A female infant is described with a complete ectopia cordis and a single atrium who presented four hours after birth. There was complete deficiency of the sternum with the absence of pericardium over the heart. There was an associated omphalocele containing an enlarged liver. The infant died 45 hours after birth following an attempt to provide tissue covering. Additional intracardiac anomalies included a ventricular septal defect overriding aorta and total anomalous venous drainage.
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