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Congenital Defects Tutorial
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Tetralogy of Fallot Pulmonary Stenosis Pulmonary Atresia Tricuspid Atresia Ebstein's Anomaly

Ebstein's Anomaly

  • Ebstein's anomaly is a rare defect that involves the tricuspid valve and right ventricle. There is significant variation in the anatomy of the valve but always involves, at least in part, the septal and posterior leaflets which are deformed and displaced toward the apex (downward), adherent to the ventricular septum. The wall of the right ventricle found near the adherent leaflets is said to be arterialized and very thin-walled. The right atrium is almost always enlarged while enlargement of the right ventricle is more variable.
  • Four subtypes have been designated based on the relative volume of the right ventricle.
    • Type A describes a right ventricle with adequate volume.
    • Type B involves a large atrialized area of the right ventricle with freely moving anterior valve leaflet.
    • Type C refers to a defect where the anterior leaflet is highly restricted in movement and may obstruct the right ventricular outflow tract.
    • Type D describes a defect where the ventricle is almost completely arterialized aside from a small infundibular portion.
  • Ebstein anomaly is almost always associated with patent foramen ovale or atrial septal defect. Other lesions including tetralogy of Fallot, ventricular septal defect, pulmonary stenosis, and pulmonary atresia may occur but are less common.
  • Tricuspid insufficiency or stenosis limits blood flow through the right heart and into the pulmonary artery. Increased right atrial pressure due to limited outflow leads to right-to-left shunting through the often patent foramen ovale. This shunting causes a variable degree of cyanosis which is present at birth and is sometimes accompanied by tachypnea. Both pulmonary arterial resistance and right-to-left shunting decrease in the days and weeks after birth which may lessen the cyanosis initially present in infancy.
  • During infancy, Ebstein anomaly is typically managed by administering prostaglandins and/or nitrous oxide to increase pulmonary blood flow and control cyanosis. Complex surgical procedures may be performed to correct lesions but long-term outcomes in controlled studies have yielded mixed results (Keane). Older children with Ebstein anomaly are generally followed medically with special attention to rhythm abnormalities and congestive heart failure. Surgery is performed only on patients who are very symptomatic, cyanotic, or in congestive heart failure.