Congenital complete atrioventricular heart block

MedGen UID:: 473062
•Concept ID:: C0340496
•: Pathologic Function

Synonym:	Complete atrioventricular block, congenital
SNOMED CT:	Congenital complete atrioventricular heart block (204383001)

Definition

A disorder characterized by an electrocardiographic finding of a complete atrioventricular block that manifests during fetal life or soon after birth. It may be isolated or associated with other congenital heart defects. Isolated congenital complete atrioventricular block may be associated with maternal connective tissue disorders. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVCongenital complete atrioventricular heart block

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Congenital anomaly of cardiovascular system
        Congenital heart disease
        Congenital complete atrioventricular heart block

Professional guidelines

PubMed

Criteria for Early Pacemaker Implantation in Patients With Postoperative Heart Block After Congenital Heart Surgery.

Duong SQ, Shi Y, Giacone H, Navarre BM, Gal DB, Han B, Sganga D, Ma M, Reddy CD, Shin AY, Kwiatkowski DM, Dubin AM, Scheinker D, Algaze CA
Circ Arrhythm Electrophysiol 2022 Nov;15(11):e011145. Epub 2022 Oct 28 doi: 10.1161/CIRCEP.122.011145. PMID: 36306332

Pathophysiology, clinical course, and management of congenital complete atrioventricular block.

Bordachar P, Zachary W, Ploux S, Labrousse L, Haissaguerre M, Thambo JB
Heart Rhythm 2013 May;10(5):760-6. Epub 2012 Dec 28 doi: 10.1016/j.hrthm.2012.12.030. PMID: 23276818

Detection and management of life threatening arrhythmias in the perinatal period.

Skinner JR, Sharland G
Early Hum Dev 2008 Mar;84(3):161-72. Epub 2008 Mar 20 doi: 10.1016/j.earlhumdev.2008.01.010. PMID: 18358642

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Recent clinical studies

Etiology

Prenatal anti-Ro antibody exposure, congenital complete atrioventricular heart block, and high-dose steroid therapy: impact on neurocognitive outcome in school-age children.

Kelly EN, Sananes R, Chiu-Man C, Silverman ED, Jaeggi E
Arthritis Rheumatol 2014 Aug;66(8):2290-6. doi: 10.1002/art.38675. PMID: 24756962

Congenital complete atrioventricular heart block. A 25 year follow-up study.

Corne RA, Mathewson FA
Am J Cardiol 1972 Mar;29(3):412-5. doi: 10.1016/0002-9149(72)90540-1. PMID: 5060814

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Diagnosis

Congenital Complete Atrioventricular Heart Block in a Pregnant Woman with Sjögren Syndrome: Prenatal Care Follow-Up and the Challenge of Intrauterine Treatment.

Carrilho MC, Bravo-Valenzuela NJ, Araujo Júnior E
Rev Bras Ginecol Obstet 2020 Apr;42(4):228-232. Epub 2020 Apr 24 doi: 10.1055/s-0040-1709738. PMID: 32330965 Free PMC Article

Congenital complete atrioventricular heart block. A 25 year follow-up study.

Corne RA, Mathewson FA
Am J Cardiol 1972 Mar;29(3):412-5. doi: 10.1016/0002-9149(72)90540-1. PMID: 5060814

See all (2)