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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

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
Supraventricular tachycardia in infants, children and adolescents: diagnosis, and pharmacological and interventional therapy.
Paul T, Bertram H, Bökenkamp R, Hausdorf G
Paediatr Drugs 2000 May-Jun;2(3):171-81. doi: 10.2165/00128072-200002030-00002. PMID: 10937468
2:1 atrioventricular block during atrioventricular node reentrant tachycardia.
Man KC, Brinkman K, Bogun F, Knight B, Bahu M, Weiss R, Goyal R, Harvey M, Daoud EG, Strickberger SA, Morady F
J Am Coll Cardiol 1996 Dec;28(7):1770-4. doi: 10.1016/S0735-1097(96)00415-9. PMID: 8962565
Atrioventricular node reentry with 'smooth' AV node function curves: a different arrhythmia substrate?
Sheahan RG, Klein GJ, Yee R, Le Feuvre CA, Krahn AD
Circulation 1996 Mar 1;93(5):969-72. doi: 10.1161/01.cir.93.5.969. PMID: 8598088
Sinus and atrioventricular nodal function: Preoperative and early postoperative assessment in children.
Wolff GS, Kaiser G, Casta A, Pickoff AS, Mehta AV, Tamer D, Garica OL, Ferrer PL, Smith K, Gelband H
J Thorac Cardiovasc Surg 1982 Jan;83(1):141-8. PMID: 7054609

Diagnosis

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
Absent Left Main Coronary Artery and Separate Ostia of Left Coronary System in a Patient with Holt-Oram Syndrome and Sinus Node Dysfunction.
Aung TT, Roberto ES, Wase A
Am J Case Rep 2016 Feb 17;17:93-6. doi: 10.12659/ajcr.896474. PMID: 26882979Free 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
Supraventricular tachycardia in infants, children and adolescents: diagnosis, and pharmacological and interventional therapy.
Paul T, Bertram H, Bökenkamp R, Hausdorf G
Paediatr Drugs 2000 May-Jun;2(3):171-81. doi: 10.2165/00128072-200002030-00002. PMID: 10937468
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
Adverse effects of long-term right ventricular apical pacing and identification of patients at risk of atrial fibrillation and heart failure.
De Sisti A, Márquez MF, Tonet J, Bonny A, Frank R, Hidden-Lucet F
Pacing Clin Electrophysiol 2012 Aug;35(8):1035-43. Epub 2012 Mar 27 doi: 10.1111/j.1540-8159.2012.03371.x. PMID: 22452247
Supraventricular tachycardia in infants, children and adolescents: diagnosis, and pharmacological and interventional therapy.
Paul T, Bertram H, Bökenkamp R, Hausdorf G
Paediatr Drugs 2000 May-Jun;2(3):171-81. doi: 10.2165/00128072-200002030-00002. PMID: 10937468
Cardiac effects of calcium antagonists in systemic hypertension.
Michalewicz L, Messerli FH
Am J Cardiol 1997 May 22;79(10A):39-46; discussion 47-8. doi: 10.1016/s0002-9149(97)00271-3. PMID: 9186066
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

Prognosis

Ventriculoatrial conduction in patients without high-grade AV block: when is it present?
Elayi CS, Morales G, Butt M, Shah J, Ogunbayo G, Misumida N, Catanzaro J, Di Biase L, Natale A, Delisle B, Darrat Y
J Interv Card Electrophysiol 2020 Nov;59(2):393-400. Epub 2019 Dec 9 doi: 10.1007/s10840-019-00658-0. PMID: 31820272
Prevalence and predictors of ventriculo-atrial conduction in structurally normal hearts.
Patil G, Phadke M, Lokhandwala Y, Shaikh Z, Nathani P, Mahajan A
Indian Heart J 2017 May-Jun;69(3):316-318. Epub 2016 Dec 21 doi: 10.1016/j.ihj.2016.12.008. PMID: 28648420Free PMC Article
Just how stable are escape rhythms after atrioventricular junction ablation?
Arora R, Spatz E, Vijayaraman P, Rosengarten M, Gross J, Kim S, Fisher J, Ferrick KJ
Pacing Clin Electrophysiol 2010 Aug;33(8):939-44. Epub 2010 May 4 doi: 10.1111/j.1540-8159.2010.02756.x. PMID: 20487338
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
Atrioventricular canal malformation interpreted as secondary to reduced compression upon the developing heart.
Hutchins GM, Liebman L, Moore GW, Gharagozloo F
Am J Pathol 1979 Jun;95(3):579-95. PMID: 453326Free PMC Article

Clinical prediction guides

Similarities and differences in the arrangement of the atrioventricular conduction axis in the canine compared to the human heart.
de Almeida MC, Macías Y, Tretter JT, Sánchez-Quintana D, Cabrera JA, Spicer DE, Anderson RH
Heart Rhythm 2021 Nov;18(11):1990-1998. Epub 2021 Jul 30 doi: 10.1016/j.hrthm.2021.07.065. PMID: 34339846
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
Prevalence and predictors of ventriculo-atrial conduction in structurally normal hearts.
Patil G, Phadke M, Lokhandwala Y, Shaikh Z, Nathani P, Mahajan A
Indian Heart J 2017 May-Jun;69(3):316-318. Epub 2016 Dec 21 doi: 10.1016/j.ihj.2016.12.008. PMID: 28648420Free PMC Article
The atrioventricular junctions in Ebstein malformation.
Ho SY, Goltz D, McCarthy K, Cook AC, Connell MG, Smith A, Anderson RH
Heart 2000 Apr;83(4):444-9. doi: 10.1136/heart.83.4.444. PMID: 10722549Free PMC Article
2:1 atrioventricular block during atrioventricular node reentrant tachycardia.
Man KC, Brinkman K, Bogun F, Knight B, Bahu M, Weiss R, Goyal R, Harvey M, Daoud EG, Strickberger SA, Morady F
J Am Coll Cardiol 1996 Dec;28(7):1770-4. doi: 10.1016/S0735-1097(96)00415-9. PMID: 8962565

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