A Neonate With Complete Congenital Heart Block Delivered Via Ex Utero Intrapartum Treatment (Exit) Procedure: A Case Report

Congenital heart block (CHB) is a rare condition affecting newborns and infants.There is a delay or a complete obstruction in the electrical signals coordinating the contractility of the heart chambers [1] that presents fetal bradycardia.Most common cause of CHB is associated with maternal autoimmune disease, particularly systemic lupus erythematosus (SLE) [2].However, CHB can occur in infants without an underlying cause.The diagnosis is made through physical examination, electrocardiogram, and echocardiogram.Presenting a case of a preterm neonate diagnosed with CHB on congenital scan during a prenatal check-up.Fetal bradycardia was noted hence referred to a pediatric cardiologist.Fetal 2D Echo revealed a complete congenital heart block hence the mother was worked up for SLE and confirmed with a positive ANA, Anti-SSA (Ro) and Anti-SSB (La).Pre-operatively, a multidisciplinary conference was convened with specialists from Newborn Medicine, Pediatric Cardiology, Electrophysiology, Thoraco-Cardiovascular Surgery, Obstetrics, Perinatology, Rheumatology and Anesthesiology.Everyone agreed to the proposed Ex Utero Intrapartum Treatment (EXIT) procedure.A scheduled primary cesarean section under general anesthesia was done 36 weeks AOG.Upon delivery, EXIT procedure, epicardial pacemaker insertion via median sternotomy, and left femoral artery cannulation were performed and the patient was further monitored at the Neonatal ICU.She stayed for 48 days, developed ventilator-associated pneumonia but eventually discharged improved.Long term plan for the patient includes frequent cardiac evaluation, growth, and development monitoring.During her follow-ups she was noted to have ASD (atrial septal defect) on diuretics with good compliance and is thriving well.