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Ebstein anomaly

DA: 235




Developmental anomaly information

Definition: Ebstein anomaly of the tricuspid valve is a congenital heart lesion that involves abnormal attachments of the tricuspid valve leaflets to the annulus of the tricuspid valve. Marked variability exists in the degree of displacement of the septal and posterior leaflets into the cavity of the right ventricle. This may result in a right ventricle that is divided into an atrialized portion above the valve leaflets and a muscular portion below the valve leaflets. An atrial septal defect (ASD) or patent foramen ovale (PFO) is present in 90% of the patients; pulmonary stenosis or atresia is present in 20-25% of the patients.


Synonyms:

  • Ebstein disease


Developmental anomaly parent: Congenital Heart Defects

Topology: Human body > Thorax > Mediastinum > Heart and great vessels > Heart

System: Human body > Cardiovascular system > Heart

Developmental anomaly category: Malformation

Case records MDB185, MDB468
Links

Humpath: 8167

OMIM: 224700

Observed in MDB cases
Observed in combinaisons (MDB)
Observed in malformative diseases (MDB)
Associated genes in profile
Associated genes in malformative diseases
Associated chromosomal diseases
Associated signaling pathways
Associated developmental anomalies

Here is the list of developmental anomalies associated with this developmental anomaly, based on the cases' information :



Last modified: 2008-12-30 17:39:24 by farideh

Created: 2008-05-16 02:00:00 by phsaw



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