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Complete atrioventricular septal defect

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • CAVC
    • Complete AVSD
    • Complete atrioventricular canal
    • Complete atrioventricular canal defect
    • Complete atrioventricular septal defect with atrial and ventricular components
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q21.2
  • ICD-11: LA87.43
  • OMIM: -
  • UMLS: C0344787
  • MeSH: -
  • GARD: 1454
  • MedDRA: -

Detailed information

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.