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Ventricular preexcitation

MedGen UID:
107848
Concept ID:
C0559106
Disease or Syndrome
Synonyms: Pre-excitation syndrome; Preexcitation; Ventricular pre-excitation
SNOMED CT: Ventricular pre-excitation (195060002)
 
HPO: HP:0004309

Definition

An abnormality in which the cardiac ventricles depolarize too early as a result of an abnormality of cardiac conduction pathways such as an accessory pathway. [from HPO]

Conditions with this feature

Ebstein anomaly
MedGen UID:
4435
Concept ID:
C0013481
Congenital Abnormality
Ebstein anomaly is characterized by downward displacement of variable severity of the tricuspid valve into the right ventricle. The valve leaflets may be dysplastic, and a variable portion of the proximal part of the right ventricle is in continuity with the right atrium ('atrialized'), because of the abnormally positioned tricuspid valve. The severity of this defect includes a spectrum ranging from severe disturbance in fetal and neonatal life to virtually asymptomatic survival to adult life. Associated extracardiac anomalies in the setting of chromosomal or mendelian disorders occur in about 20% of patients with Ebstein anomaly. Nonsyndromic Ebstein anomaly can occur as a sporadic or a familial defect (summary by Digilio et al., 2011).
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).
Hypertrophic cardiomyopathy 6
MedGen UID:
331466
Concept ID:
C1833236
Disease or Syndrome
Mutations in the PRKAG2 gene (602743) give rise to a moderate, essentially heart-specific, nonlysosomal glycogenosis with clinical onset typically in late adolescence or in the third decade of life, ventricular pre-excitation predisposing to supraventricular arrhythmias, mild to severe cardiac hypertrophy, enhanced risk of sudden cardiac death in midlife, and autosomal dominant inheritance with full penetrance (summary by Burwinkel et al., 2005).

Professional guidelines

PubMed

Leoni L, Bronzetti G, Colonna D, Porcedda G, Rimini A, Silvetti MS; Area Pediatrica, Associazione Italiana di Aritmologia e Cardiostimolazione (AIAC)
J Cardiovasc Med (Hagerstown) 2023 Sep 1;24(9):589-601. Epub 2023 May 29 doi: 10.2459/JCM.0000000000001484. PMID: 37409656Free PMC Article
Chambers S, Jnah A, Newberry D
Adv Neonatal Care 2021 Jun 1;21(3):178-188. doi: 10.1097/ANC.0000000000000785. PMID: 32826411
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
Heart Rhythm 2012 Jun;9(6):1006-24. Epub 2012 May 10 doi: 10.1016/j.hrthm.2012.03.050. PMID: 22579340

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
Delise P, Sciarra L
Card Electrophysiol Clin 2020 Dec;12(4):519-525. Epub 2020 Sep 23 doi: 10.1016/j.ccep.2020.08.002. PMID: 33162000
Brembilla-Perrot B, Girerd N, Sellal JM
Pacing Clin Electrophysiol 2018 Jul;41(7):839-844. Epub 2018 Jun 8 doi: 10.1111/pace.13367. PMID: 29754436
Kautzner J, Peichl P
Int J Cardiol 2017 Jun 15;237:29-33. Epub 2017 Apr 1 doi: 10.1016/j.ijcard.2017.03.135. PMID: 28433554
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
Pérez-Riera AR, Barbosa-Barros R, Daminello-Raimundo R, de Abreu LC, Nikus K
Curr Cardiol Rev 2021;17(1):50-59. doi: 10.2174/1573403X16666200810105504. PMID: 32778036Free PMC Article
Marazzato J, Marazzi R, Doni LA, Angeli F, Bagliani G, Leonelli FM, De Ponti R
Card Electrophysiol Clin 2020 Dec;12(4):505-518. Epub 2020 Sep 26 doi: 10.1016/j.ccep.2020.08.001. PMID: 33161999
Bagliani G, De Ponti R, Notaristefano F, Cavallini C, Padeletti M, Leonelli FM
Card Electrophysiol Clin 2020 Dec;12(4):447-464. doi: 10.1016/j.ccep.2020.08.011. PMID: 33161995
Hanna EB, Johnson CJ, Glancy DL
Am J Cardiol 2018 Jan 15;121(2):275-276. Epub 2017 Oct 19 doi: 10.1016/j.amjcard.2017.09.035. PMID: 29132651

Therapy

Zhang Y, Jiang H, Cui J, Li MT, Zhou HM, Li XM
Circ Arrhythm Electrophysiol 2023 Apr;16(4):e011569. Epub 2023 Mar 9 doi: 10.1161/CIRCEP.122.011569. PMID: 36891895
Leroux J, Strik M, Ramirez FD, Racine HP, Ploux S, Sacristan B, Chabaneix-Thomas J, Jalal Z, Thambo JB, Bordachar P
J Pediatr 2023 Feb;253:40-45.e1. Epub 2022 Sep 14 doi: 10.1016/j.jpeds.2022.09.010. PMID: 36113637
Chiale PA, Etcheverry D, Pastori JD, Fernandez PA, Garro HA, González MD, Elizari MV
Curr Cardiol Rev 2014 Aug;10(3):190-201. doi: 10.2174/1573403x10666140514102021. PMID: 24827802Free PMC Article
Hermosura T, Bradshaw WT
Neonatal Netw 2010 Jul-Aug;29(4):215-23. doi: 10.1891/0730-0832.29.4.215. PMID: 20630836
Lowenstein SR, Harken AH
J Emerg Med 1987 Nov-Dec;5(6):519-31. doi: 10.1016/0736-4679(87)90216-2. PMID: 3323302

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
Brembilla-Perrot B, Girerd N, Sellal JM
Pacing Clin Electrophysiol 2018 Jul;41(7):839-844. Epub 2018 Jun 8 doi: 10.1111/pace.13367. PMID: 29754436
Chiale PA, Etcheverry D, Pastori JD, Fernandez PA, Garro HA, González MD, Elizari MV
Curr Cardiol Rev 2014 Aug;10(3):190-201. doi: 10.2174/1573403x10666140514102021. PMID: 24827802Free PMC Article
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875
Ferrer MI
Am J Med 1977 May;62(5):715-30. doi: 10.1016/0002-9343(77)90875-0. PMID: 324276

Clinical prediction guides

Leoni L, Bronzetti G, Colonna D, Porcedda G, Rimini A, Silvetti MS; Area Pediatrica, Associazione Italiana di Aritmologia e Cardiostimolazione (AIAC)
J Cardiovasc Med (Hagerstown) 2023 Sep 1;24(9):589-601. Epub 2023 May 29 doi: 10.2459/JCM.0000000000001484. PMID: 37409656Free PMC Article
Pérez-Riera AR, Barbosa-Barros R, Daminello-Raimundo R, de Abreu LC, Nikus K
Curr Cardiol Rev 2021;17(1):50-59. doi: 10.2174/1573403X16666200810105504. PMID: 32778036Free PMC Article
Pappone C, Radinovic A, Santinelli V
Curr Pharm Des 2008;14(8):762-5. doi: 10.2174/138161208784007662. PMID: 18393875
Dovgalyuk J, Holstege C, Mattu A, Brady WJ
Am J Emerg Med 2007 Jul;25(6):688-701. doi: 10.1016/j.ajem.2006.12.016. PMID: 17606095
Sternick EB, Oliva A, Magalhães LP, Gerken LM, Hong K, Santana O, Brugada P, Brugada J, Brugada R
J Cardiovasc Electrophysiol 2006 Jul;17(7):724-32. doi: 10.1111/j.1540-8167.2006.00485.x. PMID: 16836667

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