Relation of maternal anti-Ro/La antibodies to aortic dilation in patients with congenital complete heart block

An association between congenital complete atrioventricular block (cCAVB) and aortic dilation during childhood has recently been reported. We sought to further explore this relation with particular emphasis on the natural history of aortic abnormalities over time. The relation of maternal anti-Ro/La antibody status to the aortic size of children affected with cCAVB was also assessed. The patients were evaluated longitudinally with serial echocardiography. During a 15-year period, 62 patients at our institution were diagnosed with cCAVB, of whom 40% were exposed to maternal autoimmune antibodies and 35% were not. The antibody status in the remaining patients was unknown. The patients underwent 9.3 ± 6.5 echocardiograms during the follow-up period. Dilation of the ascending aorta, defined as a z score >2.0, was present on the initial echocardiogram in all patients exposed to maternal antibodies and persisted during long-term follow-up in 96% of these patients. In contrast, 5% and 10% of patients without exposure to maternal autoimmune antibodies had aortic dilation on the initial and follow-up studies, respectively (p <0.001 and p <0.001, respectively). In conclusion, patients with autoimmune-mediated cCAVB merit periodic echocardiographic monitoring into adulthood to assess persistent or progressive aortic dilation and its attendant complications.