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Congenital tricuspid valve dysplasia

Disease definition

A rare congenital tricuspid malformation characterized by irregular thickening of the leaflet tissue by myxoid connective tissue in a normally delaminated tricuspid valve, without septal leaflet displacement, and without an atrialized right ventricle. The chordae tendineae may be short or absent. The affected valve is stenotic and/or incompetent. Clinically, most patients are asymptomatic and are diagnosed in the context of the evaluation of a murmur.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Antenatal, Neonatal
  • ICD-10: Q22.8
  • OMIM: -
  • UMLS: C4255215
  • MeSH: -
  • GARD: -
  • MedDRA: -
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