Abstract

Introduction: Cardiac manifestation of congenital heart block (CHB) in neonates following maternal anti-Ro and anti-La autoantibodies is a serious complication of neonatal lupus with a prevalence of 1: 22000 live birth and is associated with high morbidity and mortality. Disturbance of calcium homeostasis which leads to electrical conduction abnormality and eventually fibrosis of the heart tissue is the well-known pathogenesis of CHB. Most of the babies with CHB ended up with third-degree heart block and need a pacemaker as it is an irreversible injury. Case series: Here, we reported four case series of neonatal lupus with cardiac manifestation in mothers with positive anti-Ro or anti-La which have been detected during pregnancy within 24 to 32 weeks of gestation. All four cases of newborns ended up with third-degree heart block which required a permanent pacemaker by the latest age of two months old. Conclusion: A better understanding of the aetiology of the development of CHB and identifying the risk factors in mothers is crucial for the outcomes of CHB newborns. Early diagnosis through early screening, enhanced diagnostic tools for CHB, and timely treatment of the mother may aid clinicians in better management and improve the survival rate of infants with CHB.

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