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Objectives: There are no population-based estimates of the incidence or risk factors for acute cardiac manifestations in children with systemic lupus erythematosus (SLE) to guide screening and diagnostic imaging practices. We estimated the incidence and prevalence of acute cardiac manifestations of child-onset SLE compared to adult-onset SLE and identified factors associated with cardiac diagnoses. Methods: We identified children (5–17 years) and adults (18–64) with incident SLE (≥3 ICD-9 codes 710.0, >30 days apart) using Clinformatics® DataMart (OptumInsight, Eden Prairie, MN) de-identified U.S. administrative claims (2000–2013). We calculated incidence and prevalence of three outcomes: ≥1 diagnosis code for 1) pericarditis and/or myocarditis, 2) endocarditis or 3) valvular insufficiency. Negative binomial regression was used to identify characteristics associated with cardiac diagnoses in children and determine whether SLE onset in childhood versus adulthood was independently associated with cardiac involvement. Results: There were 297 children and 6927 adults with new-onset SLE. 17.8% of children had ICD-9 codes for acute cardiac diagnoses, the incidence of which were highest in the first year after SLE diagnosis (12.2 per 100 person-years). African American race (incidence rate ratio (IRR) 6.6, 95% CI [2.9, 15.0], p<0.01) and nephritis (IRR 7.0, 95% CI [2.6, 18.6], p<0.01) were associated with acute cardiac diagnoses in children. Child-onset disease was independently associated with a 4.4-fold higher rate of pericarditis or myocarditis compared to adult-onset SLE after adjustment for other disease and demographic characteristics (95% CI [2.4, 8.0], p<0.01). Conclusion: This study establishes baseline estimates of the incidence and prevalence of pericarditis and myocarditis in child-onset SLE, which is substantially higher than that of adultonset SLE. Prospective echocardiographic evaluations are needed to validate incidence measures and characterize the natural history of acute cardiac manifestations in child-onset SLE, as well as identify risk factors for poor cardiac outcomes to inform screening and management.

Background: Pulmonary insufficiency (PI) and right ventricular (RV) dysfunction are long-term complications in rTOF. We sought to investigate RV contractile reserve and changes in PI that occur during exercise in patients with repaired tetralogy of Fallot (rTOF) and the association of these changes with exercise performance using stress echocardiography. Methods: Subjects with rTOF (n = 32) and healthy controls (n=10) were prospectively enrolled and underwent rest and peak exercise echocardiograms during standard CPET protocol on cycle ergometer or treadmill. RV contractile reserve was defined as the change in RV global longitudinal strain (RVGLS) from rest to peak exercise. PI was assessed with the diastolic systolic time velocity integral ratio (DSTVI) and diastolic/systolic velocity ratio (D/S Ratio) from pulmonary artery Doppler interrogation. Exercise measures included heart rate reserve, percent-predicted maximum oxygen consumption (%mVO2), percent-predicted maximum work (%mWork) and oxygen pulse. Results: Right ventricular contractile reserve was impaired in rTOF compared to controls with a significant drop in the absolute value of RVGLS from 17% (range 8-27) at rest to 13% (range 5-28) at peak exercise. Similarly, PI decreased at peak exercise, with a drop in DSTVI and D/S ratios. Reduction in PI was directly associated with %mVO2, %mWork, and greater oxygen pulse. Heart rate reserve was directly associated with %mVO2 and %mWork. RV contractile reserve was not associated with any exercise parameters. Conclusions: Patients with rTOF have an abnormal myocardial response to exercise with impaired RV contractile reserve compared to control subjects. Heart rate reserve and reduction in PI at peak exercise are associated with better exercise performance and appear to be significant contributors to exercise performance in rTOF. Measures to improve chronotropic health in rTOF should be explored.

BACKGROUND Children with single ventricle heart disease have significant morbidity and mortality. The maternal–fetal environment (MFE) may adversely impact outcomes after neonatal cardiac surgery. We hypothesized that impaired MFE would be associated with an increased risk of death after stage 1 Norwood reconstruction. METHODS AND RESULTS We performed a retrospective cohort study of children with hypoplastic left heart syndrome (and anatomic variants) who underwent stage 1 Norwood reconstruction between 2008 and 2018. Impaired MFE was defined as maternal gestational hypertension, preeclampsia, gestational diabetes, and/or smoking during pregnancy. Cox proportional hazards regression models were used to investigate the association between impaired MFE and death while adjusting for confounders. Hospital length of stay was assessed with the competing risk of in‐hospital death. In 273 children, the median age at stage 1 Norwood reconstruction was 4 days (interquartile range [IQR], 3–6 days). A total of 72 children (26%) were exposed to an impaired MFE; they had more preterm births (18% versus 7%) and a greater percentage with low birth weights <2.5 kg (18% versus 4%) than those without impaired MFE. Impaired MFE was associated with a higher risk of death (hazard ratio [HR], 6.05; 95% CI, 3.59–10.21; P <0.001) after adjusting for age at surgery, Hispanic ethnicity, genetic syndrome, cardiac diagnosis, surgeon, and birth era. Children with impaired MFE had almost double the risk of prolonged hospital stay (HR, 1.95; 95% CI, 1.41–2.70; P <0.001). CONCLUSIONS Children exposed to an impaired MFE had a higher risk of death following stage 1 Norwood reconstruction. Prenatal exposures are potentially modifiable factors that can be targeted to improve outcomes after pediatric cardiac surgery.