• Autoimmunity
• Autoantibodies
• Epidemiology

Since the relationship of maternal autoimmunity and congenital heart block (CHB) was initially described in the late 1970s, investigators have attempted to identify additional factors associated with its development.1–4 Detection of CHB in the absence of cardiac structural abnormalities predicts the presence of maternal autoantibody responses against the ribonucleoproteins SSA/Ro and SSB/La in >85% of cases.5 CHB is associated with considerable morbidity and mortality, with a 17.5% case fatality rate and approximately 70% requiring permanent pacemaker placement.6 Sustained reversal of third-degree CHB has never been achieved and to date there is no approved medication for treatment or prevention of this disease. Recognising those mothers at increased risk of CHB in an offspring would provide insights into the pathogenesis of disease and help prioritise allocation of screening resources, including intense echocardiographic monitoring.

In a recent publication in Lupus Science and Medicine, Tonello et al sought to identify maternal autoantibody profiles conferring high risk for CHB.7 Importantly, all serological evaluations were done during the pregnancies. The …