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Absent right sided atrioventricular connection

MedGen UID:
488863
•Concept ID:
C0344624
•
Congenital Abnormality
Synonym: Absent right sided atrioventricular connexion
SNOMED CT: Absent right sided atrioventricular connection (253285007)
 
HPO: HP:0011548

Definition

A defect where there is no connection between the right atrium and right ventricle. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbsent right sided atrioventricular connection

Recent clinical studies

Etiology

The surgical anatomy of hearts with no direct communication between the right atrium and the ventricular mass--so-called tricuspid atresia.
Scalia D, Russo P, Anderson RH, Macartney FJ, Hegerty AS, Ho SY, Daliento L, Thiene G
J Thorac Cardiovasc Surg 1984 May;87(5):743-55. PMID: 6717052

Diagnosis

An interesting case of an absent right-sided AV connection with an atrioventricular septal defect and double-outlet left atrium.
Tripathi SP, Phadke MS, Nabar AA, Lanjewar CP, Kerkar PG
Eur Heart J Cardiovasc Imaging 2014 Oct;15(10):1181. Epub 2014 May 22 doi: 10.1093/ehjci/jeu092. PMID: 24855219
Absent left-sided atrioventricular connexion, with right atrium connected to left ventricle: prospective diagnosis in infancy, and outcome.
Sreeram N, Walsh K, Nobre A, Smith A, Peart I, Arnold R
Int J Cardiol 1992 Jan;34(1):7-19. doi: 10.1016/0167-5273(92)90077-g. PMID: 1372302
Identification and analysis of left atrial isomerism.
Sharma S, Devine W, Anderson RH, Zuberbuhler JR
Am J Cardiol 1987 Nov 15;60(14):1157-60. doi: 10.1016/0002-9149(87)90410-3. PMID: 3687746
Absent right atrioventricular connexion with the left atrium connected to the morphologically right ventricle, a right-sided rudimentary left ventricle, and right juxtaposition of the atrial appendages: documentation by angiography and cross-sectional echocardiography.
Casta A, Wolf WJ, Sapire DW
Int J Cardiol 1985 Jan;7(1):21-8. doi: 10.1016/0167-5273(85)90168-8. PMID: 4055132

Prognosis

Absent left-sided atrioventricular connexion, with right atrium connected to left ventricle: prospective diagnosis in infancy, and outcome.
Sreeram N, Walsh K, Nobre A, Smith A, Peart I, Arnold R
Int J Cardiol 1992 Jan;34(1):7-19. doi: 10.1016/0167-5273(92)90077-g. PMID: 1372302

Clinical prediction guides

The surgical anatomy of hearts with no direct communication between the right atrium and the ventricular mass--so-called tricuspid atresia.
Scalia D, Russo P, Anderson RH, Macartney FJ, Hegerty AS, Ho SY, Daliento L, Thiene G
J Thorac Cardiovasc Surg 1984 May;87(5):743-55. PMID: 6717052

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