Right ventricular exclusion procedure with total cavopulmonary connection: An alternative operative approach in adults with severe Ebstein anomaly

摘要

Ebstein anomaly represents less than 1% of all congenital heart defects.. This anomaly affects the tiicuspid valve (TV), but also represents a malformation of the right ventricle (RV),In symptomatic neonates, we found that single-ventricle palliation has its major impact on a combination of a poorly functioning RV, type IV Ebstein anomaly, and an unguarded TV with RV outflow tract obstruction, Our experience in older children and adults is similar to that in other published series on valve reconstruction. We describe an unusual presentation of adult Ebstein anomaly that prompted successful single-ventricle palliation#