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Arrhythmogenic Syncope

MedGen UID:
1708567
•Concept ID:
C5238636
•
Sign or Symptom

Definition

A condition in which syncope occurs due to an arrhythmia. (ACC-AHA) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVArrhythmogenic Syncope

Professional guidelines

PubMed

Current investigation and management of patients with syncope: results of the European Heart Rhythm Association survey.
Dagres N, Bongiorni MG, Dobreanu D, Madrid A, Svendsen JH, Blomström-Lundqvist C; conducted by the Scientific Initiatives Committee, European Heart Rhythm Association
Europace 2013 Dec;15(12):1812-5. doi: 10.1093/europace/eut354. PMID: 24280765
Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy.
Moss AJ
Pacing Clin Electrophysiol 1997 Aug;20(8 Pt 2):2058-60. doi: 10.1111/j.1540-8159.1997.tb03627.x. PMID: 9272508

Recent clinical studies

Etiology

Utilizing median and maximum QTc values improves prediction of breakthrough cardiac events in pediatric long QT syndrome.
Rohatgi RK, Tseng AS, Sugrue AM, Lee AT, Scott CG, Wackel PL, Cannon BC, Bos JM, Ackerman MJ
J Cardiovasc Electrophysiol 2024 Jul;35(7):1370-1381. Epub 2024 May 9 doi: 10.1111/jce.16293. PMID: 38725227
Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.
Ito Y, Sakaguchi H, Tsuda E, Kurosaki K
J Cardiol 2024 Jun;83(6):407-414. Epub 2023 Dec 2 doi: 10.1016/j.jjcc.2023.11.009. PMID: 38043708
The Evolving Role of Insertable Cardiac Monitors in Patients with Congenital Heart Disease.
Beach CM, Richardson C, Paul T
Card Electrophysiol Clin 2023 Dec;15(4):413-420. Epub 2023 Jul 16 doi: 10.1016/j.ccep.2023.06.001. PMID: 37865515
Arrhythmogenic syncope leading to cardiac rhythm management procedures during COVID-19 lockdown.
Russo V, Pafundi PC, Rapacciuolo A, D'Andrea A, de Devitiis M, Volpicelli M, Ruocco A, Nigro G, D'Onofrio A
Expert Rev Med Devices 2020 Nov;17(11):1207-1210. Epub 2020 Nov 10 doi: 10.1080/17434440.2020.1841632. PMID: 33090884
A prospective study of the standardized diagnostic evaluation of syncope.
Mitro P, Kirsch P, Valočik G, Murín P
Europace 2011 Apr;13(4):566-71. Epub 2011 Feb 11 doi: 10.1093/europace/eur014. PMID: 21317150

Diagnosis

Utilizing median and maximum QTc values improves prediction of breakthrough cardiac events in pediatric long QT syndrome.
Rohatgi RK, Tseng AS, Sugrue AM, Lee AT, Scott CG, Wackel PL, Cannon BC, Bos JM, Ackerman MJ
J Cardiovasc Electrophysiol 2024 Jul;35(7):1370-1381. Epub 2024 May 9 doi: 10.1111/jce.16293. PMID: 38725227
Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.
Ito Y, Sakaguchi H, Tsuda E, Kurosaki K
J Cardiol 2024 Jun;83(6):407-414. Epub 2023 Dec 2 doi: 10.1016/j.jjcc.2023.11.009. PMID: 38043708
The Evolving Role of Insertable Cardiac Monitors in Patients with Congenital Heart Disease.
Beach CM, Richardson C, Paul T
Card Electrophysiol Clin 2023 Dec;15(4):413-420. Epub 2023 Jul 16 doi: 10.1016/j.ccep.2023.06.001. PMID: 37865515
Low rate of life-threatening events and limitations in predicting invasive and noninvasive markers of symptoms in a cohort of type 1 Brugada syndrome patients: Data and insights from the GenBra registry.
Sacilotto L, Scanavacca MI, Olivetti N, Lemes C, Pessente GD, Wulkan F, Hachul DT, Krieger JE, Pereira AC, Darrieux FCC
J Cardiovasc Electrophysiol 2020 Nov;31(11):2920-2928. Epub 2020 Oct 5 doi: 10.1111/jce.14732. PMID: 32870538
A prospective study of the standardized diagnostic evaluation of syncope.
Mitro P, Kirsch P, Valočik G, Murín P
Europace 2011 Apr;13(4):566-71. Epub 2011 Feb 11 doi: 10.1093/europace/eur014. PMID: 21317150

