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Absent atrioventricular node

MedGen UID:
870558
•Concept ID:
C4025006
•
Finding
HPO: HP:0006681

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbsent atrioventricular node

Conditions with this feature

Congenital heart block
MedGen UID:
57432
•Concept ID:
C0149530
•
Disease or Syndrome
Congenital heart block (CHB) is a rare disorder of atrioventricular conduction, characterized by absence of conduction of atrial impulses to the ventricles with slower ventricular rhythm (atrioventricular dissociation). CHB can occur in association with immunological evidence of maternal connective disease (autoimmune CHD), fetal structural CHD or can be idiopathic.
See: Condition Record
Congenital heart block

Professional guidelines

PubMed

Role of invasive electrophysiologic testing in the evaluation and management of adult patients with focal junctional tachycardia.
Hamdan MH, Badhwar N, Scheinman MM
Card Electrophysiol Rev 2002 Dec;6(4):431-5. doi: 10.1023/a:1021144610712. PMID: 12438824
Management of atrial fibrillation in the setting of heart failure.
Crijns HJ, Van den Berg MP, Van Gelder IC, Van Veldhuisen DJ
Eur Heart J 1997 May;18 Suppl C:C45-9. doi: 10.1093/eurheartj/18.suppl_c.45. PMID: 9152675

Recent clinical studies

Etiology

Role of invasive electrophysiologic testing in the evaluation and management of adult patients with focal junctional tachycardia.
Hamdan MH, Badhwar N, Scheinman MM
Card Electrophysiol Rev 2002 Dec;6(4):431-5. doi: 10.1023/a:1021144610712. PMID: 12438824
Catheter ablation of Mahaim fibers with preservation of atrioventricular nodal conduction.
Haissaguerre M, Warin JF, Le Metayer P, Maraud L, De Roy L, Montserrat P, Massiere JP
Circulation 1990 Aug;82(2):418-27. doi: 10.1161/01.cir.82.2.418. PMID: 2115408
Fulguration for AV nodal tachycardia: results in 42 patients with a mean follow-up of 23 months.
Haissaguerre M, Warin JF, D'Ivernois C, Le Métayer PH, Montserrat P
Pacing Clin Electrophysiol 1990 Dec;13(12 Pt 2):2000-7. doi: 10.1111/j.1540-8159.1990.tb06931.x. PMID: 1704582
Conduction tissue anomalies in absence of the right superior caval vein.
Lenox CC, Hashida Y, Anderson RH, Hubbard JD
Int J Cardiol 1985 Jul;8(3):251-60. doi: 10.1016/0167-5273(85)90216-5. PMID: 4018916
Effect of atrioventricular sequential pacing in patients with no ventriculoatrial conduction.
Mahmud R, Denker S, Lehmann MH, Akhtar M
J Am Coll Cardiol 1984 Aug;4(2):273-7. doi: 10.1016/s0735-1097(84)80213-2. PMID: 6736468

Diagnosis

Adenosine and Preexcitation Variants: Reappraisal of Electrocardiographic Changes.
Ali H, Lupo P, Foresti S, De Ambroggi G, Epicoco G, Fundaliotis A, Cappato R
Ann Noninvasive Electrocardiol 2016 Jul;21(4):420-4. Epub 2016 Mar 10 doi: 10.1111/anec.12348. PMID: 26969821Free PMC Article
Arrhythmias in children.
Jat KR, Lodha R, Kabra SK
Indian J Pediatr 2011 Feb;78(2):211-8. Epub 2010 Oct 27 doi: 10.1007/s12098-010-0276-x. PMID: 20978872
Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway.
Iturralde P, Nava S, Sálica G, Medeiros A, Márquez MF, Colin L, Victoria D, De Micheli A, Gonzalez MD
J Cardiovasc Electrophysiol 2006 Dec;17(12):1332-6. doi: 10.1111/j.1540-8167.2006.00617.x. PMID: 17239096
Role of invasive electrophysiologic testing in the evaluation and management of adult patients with focal junctional tachycardia.
Hamdan MH, Badhwar N, Scheinman MM
Card Electrophysiol Rev 2002 Dec;6(4):431-5. doi: 10.1023/a:1021144610712. PMID: 12438824
Electrophysiological factors of left bundle-branch block.
Barrett PA, Yamaguchi I, Jordan JL, Mandel WJ
Br Heart J 1981 May;45(5):594-601. doi: 10.1136/hrt.45.5.594. PMID: 7236466Free PMC Article

