Wolff-Parkinson-White syndrome
Last updated 19 Sept 2024
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Wolff-Parkinson-White syndrome is a condition characterised by abnormal electrical pathways in the heart that cause a disruption of the heart's normal rhythm (arrhythmia).
Description
Wolff-Parkinson-White syndrome is a condition characterised by abnormal electrical pathways in the heart that cause a disruption of the heart's normal rhythm (arrhythmia).
The heartbeat is controlled by electrical signals that move through the heart in a highly coordinated way. A specialized cluster of cells called the atrioventricular node conducts electrical impulses from the heart's upper chambers (the atria) to the lower chambers (the ventricles). Impulses move through the atrioventricular node during each heartbeat, stimulating the ventricles to contract slightly later than the atria.
People with Wolff-Parkinson-White syndrome are born with an extra connection in the heart, called an accessory pathway, that allows electrical signals to bypass the atrioventricular node and move from the atria to the ventricles faster than usual. The accessory pathway may also transmit electrical impulses abnormally from the ventricles back to the atria. This extra connection can disrupt the coordinated movement of electrical signals through the heart, leading to an abnormally fast heartbeat (tachycardia) and other changes in heart rhythm. Resulting symptoms include dizziness, a sensation of fluttering or pounding in the chest (palpitations), shortness of breath, and fainting (syncope). In rare cases, arrhythmias associated with Wolff-Parkinson-White syndrome can lead to cardiac arrest and sudden death. The most common arrhythmia associated with Wolff-Parkinson-White syndrome is called paroxysmal supraventricular tachycardia.
Complications of Wolff-Parkinson-White syndrome can occur at any age, although some individuals born with an accessory pathway in the heart never experience any health problems associated with the condition.
Wolff-Parkinson-White syndrome often occurs with other structural abnormalities of the heart or underlying heart disease. The most common heart defect associated with the condition is Ebstein anomaly, which affects the valve that allows blood to flow from the right atrium to the right ventricle (the tricuspid valve). Additionally, the heart rhythm problems associated with Wolff-Parkinson-White syndrome can be a component of several other genetic syndromes, including hypokalemic periodic paralysis (a condition that causes episodes of extreme muscle weakness), Pompe disease (a disorder characterised by the storage of excess glycogen), Danon disease (a condition that weakens the heart and skeletal muscles and causes intellectual disability), and tuberous sclerosis complex (a condition that results in the growth of noncancerous tumours in many parts of the body).
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Ventricular pre-excitation with arrhythmia
WPW Syndrome
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Further reading and references
- Wolff-Parkinson-White syndrome; MedlinePlus, National Library of Medicine.
- Al-Khatib SM, Pritchett EL. Clinical features of Wolff-Parkinson-White syndrome. Am Heart J. 1999 Sep;138(3 Pt 1):403-13. doi: 10.1016/s0002-8703(99)70140-7.
- Benson DW, Cohen MI. Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining. Cardiol Young. 2017 Jan;27(S1):S62-S67. doi: 10.1017/S1047951116002250.
- Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, Ahmad F, Lozado R, Shah G, Fananapazir L, Bachinski LL, Roberts R. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med. 2001 Jun 14;344(24):1823-31. doi: 10.1056/NEJM200106143442403.
- Light PE. Familial Wolff-Parkinson-White Syndrome: a disease of glycogen storage or ion channel dysfunction? J Cardiovasc Electrophysiol. 2006 May;17 Suppl 1:S158-S161. doi: 10.1111/j.1540-8167.2006.00399.x.
- Pediatric and Congenital Electrophysiology Society (PACES); Heart Rhythm Society (HRS); American College of Cardiology Foundation (ACCF); American Heart Association (AHA); American Academy of Pediatrics (AAP); Canadian Heart Rhythm Society (CHRS); Cohen MI, Triedman JK, Cannon BC, Davis AM, Drago F, Janousek J, Klein GJ, Law IH, Morady FJ, Paul T, Perry JC, Sanatani S, Tanel RE. PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS). Heart Rhythm. 2012 Jun;9(6):1006-24. doi: 10.1016/j.hrthm.2012.03.050. Epub 2012 May 10. No abstract available.
- Valderrama AL. Wolff-Parkinson-White syndrome: essentials for the primary care nurse practitioner. J Am Acad Nurse Pract. 2004 Sep;16(9):378-83. doi: 10.1111/j.1745-7599.2004.tb00387.x.
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