Ebstein's Anomaly

Rare

·        POSTERIOR and SEPTAL cusps of tricuspid valve are displaced into the Right ventricle which makes Right ventricle smaller

·        Combined with tricuspid insufficiency or sometime tricuspid stenosis, the Right Atrial pressure is elevated  producing a R to L shunt  through the foramen ovale

·        Pulmonary vasculature is usually diminished

·        In those with large atrial septal defects, the pulmonary vasculature may appear prominent

·        There is an atrialized portion of the right ventricle between the AV groove and the tricuspid valve

·        The right ventricle and right atrium dilate

o    The right heart border becomes prominent

·        Think of Ebstein’s anomaly if: 

o       Pulmonary flow is decreased

o       Cyanosis is present in the neonate

o       One of the few conditions to produce cardiomegaly in the first few days of life 

Ebstein Anomaly. This is a cyanotic, newborn with a markedly enlarged heart and, in the costophrenic angles where lung may still be seen, a dearth of pulmonary vessels.