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Ebstein's anomaly occurs due to improper development of the tricuspid valve in the first eight weeks of fetal growth. It can be caused by a number of factors, though, most of the time, this heart defect occurs sporadically (by chance), with no apparent reason for its development.
Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families.
Why is Ebstein's anomaly a concern?
Ebstein's anomaly has many different presentations depending upon the severity of the lesion. Severe Ebstein's anomaly may cause symptoms in newborns including cyanosis and heart failure. Mild Ebstein's anomaly may have no symptoms during childhood and present later in life with shortness of breath, fatigue and decreasing exercise tolerance.
The abnormal tricuspid valve results in regurgitation of blood into the right atrium. The right ventricle has to then work harder to move blood forward. Over time, the right ventricle will become enlarged and may not meet the demands of the heart, resulting in failure. Additionally, Ebstein's anomaly is often associated with abnormal rhythms. These rhythm abnormalities may cause palpitations, dizziness, syncope and even sudden death.