Wolff-Parkinson-White syndrome

Title

Not a rare disease

Other Names:

WPW syndrome; Ventricular familial preexcitation syndrome; Auriculoventricular accessory pathway syndrome; WPW syndrome; Ventricular familial preexcitation syndrome; Auriculoventricular accessory pathway syndrome; False bundle branch block syndrome; Anomalous ventricular excitation syndrome; Preexcitation syndrome See More

Categories:

Congenital and Genetic Diseases

Summary Summary

Wolff-Parkinson-White syndrome causes a problem with the rate or rhythm of the heartbeat (arrhythmia). People with the syndrome are born with a heart abnormality that affects the coordinated movement of electrical signals through the heart. This leads to an abnormally fast heartbeat (tachycardia) and other arrhythmias. The most common arrhythmia associated with Wolff-Parkinson-White syndrome is paroxysmal supraventricular tachycardia. The syndrome is especially common in people of Chinese descent.^[1]

In most cases, the cause of Wolff-Parkinson-White syndrome is unknown. A small percentage of cases are caused by genetic changes (mutations or pathogenic variants) in the PRKAG2 gene. These cases appear to be inherited in an autosomal dominant manner. A diagnosis of the syndrome is based on an electrocardiogram (ECG) or Holter test that shows episodes of tachycardia. Treatment for the syndrome may depend on the severity of symptoms but can include medications or surgery.^[2]

Last updated: 3/14/2018

Symptoms Symptoms

Symptoms of Wolff-Parkinson-White syndrome occur because the heart’s normal rhythm is disrupted. This is called an arrhythmia. The most frequent type of arrhythmia associated with Wolff-Parkinson-White syndrome is paroxysmal supraventricular tachycardia. This means that there is a rapid heart rate (tachycardia) that originates from the area above the two lower chambers of the heart (supraventricular), and that the abnormal rhythm occurs intermittently (paroxysmal).[1] Other symptoms of Wolff-Parkinson-White syndrome may include dizziness, a feeling of an irregular heartbeat (palpitations), shortness of breath, low blood pressure (hypotension), and fainting. Rarely, the arrhythmia associated with Wolff-Parkinson-White syndrome can cause the heart to stop (cardiac arrest).^[1]

Some people with Wolff-Parkinson-White syndrome also have other heart abnormalities, most commonly the Ebstein anomaly. The Ebstein anomaly affects the tricuspid valve, which connects the right upper chamber of the heart (atrium) to the right lower chamber (ventricle). However, some people with Wolff-Parkinson-White syndrome do not have any other heart abnormalities, and some people have no symptoms of the syndrome at all.^[1]

People who do develop symptoms of Wolff-Parkinson-White syndrome may have them beginning at any time of life. These symptoms may occur in episodes that start and stop abruptly. The frequency of episodes can vary. Some people may have only a few episodes in a lifetime, while others may have more than one episode a week.^[3]

Last updated: 3/14/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant inheritance		0000006
Cardiomyopathy	Disease of the heart muscle	0001638
Palpitations	Missed heart beat Skipped heart beat [ more ]	0001962
Paroxysmal atrial fibrillation		0004757
Paroxysmal supraventricular tachycardia		0004763
Prolonged QRS complex		0006677
Shortened PR interval		0005165
Stroke		0001297
Sudden cardiac death	Premature sudden cardiac death	0001645
Ventricular preexcitation with multiple accessory pathways		0006684
Wolff-Parkinson-White syndrome		0001716

Showing of 11 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 3/1/2020

Do you have updated information on this disease? We want to hear from you.

Cause Cause

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.^[1]^[2] This extra electrical pathway is present from birth in people with the syndrome.^[1]

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.^[1] When there is a pathogenic variant in the PRKAG2 gene, the heart may be more likely to develop the extra electrical pathway.^[1] Some people with pathogenic variants in the PRKAG2 gene also have an enlarged heart muscle (cardiomyopathy).^[4]

Last updated: 3/14/2018

Inheritance Inheritance

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.^[5]

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.^[1] 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.^[1]

Last updated: 3/14/2018

Diagnosis Diagnosis

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.^[2]

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.^[1]^[2]

Last updated: 3/14/2018

Treatment Treatment

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.^[2]^[3]

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.[3] In some cases, surgery may be recommended, even for people who have never had symptoms of the syndrome.^[4]

Last updated: 3/14/2018

Prognosis Prognosis

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.^[6]

Last updated: 3/14/2018

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Wolff-Parkinson-White syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
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".

Patient Registry

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:
CHD GENES Study

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Heart Association
7272 Greenville Avenue
Dallas, TX 75231-4596
Toll-free: 800-242-8721
Telephone: 214-570-5978
E-mail: https://www.heart.org/en/forms/general-questions-and-latest-research-information
Website: https://www.heart.org
Arrhythmia Alliance
PO Box 5507
Hilton Head Island, SC 29938
Telephone: 843-415-1886
E-mail: info-us@heartrhythmalliance.org
Website: http://www.heartrhythmalliance.org/

Organizations Providing General Support

British Heart Foundation
Greater London House
180 Hampstead Road
London, NW1 7AW United Kingdom
Telephone: 0300 330 3322
E-mail: supporterservices@bhf.org.uk
Website: https://www.bhf.org.uk/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Genetics Home Reference (GHR) contains information on Wolff-Parkinson-White syndrome. This website is maintained by the National Library of Medicine.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
The American Heart Association has and information page on Wolff-Parkinson-White syndrome on their Web site.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Wolff-Parkinson-White syndrome. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

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

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Have a question? Contact a GARD Information Specialist.

References References

Wolff-Parkinson-White syndrome. Genetics Home Reference (GHR). June 2017; https://ghr.nlm.nih.gov/condition/wolff-parkinson-white-syndrome.
Chen MA. Wolff-Parkinson-White syndrome. MedlinePlus. February 24, 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000151.htm.
Wolff Parkinson White Syndrome. National Organization for Rare Disorders. 2007; https://rarediseases.org/rare-diseases/wolff-parkinson-white-syndrome/.
Wolff-Parkinson-White Syndrome. Online Mendelian Inheritance in Man. May 17, 2010; https://www.omim.org/entry/194200.
Wilk D and Williams JL. Genetic Forms of Wolff-Parkinson-White. Electrophysiology Lab Digest. October 2012; 12(10):https://www.eplabdigest.com/articles/Genetic-Forms-Wolff-Parkinson-White.
Ellis CR. Wolff-Parkinson-White Syndrome. Medscape Reference. January 8, 2017; https://emedicine.medscape.com/article/159222-overview.