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Congenital atrioventricular dissociation

MedGen UID:
870839
•Concept ID:
C4025299
•
Congenital Abnormality
HPO: HP:0004752

Definition

A form of atrioventricular (AV) dissociation (i.e., the atria and the ventricles are under the control of two separate pacemakers) with congenital onset. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital atrioventricular dissociation

Conditions with this feature

Atrioventricular dissociation
MedGen UID:
2496
•Concept ID:
C0004331
•
Pathologic Function
Atrioventricular (AV) dissociation is present if the atria and the ventricles are under the control of two separate pacemakers. AV dissociation can occur in the absence of a primary AV conduction disturbance.
See: Condition Record
Atrioventricular dissociation

Professional guidelines

PubMed

Fetal Arrhythmia Diagnosis and Pharmacologic Management.
Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.
Kusumoto FM, Schoenfeld MH, Barrett C, Edgerton JR, Ellenbogen KA, Gold MR, Goldschlager NF, Hamilton RM, Joglar JA, Kim RJ, Lee R, Marine JE, McLeod CJ, Oken KR, Patton KK, Pellegrini CN, Selzman KA, Thompson A, Varosy PD
Circulation 2019 Aug 20;140(8):e382-e482. Epub 2018 Nov 6 doi: 10.1161/CIR.0000000000000628. PMID: 30586772
Evaluation and management of bradycardia in neonates and children.
Baruteau AE, Perry JC, Sanatani S, Horie M, Dubin AM
Eur J Pediatr 2016 Feb;175(2):151-61. Epub 2016 Jan 16 doi: 10.1007/s00431-015-2689-z. PMID: 26780751

Recent clinical studies

Etiology

Congenital Heart Block.
Steinberg L
Cardiol Clin 2023 Aug;41(3):399-410. doi: 10.1016/j.ccl.2023.03.002. PMID: 37321690
Congenital Heart Block.
Steinberg L
Card Electrophysiol Clin 2021 Dec;13(4):691-702. doi: 10.1016/j.ccep.2021.07.006. PMID: 34689896
Fetal dysrhythmias.
Carvalho JS
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:28-41. Epub 2019 Jan 9 doi: 10.1016/j.bpobgyn.2019.01.002. PMID: 30738635
Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.
Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, Combes N, Liberman L, McLeod CJ
Eur J Pediatr 2016 Sep;175(9):1235-1248. Epub 2016 Jun 28 doi: 10.1007/s00431-016-2748-0. PMID: 27351174Free PMC Article
Cardiac manifestations of systemic lupus erythematosus.
Miner JJ, Kim AH
Rheum Dis Clin North Am 2014 Feb;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003. PMID: 24268009

Diagnosis

Congenital Heart Block.
Steinberg L
Cardiol Clin 2023 Aug;41(3):399-410. doi: 10.1016/j.ccl.2023.03.002. PMID: 37321690
Congenital Heart Block.
Steinberg L
Card Electrophysiol Clin 2021 Dec;13(4):691-702. doi: 10.1016/j.ccep.2021.07.006. PMID: 34689896
Neonatal lupus erythematosus - practical guidelines.
Derdulska JM, Rudnicka L, Szykut-Badaczewska A, Mehrholz D, Nowicki RJ, Barańska-Rybak W, Wilkowska A
J Perinat Med 2021 Jun 25;49(5):529-538. Epub 2021 Jan 18 doi: 10.1515/jpm-2020-0543. PMID: 33470961
Fetal dysrhythmias.
Carvalho JS
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:28-41. Epub 2019 Jan 9 doi: 10.1016/j.bpobgyn.2019.01.002. PMID: 30738635
Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.
Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, Combes N, Liberman L, McLeod CJ
Eur J Pediatr 2016 Sep;175(9):1235-1248. Epub 2016 Jun 28 doi: 10.1007/s00431-016-2748-0. PMID: 27351174Free PMC Article

Therapy

Outcomes in congenital and childhood complete atrioventricular block: A meta-analysis.
Deshpande S, Shenthar J, Khanra D, Isath A, Banavalikar B, Reddy S, Krishnappa D, Khan H, Kella D, Padmanabhan D
J Cardiovasc Electrophysiol 2022 Mar;33(3):493-501. Epub 2022 Jan 27 doi: 10.1111/jce.15358. PMID: 35018695
Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers.
Izmirly P, Kim M, Friedman DM, Costedoat-Chalumeau N, Clancy R, Copel JA, Phoon CKL, Cuneo BF, Cohen RE, Robins K, Masson M, Wainwright BJ, Zahr N, Saxena A, Buyon JP
J Am Coll Cardiol 2020 Jul 21;76(3):292-302. doi: 10.1016/j.jacc.2020.05.045. PMID: 32674792Free PMC Article
Fetal dysrhythmias.
Carvalho JS
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:28-41. Epub 2019 Jan 9 doi: 10.1016/j.bpobgyn.2019.01.002. PMID: 30738635
2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.
Kusumoto FM, Schoenfeld MH, Barrett C, Edgerton JR, Ellenbogen KA, Gold MR, Goldschlager NF, Hamilton RM, Joglar JA, Kim RJ, Lee R, Marine JE, McLeod CJ, Oken KR, Patton KK, Pellegrini CN, Selzman KA, Thompson A, Varosy PD
Circulation 2019 Aug 20;140(8):e382-e482. Epub 2018 Nov 6 doi: 10.1161/CIR.0000000000000628. PMID: 30586772
Foetal therapy, what works? An overview.
Mellander M, Gardiner H
Cardiol Young 2014 Oct;24 Suppl 2:36-40. Epub 2014 Aug 28 doi: 10.1017/S1047951114001231. PMID: 25167080

