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Ventricular preexcitation with multiple accessory pathways

MedGen UID:
870556
Concept ID:
C4025004
Finding
HPO: HP:0006684

Definition

A form of ventricular preexcitation due to the presence of multiple accessory pathways for cardiac conduction. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVentricular preexcitation with multiple accessory pathways

Conditions with this feature

Wolff-Parkinson-White pattern
MedGen UID:
12162
Concept ID:
C0043202
Disease or Syndrome
Wolff-Parkinson-White syndrome is a condition characterized by abnormal electrical pathways in the heart that cause a disruption of the heart's normal rhythm (arrhythmia).\n\nThe 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.\n\nPeople 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.\n\nComplications 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.\n\nWolff-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 characterized 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 tumors in many parts of the body).

Professional guidelines

PubMed

Jebberi Z, Marazzato J, De Ponti R, Bagliani G, Leonelli FM, Boveda S
Card Electrophysiol Clin 2019 Jun;11(2):333-344. Epub 2019 Apr 12 doi: 10.1016/j.ccep.2019.02.004. PMID: 31084854

Recent clinical studies

Etiology

Przybylski R, Saravu Vijayashankar S, O'Leary ET, Hylind RJ, Noon J, Dionne A, DeWitt ES, Bezzerides VJ, Abrams DJ
Circ Arrhythm Electrophysiol 2023 Nov;16(11):e012191. Epub 2023 Oct 25 doi: 10.1161/CIRCEP.123.012191. PMID: 37877314Free PMC Article
Robles AG, Palamà Z, Pernat A, Gianfrancesco D, Bartolomucci F, Scarà A, Borrelli A, De Ruvo E, Calò L, Penco M, Romano S, Sciarra L
Pacing Clin Electrophysiol 2023 Sep;46(9):1049-1055. Epub 2023 Aug 1 doi: 10.1111/pace.14798. PMID: 37527153
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Jebberi Z, Marazzato J, De Ponti R, Bagliani G, Leonelli FM, Boveda S
Card Electrophysiol Clin 2019 Jun;11(2):333-344. Epub 2019 Apr 12 doi: 10.1016/j.ccep.2019.02.004. PMID: 31084854
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875

Diagnosis

Przybylski R, Saravu Vijayashankar S, O'Leary ET, Hylind RJ, Noon J, Dionne A, DeWitt ES, Bezzerides VJ, Abrams DJ
Circ Arrhythm Electrophysiol 2023 Nov;16(11):e012191. Epub 2023 Oct 25 doi: 10.1161/CIRCEP.123.012191. PMID: 37877314Free PMC Article
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Jebberi Z, Marazzato J, De Ponti R, Bagliani G, Leonelli FM, Boveda S
Card Electrophysiol Clin 2019 Jun;11(2):333-344. Epub 2019 Apr 12 doi: 10.1016/j.ccep.2019.02.004. PMID: 31084854
Lima da Silva G, Cortez-Dias N, Bernardes A, Sousa J
Rev Port Cardiol (Engl Ed) 2018 Mar;37(3):265.e1-265.e5. Epub 2018 Apr 2 doi: 10.1016/j.repc.2017.01.008. PMID: 29622376
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875

Therapy

Capocci S, Rubino F, Setti M, Butturini C, Tomasi L, Bolzan B, Piccoli A, Ribichini FL, Mugnai G
J Electrocardiol 2023 May-Jun;78:21-24. Epub 2023 Jan 30 doi: 10.1016/j.jelectrocard.2023.01.011. PMID: 36731165
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Obel OA, Camm AJ
Eur Heart J 1998 May;19 Suppl E:E13-24, E50-1. PMID: 9717020
Montenero AS, Crea F, Bendini MG, Bellocci F, Zecchi P
J Interv Cardiol 1995 Dec;8(6 Suppl):806-12. doi: 10.1111/j.1540-8183.1995.tb00934.x. PMID: 10159772
Tai CT, Chen SA, Chiang CE, Wu TJ, Cheng CC, Chiou CW, Lee SH, Ueng KC, Wang SP, Chiang BN
Jpn Heart J 1995 May;36(3):305-17. doi: 10.1536/ihj.36.305. PMID: 7650838

Prognosis

Przybylski R, Saravu Vijayashankar S, O'Leary ET, Hylind RJ, Noon J, Dionne A, DeWitt ES, Bezzerides VJ, Abrams DJ
Circ Arrhythm Electrophysiol 2023 Nov;16(11):e012191. Epub 2023 Oct 25 doi: 10.1161/CIRCEP.123.012191. PMID: 37877314Free PMC Article
Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Orczykowski M, Walczak F, Derejko P, Bodalski R, Urbanek P, Zakrzewska-Koperska J, Przybylski A, Kępski R, Jedynak Z, Maryniak A, Miszczak-Knecht M, Bieganowska K, Szufladowicz E, Biederman A, Bilińska M, Szumowski L
Int J Cardiol 2013 Jul 31;167(2):525-30. Epub 2012 Feb 21 doi: 10.1016/j.ijcard.2012.01.076. PMID: 22357421
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875
Montenero AS, Crea F, Bendini MG, Bellocci F, Zecchi P
J Interv Cardiol 1995 Dec;8(6 Suppl):806-12. doi: 10.1111/j.1540-8183.1995.tb00934.x. PMID: 10159772

Clinical prediction guides

Khairy P
Can J Cardiol 2019 Dec;35(12):1698-1707. Epub 2019 Jul 16 doi: 10.1016/j.cjca.2019.07.009. PMID: 31703824
Szili Torok T, DE Vries LJ, Özcan EE, Hasdemir C, Kis Z, Kardos A, Géczy T, Kovacs I, Benedek I, Oosterwerff E, Hendriks AA, Khan M, Yap SC
J Cardiovasc Electrophysiol 2017 Jan;28(1):78-84. Epub 2016 Oct 6 doi: 10.1111/jce.13098. PMID: 27625076
Orczykowski M, Walczak F, Derejko P, Bodalski R, Urbanek P, Zakrzewska-Koperska J, Przybylski A, Kępski R, Jedynak Z, Maryniak A, Miszczak-Knecht M, Bieganowska K, Szufladowicz E, Biederman A, Bilińska M, Szumowski L
Int J Cardiol 2013 Jul 31;167(2):525-30. Epub 2012 Feb 21 doi: 10.1016/j.ijcard.2012.01.076. PMID: 22357421
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875
Montenero AS, Crea F, Bendini MG, Bellocci F, Zecchi P
J Interv Cardiol 1995 Dec;8(6 Suppl):806-12. doi: 10.1111/j.1540-8183.1995.tb00934.x. PMID: 10159772

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