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Partial atrioventricular canal

Orpha number ORPHA1330
Synonym(s) -
Prevalence 1-5 / 10 000
Inheritance -
Age of onset All ages
  • Q21.2
  • C0344735
  • C536112
MedDRA -


Partial atrioventricular canal (PAVC) is a congenital heart malformation characterized by a primum atrial septal defect (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.

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