The case

Ms. L was pregnant at 37 years of age. Amniocentesis, performed because of her advanced maternal age, revealed a normal 46 XY chromosome analysis. At 34 weeks estimated gestational age she underwent a routine obstetrical ultrasound which revealed an abnormal four-chamber image of the fetus' heart. A complete fetal echocardiogram revealed a small left atrium and ventricle with mitral atresia, and a hypoplastic aorta without forward flow from the left ventricle to the ascending aorta. The coronary arteries and head and neck vessels were supplied by a widely patent ductus arteriosus. A diagnosis of hypoplastic left heart syndrome (HLHS) was made.

As HLHS carries a grave prognosis if untreated, the parents were given the following options: (1) no treatment, (2) conventional surgical approach (Norwood procedure), or (3) heart transplantation. After much discussion with the pediatric cardiologists at the local children's hospital, the family opted for heart transplantation and the fetus was placed on the heart transplant waiting list at 36 weeks gestation. If an appropriate donor heart became available, Ms. L would undergo a cesarean section, the infant would be immediately resuscitated and started on prostaglandin to maintain ductal patency, and would then undergo the heart transplant operation.

Ms. L gave birth at 38 weeks estimated gestational age. A complete neonatal team was present in the delivery room and observed that the neonate was cyanotic with an oxygen saturation of 80% in room air. The umbilical vein was cannulated and prostaglandin therapy instituted.