Neonatal lupus erythematosus presenting with congenital heart block: clinical characteristics and follow-up.

Objective: Congenital heart block (CHB) in patients with neonatal lupus erythematosus (NLE) is usually irreversible. Our study aimed to investigate the clinical characteristics and prognostic follow-up results of patients with NLE presenting with CHB and to provide a reference for the clinical treatment and management of such patients.

Methods: Six patients with NLE and CHB at the Children's Hospital of Soochow University between January 1, 2011, and December 31, 2023, were retrospectively analyzed. Prenatal maternal data, clinical characteristics, laboratory investigations, treatments, and follow-ups were analyzed using descriptive statistics.

Results: Six patients with NLE and CHB-four females, two males, and five preterm infants-were included. Three patients had degree III CHB, one had degree II CHB, and two had degree I CHB. The diagnostic gestational age for CHB was 23.6 ± 2.7 weeks. Four patients experienced varying degrees of bradycardia during the fetal period. Of the six pregnant mothers, three had systemic lupus erythematosus, one had Sjogren's syndrome, and one was positive for the anti-SSA/Ro autoantibody after the postpartum patient was seropositive. All pregnant mothers with autoimmune diseases were in remission and stable before delivery. One case of mildly active lupus in early pregnancy resolved after low-dose oral steroid hormone administration. All six patients with NLE-related manifestations, except CHB, showed improvement in systemic symptoms within 6 months. One patient was implanted with a pacemaker for exercise intolerance and complete atrioventricular block (CAVB) at the age of 1 year and 5 months and is now tolerating activity well without abnormal cardiac rhythms. One of the six patients had a global developmental delay, and two had a motor developmental delay.

Conclusions: Fetal echocardiography should be performed in high-risk pregnancies between 16 weeks of gestation and 1 month after birth. Patients with NLE and CHB are prone to varying degrees of growth retardation. Pacemaker implantation is an effective treatment for children with CAVB. Key Points • CHB in patients with NLE is usually irreversible. • Patients with NLE and CHB are prone to varying degrees of growth retardation. • Implantation of a pacemaker is an effective treatment for children with CAVB.