Prognosis

Neonatal lupus erythematosus - practical guidelines.
Derdulska JM, Rudnicka L, Szykut-Badaczewska A, Mehrholz D, Nowicki RJ, Barańska-Rybak W, Wilkowska A
J Perinat Med 2021 Jun 25;49(5):529-538. Epub 2021 Jan 18 doi: 10.1515/jpm-2020-0543. PMID: 33470961
Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers.
Izmirly P, Kim M, Friedman DM, Costedoat-Chalumeau N, Clancy R, Copel JA, Phoon CKL, Cuneo BF, Cohen RE, Robins K, Masson M, Wainwright BJ, Zahr N, Saxena A, Buyon JP
J Am Coll Cardiol 2020 Jul 21;76(3):292-302. doi: 10.1016/j.jacc.2020.05.045. PMID: 32674792Free PMC Article
Fetal dysrhythmias.
Carvalho JS
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:28-41. Epub 2019 Jan 9 doi: 10.1016/j.bpobgyn.2019.01.002. PMID: 30738635
Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management.
Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, Combes N, Liberman L, McLeod CJ
Eur J Pediatr 2016 Sep;175(9):1235-1248. Epub 2016 Jun 28 doi: 10.1007/s00431-016-2748-0. PMID: 27351174Free PMC Article
Cardiac manifestations of systemic lupus erythematosus.
Miner JJ, Kim AH
Rheum Dis Clin North Am 2014 Feb;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003. PMID: 24268009

Clinical prediction guides

Fetal Arrhythmia Diagnosis and Pharmacologic Management.
Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
Pregnancy and Systemic Lupus Erythematosus.
Petri M
Best Pract Res Clin Obstet Gynaecol 2020 Apr;64:24-30. Epub 2019 Oct 8 doi: 10.1016/j.bpobgyn.2019.09.002. PMID: 31677989
The expanding phenotypes of cohesinopathies: one ring to rule them all!
Piché J, Van Vliet PP, Pucéat M, Andelfinger G
Cell Cycle 2019 Nov;18(21):2828-2848. Epub 2019 Sep 13 doi: 10.1080/15384101.2019.1658476. PMID: 31516082Free PMC Article
Evaluation and management of bradycardia in neonates and children.
Baruteau AE, Perry JC, Sanatani S, Horie M, Dubin AM
Eur J Pediatr 2016 Feb;175(2):151-61. Epub 2016 Jan 16 doi: 10.1007/s00431-015-2689-z. PMID: 26780751
Cardiac manifestations of systemic lupus erythematosus.
Miner JJ, Kim AH
Rheum Dis Clin North Am 2014 Feb;40(1):51-60. doi: 10.1016/j.rdc.2013.10.003. PMID: 24268009

Recent systematic reviews

Epicardial Versus Endocardial Pacing in Paediatric Patients with Atrioventricular Block or Sinus Node Dysfunction: A Systematic Review and Meta-analysis.
Patsiou V, Haidich AB, Baroutidou A, Giannopoulos A, Giannakoulas G
Pediatr Cardiol 2023 Dec;44(8):1641-1648. Epub 2023 Jul 22 doi: 10.1007/s00246-023-03213-x. PMID: 37480376Free PMC Article
Maternal and infant outcomes of pregnancy associated with anti-SSA/RO antibodies: a systematic review and meta-analysis.
Sheng X, Song X, Xiong Y, Ren T, Chang X, Wu J, Cao J, Cheng T, Wang M
Pediatr Rheumatol Online J 2023 Mar 4;21(1):22. doi: 10.1186/s12969-023-00803-0. PMID: 36870970Free PMC Article
An International, Multicentered, Evidence-Based Reappraisal of Genes Reported to Cause Congenital Long QT Syndrome.
Adler A, Novelli V, Amin AS, Abiusi E, Care M, Nannenberg EA, Feilotter H, Amenta S, Mazza D, Bikker H, Sturm AC, Garcia J, Ackerman MJ, Hershberger RE, Perez MV, Zareba W, Ware JS, Wilde AAM, Gollob MH
Circulation 2020 Feb 11;141(6):418-428. Epub 2020 Jan 27 doi: 10.1161/CIRCULATIONAHA.119.043132. PMID: 31983240Free PMC Article
Maternal steroid therapy for fetuses with second-degree immune-mediated congenital atrioventricular block: a systematic review and meta-analysis.
Ciardulli A, D'Antonio F, Magro-Malosso ER, Manzoli L, Anisman P, Saccone G, Berghella V
Acta Obstet Gynecol Scand 2018 Jul;97(7):787-794. Epub 2018 Apr 18 doi: 10.1111/aogs.13338. PMID: 29512819
Cardiac resynchronization therapy in congenital heart disease.
Khairy P, Fournier A, Thibault B, Dubuc M, Thérien J, Vobecky SJ
Int J Cardiol 2006 May 10;109(2):160-8. Epub 2005 Aug 10 doi: 10.1016/j.ijcard.2005.06.065. PMID: 16095734

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