Objectives Cardiac manifestations of neonatal lupus (NL) have been associated with significant morbidity and mortality; however, there is minimal information on long-term outcomes of affected individuals. This study was initiated to evaluate the presence of and the risk factors associated with cardiac dysfunction in NL after birth in multiple age groups to improve counselling, to further understand pathogenesis and to provide potential preventative strategies.
Methods Echocardiogram reports were evaluated in 239 individuals with cardiac NL: 143 from age 0–1 year, 176 from age >1–17 years and 64 from age >17 years. Logistic regression analyses evaluated associations of cardiac dysfunction at each age group with demographic, fetal and postnatal factors, using imputation to address missing data.
Results Cardiac dysfunction was identified in 22.4% at age 0–1 year, 14.8% at age >1–17 years and 28.1% at age >17 years. Dysfunction in various age groups was significantly associated with male sex, black race, lower fetal heart rates, fetal extranodal cardiac disease and length of time paced. In 106 children with echocardiograms at ages 0–1 year and >1–17 years, 43.8% with dysfunction at age 0–1 year were also affected at age >1–17 years, while the others reverted to normal. Of children without dysfunction at age 0–1 year, 8.9% developed new dysfunction between ages >1 and 17 years. Among 34 with echocardiograms at ages >1–17 years and >17 years, 6.5% with normal function at age >1–17 years developed dysfunction in adulthood.
Conclusions Risk factors in fetal life can influence cardiac morbidity into adulthood.
Although limited by a small number of cases, cardiac dysfunction in the first year often normalises by later childhood. New-onset dysfunction, although rare, can occur de novo after the first year.
- neonatal lupus
- cardiac dysfunction
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Handling editor Josef S Smolen
Contributors AS designed the study, collected and analysed the data and wrote the manuscript. PMI designed the study, analysed the data and helped write the manuscript. RPB collected the data and helped write the manuscript. RSG collected data and helped write the manuscript. DMF helped design the study, analyse the data and write the manuscript. RE and MYK helped design the study, performed statistical analysis and helped write the manuscript. JPB designed the study, analysed the data and led the study.
Funding This work was supported by a National Institutes of Health Merit Award (R37 AR042455, R37 AR042455-21S1 and R37 AR042455-21S2), the NIH/NIAMS contract for the Research Registry for Neonatal Lupus (N01 AR-4-2220), the Lupus Foundation of America Lifeline and National Institutes of Health (R03 HD069986 and R01 HD079951-01A1) awarded to JPB. AS was funded by the American Heart Association Founders Affiliate Clinical Research Program Award (#11CRP795008) and a NYU Clinical and Translational Science Institute Core Resources grant (NIH/NCRR 5UL1RR029893).
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval The institutional review board of the NYU School of Medicine has approved the evaluation of deidentified data.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are available upon reasonable request.
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