Fetal Complete Heart Block. A Successful Surgical Permanent Pacemaker Implantation in a Small for Gestational Age Baby (2.4 Kg): A Case report and Mini Review

Congenital complete heart block (CCHB) without structural cardiac anomalies is a rare disease with an incidence of 1 in 15 000 – 20000 live births. CCHB is associated with high intrauterine and post-natal mortality. Maternal autoimmune diseases are often the predisposing factors for the isolated CCHB. Antibodies cross the placenta and cross-react with the developing conduction system, leading to injury and CCHB. Prenatal detection using intrauterine foetal echocardiography at the time of the obstetrics scan, and management with steroids, as well as appropriate delivery planning, and urgent permanent pacemaker implantation (PPI) in the newborn may improve the outcomes in CCHB. However, PPI in neonates can be challenging due to their small size, small vascular and ventricular sizes, and even leadless pacemakers and transvenous implantation in the neonates remain unsuitable. We describe a rare case of CCHB diagnosed at 19th week of pregnancy onwards in 25 yrs old mother, who was a known case of SLE for the last 5 yrs. The CCHB in the small -for -gestational -age (2.4 kg) male, newborn was refractory to the infusion of dobutamine, isoprenaline and dexamethasone. A detailed informed consent was obtained from the parents, and the CCHB was successfully managed with surgical LV epicardial PPI and using a VVI pacing mode at 120 bpm on the first day of life. Only few cases have been reported on the surgical LV epicardial PPI where both leads are attached to the LV and device has been fixed in the left thoracic cavity.