Wolff-Parkinson-White syndrome

What causes Wolff-Parkinson-White syndrome?

Wolff-Parkinson-White syndrome is caused by having an extra pathway in the heart that causes a very rapid heart rate. Normally, electrical signals in the heart go through a pathway that helps the heart beat regularly. The electrical pathway of the heart prevents extra beats from occurring and keeps the next beat from happening too soon. In people with Wolff-Parkinson-White syndrome, there is an extra, or accessory, pathway that may cause a very rapid heart rate. This extra electrical pathway is present from birth in people with the syndrome.

In most cases, it is not known why a person with Wolff-Parkinson-White syndrome has an extra electrical pathway in the heart. In some cases, a genetic change (mutation or pathogenic variant) in the PRKAG2 gene causes the syndrome. This gene provides instructions to the body to make a protein that is likely involved in the development of the heart. When there is a pathogenic variant in the PRKAG2 gene, the heart may be more likely to develop the extra electrical pathway. Some people with pathogenic variants in the PRKAG2 gene also have an enlarged heart muscle (cardiomyopathy).

Last updated on 05-01-20

How is Wolff-Parkinson-White syndrome diagnosed?

Wolff-Parkinson-White syndrome is suspected when a doctor sees a person who has symptoms of the syndrome such as a rapid heartbeat (tachycardia) or palpitations. The doctor may recommend tests including an electrocardiogram (ECG), which records the electrical activity of the heart over time. Because the symptoms of Wolff-Parkinson-White syndrome may only occur at certain times, a doctor may also recommend the use of a Holter monitor to confirm the diagnosis. A Holter monitor is a portable device that records the heart’s rhythms over time. This device can therefore be used over a number of days to confirm if there is an abnormal heart rhythm.

Because some people with Wolff-Parkinson-White syndrome have no symptoms of the syndrome, in some cases a person who has no noticeable symptoms may be diagnosed due to discovering episodes of tachycardia by ECG or Holter monitor when they are having testing of the heart for other reasons.

Last updated on 05-01-20

Is Wolff-Parkinson-White syndrome inherited?

Most cases of Wolff-Parkinson-White syndrome occur in people with no apparent family history of the syndrome. These cases are described as sporadic, meaning they are not inherited. In these cases, it is possible that a combination of genetic and environmental causes are responsible for the development of the syndrome.

People who have a genetic change (mutation or pathogenic variant) in PRKAG2 causing Wolff-Parkinson-White syndrome have an inherited form of the syndrome. In these cases, the syndrome is inherited in an autosomal dominant manner. This means that only one copy of the PRKAG2 gene is changed for a person to have symptoms of the syndrome. We inherit one copy of each gene from our mother and the other from our father. When a person with Wolff-Parkinson-White syndrome that is caused by a pathogenic variant in PRKAG2 has children, for each child there is a:

  • 50% chance to inherit the changed copy of the PRKAG2 gene, meaning he or she will have Wolff-Parkinson-White syndrome
  • 50% chance to inherit the working copy of the PRKAG2 gene, meaning he or she will not have Wolff-Parkinson-White syndrome.

A doctor may determine if other family members are at risk to have symptoms of Wolff-Parkinson-White syndrome by taking a detailed family history. If no other family members have symptoms of the syndrome, it is most likely that the syndrome is sporadic and is not caused by a pathogenic variant in PRKAG2.

Last updated on 05-01-20

What is the long-term outlook for people with Wolff-Parkinson-White syndrome?

The long-term outlook for people with Wolff-Parkinson-White syndrome is considered excellent due to effective treatment options. Some people with Wolff-Parkinson-White syndrome may be at an increased risk for cardiac arrest or sudden cardiac death (SCD) due to a number of factors, including family history of SCD, more complicated accessory electrical pathways in the heart, and more severe tachycardia symptoms. However, even for those at an increased risk, the availability of treatments that can permanently stop symptoms of the syndrome such as catheter ablation has made SCD a rare outcome.

Last updated on 05-01-20

How might Wolff-Parkinson-White syndrome be treated?

The treatment for Wolff-Parkinson-White syndrome may depend on whether a person has any symptoms of the syndrome. For people who do have symptoms, medications such as antiarrhythmic drugs may be recommended. In other cases, a doctor may recommend a surgical procedure to determine the location of the extra electrical pathway, followed by a catheter ablation. During this procedure, a catheter is used to direct radiofrequency energy at the location of the extra electrical pathway in the heart, which destroys the tissue. This procedure can help restore a regular heart rhythm with a high success rate.

If a person with Wolff-Parkinson-White syndrome does not have any symptoms, a doctor may recommend monitoring of the heart to see if symptoms will develop. The exact treatment recommendation depends on the symptoms of each person as well as family history information and other health problems that a person may have. In some cases, surgery may be recommended, even for people who have never had symptoms of the syndrome.

Last updated on 05-01-20

Clinical Research Resources


The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Last updated on 04-27-20

Patient Registry

Cure RTD Foundation

A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Wolff-Parkinson-White syndrome. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

Registries for Wolff-Parkinson-White syndrome:

Last updated on 04-27-20

Selected Full-Text Journal Articles


Wilk D, Williams JL. Genetic Forms of Wolff-Parkinson- White EP Lab Digest. October 2012; 12(10).

Last updated on 04-27-20

Name: American Heart Association 7272 Greenville Avenue
Dallas, TX, 75231-4596, United States
Phone: 214-570-5978 Toll Free: 800-242-8721 Email: https://www.heart.org/en/forms/general-questions-and-latest-research-information Url: https://www.heart.org
Name: Arrhythmia Alliance PO Box 5507
Hilton Head Island, SC, 29938, United States
Phone: 843-415-1886 Email: info-us@heartrhythmalliance.org Url: http://www.heartrhythmalliance.org/
Name: British Heart Foundation Greater London House 180 Hampstead Road
London, NW1 7AW, United Kingdom
Phone: 0300 330 3322 Email: supporterservices@bhf.org.uk Url: https://www.bhf.org.uk/

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