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Start free with EleplanCongenital tricuspid valve dysplasia
ORPHA:555874Morphological anomaly
What it is
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.
Key facts
- Age of onset
- Antenatal, Neonatal
- Inheritance
- Not applicable
- Classified as
- Morphological anomaly
Signs and symptoms
Common30–79%
16- Abnormal tricuspid valve annulus morphology
- Abnormal tricuspid valve leaflet morphology
- Anomalous pulmonary venous return
- Cardiomegaly
- Cyanosis
- Hepatomegaly
- Hypoxemia
- Intrauterine growth retardation
- Premature birth
- Respiratory failure
- Respiratory failure requiring assisted ventilation
- Right ventricular hypertrophy
- Small for gestational age
- Systolic heart murmur
- Tachypnea
- Tricuspid regurgitation
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
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
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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