Congenital tricuspid valve dysplasia
ORPHA:555874· ICD-10 Q22.8
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.
- Prevalence
- Unknown
- Age of onset
- Antenatal, Neonatal