Background/Purpose: Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality, however there has been minimal information on long-term outcomes of affected individuals.  This study was initiated to evaluate the presence of and risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counseling, further understand pathogenesis and provide potential preventative strategies.

Methods: Echocardiogram reports throughout life were evaluated in 239 individuals with cardiac-NL: 143 from ages 0-1 years, 176 from >1-17 years, and 64 >17 years.  Cardiac dysfunction was defined as one or more of the following 1) qualitatively decreased left ventricular function on echocardiogram, 2) concurrent use of cardiac medications 3) heart transplant.  Aortic dilation was defined as root or ascending aorta z-score >2.0.  Multivariable logistic regression analyses were performed to evaluate associations of cardiac dysfunction at each age group with demographic, fetal and postnatal factors.

Results: Cardiac dysfunction was identified in 22.4% of children at ages 0-1, 14.8% ages >1-17 and 28.1% ages >17.  In multivariable analysis of the age 0-1 group, female sex (OR 0.41, p=0.046), older age at the time of echo (OR 0.14, p=0.067) and higher in utero nadir ventricular heart rate (OR 0.95, p=0.077) were protective, while greater length of time paced (OR 10.82, p=0.035) was associated with increased odds of cardiac dysfunction.  In the age >1-17 group, black race (OR 10.28, p=0.021), length of time paced (OR 1.31, p=0.039), in utero extranodal disease (OR 3.11, p=0.061) and a severity score representing overall fetal disease weighted by mortality risk factors (OR 1.17, p=0.048) associated with cardiac dysfunction.  In those >17, a greater length of time paced (OR 1.29, p=0.018) and the severity score (OR 1.42, p=0.037) were associated with dysfunction.  In 106 children with echos at ages 0-1 and >1-17, 43.8% (95% CI: 19.8, 70.1%) of those with dysfunction at 0-1 were also affected at >1-17, while all others reverted to normal.  Of those without dysfunction at ages 0-1, 8.9% (CI: 3.3, 16.8%) developed new dysfunction at >1-17 years.  Among the 34 with echos at ages >1-17 and >17, 6.5% (CI: 0.8, 21.4%) with normal function at >1-17 developed dysfunction in adulthood.  Aortic dilation was present in 13.5% at ages 0-1, 15.0% at >1-17, and 9.4% at >17.  Of the 15 (14.3%; CI: 8.2, 22.5%) of 105 children with dilation at age 0-1, 9 (60.0%; CI: 32.2, 83.7%) still had dilation at >1-17, while the other 6 reverted to normal.  Among the 90 cases without dilation at age 0-1, 8 (8.9%; CI: 3.9, 16.8%) developed dilation at >1-17 years.  For the 34 children with echos at both >1-17 and >17, none had aortic dilation at age 1-17, with one (2.9%; CI: 0.1, 15.3%) developing dilation after 17. 

Conclusion: Risk factors in early fetal life can influence cardiac morbidity into the adult years.  Cardiac dysfunction in the first year normalizes by later childhood in the majority of cases, possibly due to the short-term effects of cardiac pacing or resolution of inflammation with the clearance of maternal autoantibodies.  New onset dysfunction and aortic dilation, albeit rare, can occur de novo after the first year of life.   

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/factors-associated-with-cardiac-dysfunction-in-a-longitudinal-follow-up-of-neonatal-lupus-2/