. Often, cases are sporadic, affecting just one member of a family. It can be more common in children who have structural heart disease, such as Ebstein’s anomaly. 

What Are the Signs and Symptoms of WPW Syndrome? 

WPW syndrome doesn’t always cause symptoms. When arrhythmia symptoms occur, they may last for a few seconds, minutes or hours. Symptoms can happen at any time, including during rest or physical activity. 

Signs include:

• Chest pain or discomfort
• Dizziness or fainting (syncope)
• Heart palpitations (sensation of skipped heartbeats or an unusually fast heartbeat)
• Shortness of breath

How Is WPW Syndrome Diagnosed?

Some children who don’t have symptoms learn they have WPW syndrome after getting an electrocardiogram (EKG/ECG) as part of a sports physical. A child may also get an EKG to determine the cause of heart palpitations or other symptoms. An EKG records the heart’s electrical activity and can detect an arrhythmia or the presence of extra electrical pathways.

If your child is being evaluated for WPW syndrome, they may also receive these tests:

• Echocardiogram (echo) to look for heart structure changes
• Holter monitor (portable EKG) to record the heart’s electrical activity for at least 24 consecutive hours
• Cardiac stress test to record heart activity while your child is physically active 

How Is WPW Syndrome Treated?

Treatments depend on your child’s symptoms. At Lurie Children’s, your child receives expert care from a pediatric electrophysiologist. These doctors specialize in heart rhythm disorders. The management of WPW syndrome continues to evolve as ongoing research helps doctors better understand the condition. 

Our electrophysiologists partner with your family to make decisions. We discuss the most up-to-date medical recommendations on how to treat WPW syndrome, review your child’s unique risk factors for developing an arrhythmia and listen to your family’s concerns to help you make an informed decision about care. 

We offer comprehensive treatments, including:

Nonsurgical Approaches

Nonsurgical options for WPW syndrome include:

• Active surveillance: Your child continues to get periodic EKGs, echocardiograms and evaluations by a pediatric electrophysiologist to check for heart changes.
• Medications: Daily oral antiarrhythmic medications can decrease the frequency of SVT if your child frequently has this arrhythmia. 

Electrophysiology (EP) study and cardiac ablation

During an EP study, your child’s doctor tests the extra pathway and the heart’s electrical activity. If necessary, they perform cardiac ablation. This minimally invasive procedure may deliver high heat (radiofrequency ablation) or extreme cold (cryoablation) to destroy the extra pathway. 

A successful ablation is the only “cure” for WPW syndrome and eliminates the risk of sudden death. It takes place using thin, flexible tubes (catheters) threaded through blood vessels into your child’s heart. It doesn’t require surgery. 

CPR and AEDs

We recommend that all people know CPR and how to use an automated external defibrillator (AED). This portable device delivers an electric shock that can restore a normal heartbeat when someone is in cardiac arrest. Performing these actions could save the life of someone experiencing a sudden cardiac arrest from any cause, including WPW syndrome.

We also recommend working with your child’s school and athletic programs to ensure AEDs are available. Lurie Children’s is part of a national program called Project ADAM (Automated Defibrillators in Adam’s Memory) that provides CPR and AED training for school staff. We can help your child’s school become a Heart Safe School.

What Is Lurie Children’s Approach to Caring for Children With WPW Syndrome?

Children with WPW syndrome receive advanced care at Lurie Children’s Electrophysiology (EP) Program. We offer:

• Care you can trust: Our EP team is nationally known for excellence in managing arrhythmia in children. We perform a high volume of cardiac ablations every year. This experience has led to success rates that exceed 95 percent, with complication rates below the national average. 
• Recognized experts: Some of our doctors are part of an American College of Cardiology (ACC) workforce helping to develop new clinical recommendations for WPW syndrome. They also participate in international research studies that improve our understanding of WPW syndrome and the best ways to treat it. We’re helping to set the standard for care.
• Second opinions: We see children from across the Midwest and the nation. We offer in-person and virtual second opinions, providing comprehensive evaluations and care plans during a single visit. 
• Heart surgery options: When needed, our EP team and pediatric heart surgeons work together to manage WPW syndrome that occurs with structural heart changes. Surgery takes place at our Level I Children’s Surgery Center, which has the highest-level recognition from the American College of Surgeons (ACS). 
• Dedicated heart care unit: Children recover from cardiac ablation or heart surgery at the Regenstein Cardiac Care Unit. This unit is among a few in the nation that keep your child in the same private room from admission to discharge. You can stay with your child.

The Heart Center at Lurie Children’s 

The pediatric cardiology and cardiac surgery programs at Lurie Children’s Heart Center rate among the country’s best by Newsweek and U.S. News & World Report. Your child receives compassionate care from experts with deep expertise in treating common and complex pediatric heart conditions and arrhythmias. We partner with your family to manage chronic heart diseases and help your child lead a full, active life.

Learn more about our Heart Center