Management with a staged approach of the premature hydropic fetus due to complete congenital heart block

The management of the preterm fetus with hydrops due to complete congenital heart block is difficult. The outcome is frequently associated with significant morbidity and mortality. Two fetuses presented at the post menstrual age of 29 and 30 weeks respectively with severe hydrops due to complete heart block. The following staged approach was adopted: (1) enhance fetal lung maturation with maternal corticosteroids and thyroid releasing hormone for 48 h; (2) elective Caesarean section; (3) classical neonatal management consisting of intubation and ventilation, drainage of all cavities with effusions; (4) increase neonatal heart rate by administration of i.v. isoprenaline, by bipolar trans-oesophageal pacing or epicutaneo-oesophageal pacing; (5) after the regression of the hydrops, start epicardial pacing after implantation of 2 or 3 temporary epicardial 3/0 pacemaker; (6) implantation of a permanent abdominal pacing system with steroid epicardial tip once the threshold exceeds 20 mA or when the baby weighs more than 1500 g. In these patients a permanent pacing system was implanted at the ages of 8 weeks (2045 g) and 4 weeks (1560 g) respectively. No major complications occurred; the cardiac outcome with 37 and 34 months of follow up is excellent. This proposed staged approach with temporary epicardial leads can improve the outcome of hydropic fetuses due to complete congenital AV block.