Rare Medical News

Disease Profile

Wolff-Parkinson-White syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

no.svg

Other names (AKA)

WPW syndrome; Ventricular familial preexcitation syndrome; Auriculoventricular accessory pathway syndrome;

Categories

Congenital and Genetic Diseases

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]

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]

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.

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

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]

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]

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]

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

    Organizations Providing General Support

      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

      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • Genetics Home Reference (GHR) contains information on Wolff-Parkinson-White syndrome. This website is maintained by the National Library of Medicine.
      • 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

            References

            1. Wolff-Parkinson-White syndrome. Genetics Home Reference (GHR). June 2017; https://ghr.nlm.nih.gov/condition/wolff-parkinson-white-syndrome.
            2. Chen MA. Wolff-Parkinson-White syndrome. MedlinePlus. February 24, 2016; https://www.nlm.nih.gov/medlineplus/ency/article/000151.htm.
            3. Wolff Parkinson White Syndrome. National Organization for Rare Disorders. 2007; https://rarediseases.org/rare-diseases/wolff-parkinson-white-syndrome/.
            4. Wolff-Parkinson-White Syndrome. Online Mendelian Inheritance in Man. May 17, 2010; https://www.omim.org/entry/194200.
            5. 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.
            6. Ellis CR. Wolff-Parkinson-White Syndrome. Medscape Reference. January 8, 2017; https://emedicine.medscape.com/article/159222-overview.

            Rare Medical News