Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Complete atrioventricular septal defect

Synonyms

Complete AVSD

Definitions

A rare, congenital cardiac anomaly characterized by a common atrioventricular junction with a common AV valve, an interatrial communication just above the common AV valve (ostium primum defect), a posterior interventricular communication (inlet VSD), that results in shunting at both the atrial and ventricular level. Morphologically, the common atrioventricular valve has 4 or 5 leaflets including superior and inferior bridging leaflets with a single annulus.

ID

http://www.orpha.net/ORDO/Orphanet_1329

alternative_term

Complete AVSD

Complete atrioventricular canal

Complete atrioventricular canal defect

CAVC

Complete atrioventricular septal defect with atrial and ventricular components

definition

A rare, congenital cardiac anomaly characterized by a common atrioventricular junction with a common AV valve, an interatrial communication just above the common AV valve (ostium primum defect), a posterior interventricular communication (inlet VSD), that results in shunting at both the atrial and ventricular level. Morphologically, the common atrioventricular valve has 4 or 5 leaflets including superior and inferior bridging leaflets with a single annulus.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=1329

has_age_of_onset

Infancy

Neonatal

has_inheritance

Not applicable

hasDbXref

UMLS:C0344787

ICD-10:Q21.2

ICD-11:LA87.43

label

Complete atrioventricular septal defect

notation

ORPHA:1329

part_of

http://www.orpha.net/ORDO/Orphanet_98722

prefixIRI

ORDO:Orphanet_1329

prefLabel

Complete atrioventricular septal defect

present_in

Worldwide AND has_point_prevalence_range : Unknown

Europe AND has_birth_prevalence_average_value : 20.0 AND has_birth_prevalence_range : 1-5 / 10 000

treeView

http://www.orpha.net/ORDO/Orphanet_98722

subClassOf

http://www.orpha.net/ORDO/Orphanet_557493

http://www.orpha.net/ORDO/Orphanet_377791

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