Background/Purpose: Transplacental passage of maternal anti-Ro IgG is thought to be critical in initiating inflammation leading to congenital heart block in neonatal lupus erythematosus (NLE). Congenital heart block is the most frequent manifestation of cardiac NLE. To date, the majority of studies have focused on babies born to mothers with systemic lupus erythematosus (SLE) or congenital heart block babies born to anti-Ro and/or anti-La mothers. In this large cohort study, we aimed to determine the proportion of babies born to anti-Ro and/or anti-La positive mothers with SLE, Sjögren syndrome or rheumatoid arthritis (RA), with congenital heart defects (CHD).

Methods: The Hospital for Sick Children offers serial fetal echocardiography for women with anti-Ro and/or anti-La antibodies. Babies born to anti-Ro and/or anti-La mothers are referred to the NLE clinic for follow-up. We performed an observational study of our large, single-centre NLE clinic cohort, on whom prospective data has been collected since 1984. We included all infants, seen in our clinic, born to mothers positive for anti-Ro and/or anti-La antibodies, with SLE, Sjögren syndrome or RA. Among them we identified infants with cardiac NLE disease diagnosed either prenatally or postnatally. We identified those babies with CHD, including septal defects, patent ductus arteriosus, and other rare structural heart defects. We compared the proportion of babies with CHD, with and without NLE congenital heart block using Fisher’s exact test.

Results: A total of 451 children were included. Seven (1.6%) children had CHD. The most frequently observed CHD were ventricular septal defects and patent ductus arteriosus. Among cases with congenital heart block (N=28), the frequency of CHD was further increased, with 3 (10.7%) children diagnosed with at least one CHD. This was significantly higher when compared to the rate of CHD among babies without congenital heart block (0.9%, p < 0.006).

Conclusion: In our NLE clinic cohort of children born to mothers with anti-Ro and/or anti-La antibodies and SLE, Sjögren syndrome or RA, we observed higher rates of CHD among those with congenital heart block when compared to our NLE clinic babies without congenital heart block. This suggests a common pathway in the development of congenital heart block and CHD. The precise role of maternal antibodies in the development of CHD requires further investigation and replication.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/congenital-heart-defects-in-children-born-to-mothers-with-rheumatic-disease-and-anti-ro-andor-anti-la-antibodies/