Congenital anomaly of the tricuspid valve…

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Congenital anomaly of the tricuspid valve chordae

ORPHA:99055Morphological anomaly

Also called Congenital anomaly of tricuspid chordae tendineae · Congenital anomaly of tricuspid tendinous chords

What it is

A rare, congenital anomaly of the tricuspid subvalvular apparatus characterized by aberrant tendinous chords, which insert at the clear zone of the leaflet instead of its free edge and connect to the endocardium instead of the papillary muscles. Resulting tethering of one or more tricuspid leaflets leads to their impaired mobility and tricuspid regurgitation. Association with other congenital cardiac anomalies has been reported.

Key facts

Classified as
Morphological anomaly

ICD-10 codes

Q22.8filed under a broader ICD-10 category — shared with 5 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0020396UMLS C3165162

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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