G208(P) Neonatal congenital heart block – management and outcome on cases admitted to a regional neonatal intensive care unit

Abstract

Aim Congenital heart block (CHB)1,2 detected at or before birth is strongly associated with maternal autoimmune antibodies, anti –La and anti Ro. The majority of cases are diagnosed between 18–24th weeks of gestation. Most mothers carrying auto-immune antibodies are not aware until their child is diagnosed with CHB. Our aim was to review the presentation, management and outcome of neonates admitted with CHB to a regional neonatal intensive care unit (NICU). Method We conducted a retrospective case notes review of all infants admitted with CHB to NICU over an 8 year period, 07/2009 to 08/2017. Results 14 babies, 8 females and 6 males were admitted during the study period. 12 cases were diagnosed during the antenatal period and 2 cases postnatally (including undiagnosed CHB presenting with foetal bradycardia at 27 weeks). All 14 infants were born by caesarean section in view of foetal bradycardia (range 35–90 bpm). The median gestational age was 36 weeks(27–39 weeks) and the mean birth weight was 2442 g (1138 g-3360 g).The reasons of CHB in these 14 babies are explained as follows: 10 cases had maternal Anti–Ro and Anti–La antibodies (3 cases of Sjogren’s syndrome,2 cases of Systemic Lupus Eryhtematosus and 5 cases were asymptomatic). 3 cases associated with Congenital heart disease (1 congenitally corrected TGA, 1 Left atrial isomerism and 1 VSD,ASD,PDA). 1 case of Long QTc syndrome with KCNH2 genetic mutation They were admitted to NICU and assessed with 12-lead and 24 hour ECG, echocardiography and electrolyte analysis (Potassium, calcium and Magnesium). 3 infants developed life threatening arrhythmias with pulseless ventricular tachycardia requiring resuscitation. 6 infants had pacemaker placement in the neonatal period (day 3–21) due to severe bradycardia, heart rate Conclusion Congenital heart block is associated with significant morbidity and mortality. In our cohort, indications for pacemaker placement included a neonatal baseline heart rate less than 50 bpm or presence of arrhythmias (Ventricular tachycardia) on 24 hour ECG monitoring. References . Kertesz NJ, Fenrich AL, Friedman RA. Congenital complete atrioventricular block. Texas Heart Institute Journal1997;24(4):301–307. . Friedman D, Duncanson L, Glickstein J, Buyon J. A review of congenital heart block. Images in Paediatric Cardiology2003;5(3):36–48.