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Partial atrioventricular canal

Disease definition

Partial atrioventricular canal (PAVC) is a congenital heart malformation characterized by an atrial septal defect (ASD; ostium primum), clefts of mitral and occasionally tricuspid valves, two separate atrioventricular (AV) valve annuli and an intact ventricular septum. The typical symptoms of PAVC are impaired exercise capacity and exertional dyspnea.

ORPHA:1330

  • Synonym(s):
    • PAVC
    • Partial atrioventricular canal defect
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: Q21.2
  • OMIM: -
  • UMLS: C0344735
  • MeSH: C536112
  • GARD: 4229
  • MedDRA: -

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