Health Outcome of 215 Mothers of Children with Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry

Objective Transplacental passage of maternal anti-SSA and -SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).

Methods This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.

Results We analyzed 215 women with at least one pregnancy with CHB. Before this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE) (n=26, 12%) and Sjögren's syndrome (SS) (n=16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis: mainly SLE (n=54, 25%) and SS (n=72, 33%). Three SLE patients had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (p<0.0001), dry syndrome (p=0.01), and parotid swelling (p=0.05).

Conclusion One quarter of the patients had an autoimmune disease diagnosis at the fetal CHB diagnosis. Nearly half those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs rarely required.