Therapy

Congenital heart block: Pace earlier (Childhood) than later (Adulthood).
Manolis AA, Manolis TA, Melita H, Manolis AS
Trends Cardiovasc Med 2020 Jul;30(5):275-286. Epub 2019 Jun 20 doi: 10.1016/j.tcm.2019.06.006. PMID: 31262557
Adenosine and Preexcitation Variants: Reappraisal of Electrocardiographic Changes.
Ali H, Lupo P, Foresti S, De Ambroggi G, Epicoco G, Fundaliotis A, Cappato R
Ann Noninvasive Electrocardiol 2016 Jul;21(4):420-4. Epub 2016 Mar 10 doi: 10.1111/anec.12348. PMID: 26969821Free PMC Article
Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway.
Iturralde P, Nava S, Sálica G, Medeiros A, Márquez MF, Colin L, Victoria D, De Micheli A, Gonzalez MD
J Cardiovasc Electrophysiol 2006 Dec;17(12):1332-6. doi: 10.1111/j.1540-8167.2006.00617.x. PMID: 17239096
Role of invasive electrophysiologic testing in the evaluation and management of adult patients with focal junctional tachycardia.
Hamdan MH, Badhwar N, Scheinman MM
Card Electrophysiol Rev 2002 Dec;6(4):431-5. doi: 10.1023/a:1021144610712. PMID: 12438824
Initial and long-term evaluation of escape rhythm after radiofrequency ablation of the AV junction in 50 patients.
Piot O, Sebag C, Lavergne T, Ollitrault J, Johnson N, Dinanian S, Le Heuzey JY, Guize L, Motté G
Pacing Clin Electrophysiol 1996 Nov;19(11 Pt 2):1988-92. doi: 10.1111/j.1540-8159.1996.tb03267.x. PMID: 8945083

Prognosis

Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway.
Iturralde P, Nava S, Sálica G, Medeiros A, Márquez MF, Colin L, Victoria D, De Micheli A, Gonzalez MD
J Cardiovasc Electrophysiol 2006 Dec;17(12):1332-6. doi: 10.1111/j.1540-8167.2006.00617.x. PMID: 17239096
Role of invasive electrophysiologic testing in the evaluation and management of adult patients with focal junctional tachycardia.
Hamdan MH, Badhwar N, Scheinman MM
Card Electrophysiol Rev 2002 Dec;6(4):431-5. doi: 10.1023/a:1021144610712. PMID: 12438824
Initial and long-term evaluation of escape rhythm after radiofrequency ablation of the AV junction in 50 patients.
Piot O, Sebag C, Lavergne T, Ollitrault J, Johnson N, Dinanian S, Le Heuzey JY, Guize L, Motté G
Pacing Clin Electrophysiol 1996 Nov;19(11 Pt 2):1988-92. doi: 10.1111/j.1540-8159.1996.tb03267.x. PMID: 8945083
Catheter ablation of Mahaim fibers with preservation of atrioventricular nodal conduction.
Haissaguerre M, Warin JF, Le Metayer P, Maraud L, De Roy L, Montserrat P, Massiere JP
Circulation 1990 Aug;82(2):418-27. doi: 10.1161/01.cir.82.2.418. PMID: 2115408
Fulguration for AV nodal tachycardia: results in 42 patients with a mean follow-up of 23 months.
Haissaguerre M, Warin JF, D'Ivernois C, Le Métayer PH, Montserrat P
Pacing Clin Electrophysiol 1990 Dec;13(12 Pt 2):2000-7. doi: 10.1111/j.1540-8159.1990.tb06931.x. PMID: 1704582

Clinical prediction guides

Ventricular extrasystoles with interpolation or postponed compensatory pause during atrial fibrillation: fact or fiction?
Carbone V, Oreto L, Oreto G
Ann Noninvasive Electrocardiol 2013 Jan;18(1):90-4. Epub 2012 Aug 13 doi: 10.1111/j.1542-474X.2012.00541.x. PMID: 23347032Free PMC Article
Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway.
Iturralde P, Nava S, Sálica G, Medeiros A, Márquez MF, Colin L, Victoria D, De Micheli A, Gonzalez MD
J Cardiovasc Electrophysiol 2006 Dec;17(12):1332-6. doi: 10.1111/j.1540-8167.2006.00617.x. PMID: 17239096
Initial and long-term evaluation of escape rhythm after radiofrequency ablation of the AV junction in 50 patients.
Piot O, Sebag C, Lavergne T, Ollitrault J, Johnson N, Dinanian S, Le Heuzey JY, Guize L, Motté G
Pacing Clin Electrophysiol 1996 Nov;19(11 Pt 2):1988-92. doi: 10.1111/j.1540-8159.1996.tb03267.x. PMID: 8945083
Possible role of a ventricular conduction disturbance in the electrogenesis of the ECG-VCG signs of myocardial infarction.
Piccolo E, Delise P, Raviele A, D'Este D, Lucangeli F, Pascotto P, Dainese F, Di Pede F
J Electrocardiol 1983 Oct;16(4):385-96. doi: 10.1016/s0022-0736(83)80089-2. PMID: 6644220
Anatomical study of truncus arteriousus communis with embryological and surgical considerations.
Thiene G, Bortolotti U, Gallucci V, Terribile V, Pellegrino PA
Br Heart J 1976 Nov;38(11):1109-23. doi: 10.1136/hrt.38.11.1109. PMID: 1008953Free PMC Article

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