Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Echocardiographic examination showed a giant Eustachian valve moving towards the tricuspid valve during late systole and leading to complete obstruction of the inflow. Blood flow was redirected through the persistent foramen ovale, producing a right-to-left shunt.
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