Ebsteins anomaly of the tricuspid valve natural history, complications & prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]}; Claudia P. Hochberg, M.D.; Priyamvada Singh, MBBS [3] Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]
Overview
The symptoms of Ebstein's anomaly vary in severity, with some patients experiencing either no symptoms or very mild symptoms and others experiencing symptoms that may worsen over time such as (cyanosis), heart failure, heart block, or other tachyarrhythmias or bradyarrhythmias. Paradoxical embolization, brain abscesses and pulmonary embolism may also occur.
Natural History
The earlier the symptoms develop, the more severe the disease.
Complications
- Severe tricuspid regurgitation may lead to dilation of the right ventricle, hepatic congestion and congestive heart failure.
- Other complications may include:
- Abnormal heart rhythms (arrhythmias), including abnormally fast rhythms (tachyarrhythmias) and abnormally slow rhythms (bradyarrhythmias and heart block)
- Paradoxical embolization due to an associated ASD
- Brain abscess
Prognosis
The prognosis of Ebstein's anomaly of the tricuspid valve is extremely variable and depends on the spectrum of the severity of disease. The mortality depends upon the cardiac output. Intrauterine mortality is up to 85%. Among newborns the mortality 70% if cyanosis is present versus 15% if cyanosis is absent. The one-year survival is 67%, and the ten year survival is 59%.