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Familial isolated complete right bundle branch block

MedGen UID:
83311
•Concept ID:
C0340504
•
Disease or Syndrome
Synonym: Bundle Branch Block, Familial Isolated Complete Right
SNOMED CT: Familial isolated complete right bundle branch block (233919006)
 
Monarch Initiative: MONDO:0007241
OMIM®: 113950

Clinical features

From HPO
Right bundle branch block
MedGen UID:
88445
•Concept ID:
C0085615
•
Disease or Syndrome
A conduction block of the right branch of the bundle of His. This manifests as a prolongation of the QRS complex (greater than 0.12 s) with delayed activation of the right ventricle and terminal delay on the EKG.
See: Feature record | Search on this feature
Abnormality of the cardiovascular system
MedGen UID:
116727
•Concept ID:
C0243050
•
Congenital Abnormality
Any abnormality of the cardiovascular system.
See: Feature record | Search on this feature

Recent clinical studies

Etiology

Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block.
Baruteau AE, Behaghel A, Fouchard S, Mabo P, Schott JJ, Dina C, Chatel S, Villain E, Thambo JB, Marçon F, Gournay V, Rouault F, Chantepie A, Guillaumont S, Godart F, Martins RP, Delasalle B, Bonnet C, Fraisse A, Schleich JM, Lusson JR, Dulac Y, Daubert JC, Le Marec H, Probst V
Circulation 2012 Sep 18;126(12):1469-77. Epub 2012 Aug 16 doi: 10.1161/CIRCULATIONAHA.111.069161. PMID: 22899775

Diagnosis

Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block.
Baruteau AE, Behaghel A, Fouchard S, Mabo P, Schott JJ, Dina C, Chatel S, Villain E, Thambo JB, Marçon F, Gournay V, Rouault F, Chantepie A, Guillaumont S, Godart F, Martins RP, Delasalle B, Bonnet C, Fraisse A, Schleich JM, Lusson JR, Dulac Y, Daubert JC, Le Marec H, Probst V
Circulation 2012 Sep 18;126(12):1469-77. Epub 2012 Aug 16 doi: 10.1161/CIRCULATIONAHA.111.069161. PMID: 22899775

Prognosis

Isolated congenitally complete heart block attributable to combined nodoventricular and intraventricular discontinuity.
Chow LT, Cook AC, Ho SY, Leung MP, Anderson RH
Hum Pathol 1998 Jul;29(7):729-36. doi: 10.1016/s0046-8177(98)90283-0. PMID: 9670831

Clinical prediction guides

Parental electrocardiographic screening identifies a high degree of inheritance for congenital and childhood nonimmune isolated atrioventricular block.
Baruteau AE, Behaghel A, Fouchard S, Mabo P, Schott JJ, Dina C, Chatel S, Villain E, Thambo JB, Marçon F, Gournay V, Rouault F, Chantepie A, Guillaumont S, Godart F, Martins RP, Delasalle B, Bonnet C, Fraisse A, Schleich JM, Lusson JR, Dulac Y, Daubert JC, Le Marec H, Probst V
Circulation 2012 Sep 18;126(12):1469-77. Epub 2012 Aug 16 doi: 10.1161/CIRCULATIONAHA.111.069161. PMID: 22899775
An isolated cardiac conduction disease maps to chromosome 19q.
de Meeus A, Stephan E, Debrus S, Jean MK, Loiselet J, Weissenbach J, Demaille J, Bouvagnet P
Circ Res 1995 Oct;77(4):735-40. doi: 10.1161/01.res.77.4.735. PMID: 7554120

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