Background/Purpose: There are minimal data and no longitudinal studies regarding the long term cardiac health of children with cardiac manifestations of neonatal lupus (NL). This study was performed to evaluate risk factors for morbidity over time and provide evidence based guidance regarding the course of cardiac NL.

Methods: Echocardiograms throughout life were evaluated in 200 individuals born with cardiac NL from the Research Registry for Neonatal Lupus: 111 from ages 0-1 years, 156 ages 1-17 years, and 63 >17 years. A composite adverse outcome defined as qualitatively decreased left ventricular (LV) function or concurrent use of cardiac medications was assessed. Aortic dilation (root or ascending aorta z-score >2.0) was also recorded. Analyses were performed to associate the composite adverse outcome and aortic dilation with maternal medications, pacing, and fetal disease status, including a severity score based on mortality risk factors such as lower fetal heart rate and extranodal disease.

Results: The composite adverse outcome for cardiac dysfunction was identified in 18.9% of echos in children ages 0-1, 11.3% ages 1-17 and 20.6% ages >17. In 89 children in which echos were available at ages 0-1 and 1-17, 4/14 with dysfunction at ages 0-1 were also affected at ages 1-17, while 10 reverted to normal, and only 7/75 developed new dysfunction during age 1-17. In 31 cases with echos at ages 1-17 and >17, 1/2 cases with dysfunction at age 1-17 was also affected >17, and 3/29 developed new dysfunction in adulthood. Earlier age at pacemaker placement was associated with dysfunction in the 0-1 age group (p=0.018), but not in later ages. Cardiac dysfunction was significantly associated with number of years paced at ages >17 (p=0.001), but not earlier. A lower fetal ventricular heart rate at initial heart block detection was associated with cardiac dysfunction in all age groups (p=0.038, 0.009, 0.016 respectively). Fetal extranodal cardiac disease was associated with dysfunction (p=0.046) in ages >17. Higher fetal severity score associated with postnatal dysfunction in all age groups (p=0.063, 0.03, 0.005). Aortic dilation was present in 15.3% at ages 0-1 and 17.6% at ages 1-17, but at >17, dilation only occurred in 7.9%. There was no association of postnatal cardiac dysfunction or aortic dilation with maternal medication use, maternal rheumatic disease, fetal age at heart block detection or gestational age of birth.

Conclusion: 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. Aortic dilation can continue for longer periods, but also decreases in frequency with age. Nevertheless, cardiac dysfunction does persist in a fifth of cases and in adulthood there are associations with fetal extranodal disease and heart rate at detection. Patients who develop morbidity in utero may have subclinical damage or be more susceptible to future insults that manifest later in life, which can be exacerbated by prolonged pacing. Close monitoring and aggressive treatment of early extranodal disease in cardiac NL may have long term benefit in preventing subsequent morbidity.

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