In-utero course:
Followup echocardiography at 34 weeks gestation revealed hydrops fetalis, severe right atrial dilatation, RV dysfunction, bradycardia (80 bpm) and absent end diastolic velocity in the umbilical arteries.[Figure 7A and B] The fetus was died in-utero. A 2000g male baby was delivered with absent cardiac activity through normal delivery. Option of fetal autopsy and genetic evaluation of the fetal intracardiac blood were given but parents denied further evaluation. A schematic cartoon demonstrating the surgeons view of this anomaly after opening the right atrium is shown in the Figure 8.