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.