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Don’t fight Wolff-Parkinson-White syndrome alone.
Find your community on the free RareGuru App.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.
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.
Source: GARD Last updated on 05-01-20
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). 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).
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.
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.
Last updated on 05-01-20
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
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
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:
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
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
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
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
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
Last updated on 04-27-20
Wilk D, Williams JL. Genetic Forms of Wolff-Parkinson- White EP Lab Digest. October 2012; 12(10).
Last updated on 04-27-20
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