Abstract
Ebstein's anomaly (EA) and dysplasia of the tricuspid valve (TVD) are rare but serious fetal observations with a probability of survival to infancy of less than 25% in all major patient series. The worst end of the disease spectrum is detected in utero, with many affected fetuses notable for striking cardiomegaly, significant tricuspid incompetence and absent antegrade pulmonary blood flow. To survive the fetal left ventricle needs to compensate for the dysfunctional right ventricle. Survival after birth rather depends on the right ventricle to restore sufficient pulmonary blood flow.
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