Therapy

Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.
Ito Y, Sakaguchi H, Tsuda E, Kurosaki K
J Cardiol 2024 Jun;83(6):407-414. Epub 2023 Dec 2 doi: 10.1016/j.jjcc.2023.11.009. PMID: 38043708
Rationale and design of the EU-CERT-ICD prospective study: comparative effectiveness of prophylactic ICD implantation.
Zabel M, Sticherling C, Willems R, Lubinski A, Bauer A, Bergau L, Braunschweig F, Brugada J, Brusich S, Conen D, Cygankiewicz I, Flevari P, Taborsky M, Hansen J, Hasenfuß G, Hatala R, Huikuri HV, Iovev S, Kääb S, Kaliska G, Kasprzak JD, Lüthje L, Malik M, Novotny T, Pavlović N, Schmidt G, Shalganov T, Sritharan R, Schlögl S, Szavits Nossan J, Traykov V, Tuinenburg AE, Velchev V, Vos MA, Willich SN, Friede T, Svendsen JH, Merkely B; EU-CERT-ICD Study Investigators
ESC Heart Fail 2019 Feb;6(1):182-193. Epub 2018 Oct 9 doi: 10.1002/ehf2.12367. PMID: 30299600Free PMC Article
Syncope in high-risk cardiomyopathy patients with implantable defibrillators: frequency, risk factors, mechanisms, and association with mortality: results from the multicenter automatic defibrillator implantation trial-reduce inappropriate therapy (MADIT-RIT) study.
Ruwald MH, Okumura K, Kimura T, Aonuma K, Shoda M, Kutyifa V, Ruwald AC, McNitt S, Zareba W, Moss AJ
Circulation 2014 Feb 4;129(5):545-52. Epub 2013 Nov 7 doi: 10.1161/CIRCULATIONAHA.113.004196. PMID: 24201303
Left cardiac sympathetic denervation in long QT syndrome: analysis of therapeutic nonresponders.
Bos JM, Bos KM, Johnson JN, Moir C, Ackerman MJ
Circ Arrhythm Electrophysiol 2013 Aug;6(4):705-11. Epub 2013 May 31 doi: 10.1161/CIRCEP.113.000102. PMID: 23728945
Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy.
Moss AJ
Pacing Clin Electrophysiol 1997 Aug;20(8 Pt 2):2058-60. doi: 10.1111/j.1540-8159.1997.tb03627.x. PMID: 9272508

Prognosis

Utilizing median and maximum QTc values improves prediction of breakthrough cardiac events in pediatric long QT syndrome.
Rohatgi RK, Tseng AS, Sugrue AM, Lee AT, Scott CG, Wackel PL, Cannon BC, Bos JM, Ackerman MJ
J Cardiovasc Electrophysiol 2024 Jul;35(7):1370-1381. Epub 2024 May 9 doi: 10.1111/jce.16293. PMID: 38725227
Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.
Ito Y, Sakaguchi H, Tsuda E, Kurosaki K
J Cardiol 2024 Jun;83(6):407-414. Epub 2023 Dec 2 doi: 10.1016/j.jjcc.2023.11.009. PMID: 38043708
Low rate of life-threatening events and limitations in predicting invasive and noninvasive markers of symptoms in a cohort of type 1 Brugada syndrome patients: Data and insights from the GenBra registry.
Sacilotto L, Scanavacca MI, Olivetti N, Lemes C, Pessente GD, Wulkan F, Hachul DT, Krieger JE, Pereira AC, Darrieux FCC
J Cardiovasc Electrophysiol 2020 Nov;31(11):2920-2928. Epub 2020 Oct 5 doi: 10.1111/jce.14732. PMID: 32870538
Current investigation and management of patients with syncope: results of the European Heart Rhythm Association survey.
Dagres N, Bongiorni MG, Dobreanu D, Madrid A, Svendsen JH, Blomström-Lundqvist C; conducted by the Scientific Initiatives Committee, European Heart Rhythm Association
Europace 2013 Dec;15(12):1812-5. doi: 10.1093/europace/eut354. PMID: 24280765
A prospective study of the standardized diagnostic evaluation of syncope.
Mitro P, Kirsch P, Valočik G, Murín P
Europace 2011 Apr;13(4):566-71. Epub 2011 Feb 11 doi: 10.1093/europace/eur014. PMID: 21317150

Clinical prediction guides

Utilizing median and maximum QTc values improves prediction of breakthrough cardiac events in pediatric long QT syndrome.
Rohatgi RK, Tseng AS, Sugrue AM, Lee AT, Scott CG, Wackel PL, Cannon BC, Bos JM, Ackerman MJ
J Cardiovasc Electrophysiol 2024 Jul;35(7):1370-1381. Epub 2024 May 9 doi: 10.1111/jce.16293. PMID: 38725227
Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.
Ito Y, Sakaguchi H, Tsuda E, Kurosaki K
J Cardiol 2024 Jun;83(6):407-414. Epub 2023 Dec 2 doi: 10.1016/j.jjcc.2023.11.009. PMID: 38043708
Arrhythmogenic syncope leading to cardiac rhythm management procedures during COVID-19 lockdown.
Russo V, Pafundi PC, Rapacciuolo A, D'Andrea A, de Devitiis M, Volpicelli M, Ruocco A, Nigro G, D'Onofrio A
Expert Rev Med Devices 2020 Nov;17(11):1207-1210. Epub 2020 Nov 10 doi: 10.1080/17434440.2020.1841632. PMID: 33090884
Rationale and design of the EU-CERT-ICD prospective study: comparative effectiveness of prophylactic ICD implantation.
Zabel M, Sticherling C, Willems R, Lubinski A, Bauer A, Bergau L, Braunschweig F, Brugada J, Brusich S, Conen D, Cygankiewicz I, Flevari P, Taborsky M, Hansen J, Hasenfuß G, Hatala R, Huikuri HV, Iovev S, Kääb S, Kaliska G, Kasprzak JD, Lüthje L, Malik M, Novotny T, Pavlović N, Schmidt G, Shalganov T, Sritharan R, Schlögl S, Szavits Nossan J, Traykov V, Tuinenburg AE, Velchev V, Vos MA, Willich SN, Friede T, Svendsen JH, Merkely B; EU-CERT-ICD Study Investigators
ESC Heart Fail 2019 Feb;6(1):182-193. Epub 2018 Oct 9 doi: 10.1002/ehf2.12367. PMID: 30299600Free PMC Article
Multiple clinical profiles of families with the short QT syndrome.
Akdis D, Saguner AM, Medeiros-Domingo A, Schaller A, Balmer C, Steffel J, Brunckhorst C, Duru F
Europace 2018 Jun 1;20(FI1):f113-f121. doi: 10.1093/europace/eux186. PMID: 29016797

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