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Ebsteins Anomaly

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  • Ebsteins anomaly

    Pathophysiology:

    Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Ebstein's anomaly is a rare heart defect that's present at birth (congenital). In Ebstein's anomaly, tricuspid valve i.e valve between the chambers on the right side of your heart doesn't work properly. Blood leaks back through the valve, making your heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure.

  • Ebsteins anomaly

    Treatment:

    Physical findings in patients with Ebstein anomaly span a spectrum from subtle to dramatic. They may include the following: Cyanosis and clubbing Precordial asymmetry Jugular venous pulse Arterial pulses: Usually normal but are diminished late in the disease course Heart sounds

  • Ebsteins anomaly

    Statistics:

    The natural course of the disease varies according to the severity of tricuspid valve displacement. Patients presenting in infancy generally have severe disease and unfavorable prognosis. Mean age of presentation is in the middle teenage years. According to older observational data, appro Ebstein anomaly is more common in children of white females. Approximately 5% of these patients survive beyond age 50 years. The oldest recorded patient lived to age 85 years.

 

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