Background/Purpose:

All women with anti-Ro antibodies face the risk of cardiac neonatal lupus (cardiac NL), which presents as congenital heart block (CHB) and/or cardiomyopathy in their offspring. While several groups report risk factors for short-term outcomes including mortality, follow up studies beyond infancy are scant.  Such information is critical to family counseling as well as formulating strategies for management. 

Methods:

Postnatal echocardiograms most distant to birth were evaluated in 178 children with cardiac NL enrolled in the Research Registry for Neonatal Lupus (mean date of service 2/25/2012 ± 2.51 y).   M-mode measurements were analyzed for abnormalities (z score > +2.0) of cardiac and aortic dimensions, as well as qualitative descriptions of abnormal ventricular function, chamber enlargement, and valvular dysfunction (at least moderate stenosis or regurgitation).  Bivariate analyses of outcomes were performed with potential risk factors for morbidity including demographics, maternal medications during pregnancy, fetal heart rate at time of detection and lowest documented fetal rate, fetal echo dilated cardiomyopathy (DCM), endocardial fibroelastosis or hydrops, postnatal septal defects, permanent pacemaker (PPM) placement, age at PPM implantation and number of years with PPM.

Results:

Echo reports were available for 15 (8.4%) children age 0-1, 40 (22.5%) >1-5, 34 (19.1%) >5-10, 26 (14.6%) >10-15, 33 (18.5%) >15-20, and 30 (16.9%) >20.  Left ventricular (LV) systolic function was qualitatively abnormal in 13.7% and low-normal in 9.1%.  Abnormal LV function was more frequent in age groups 0-1 (26.7%) and >20 (26.7%) compared to others (p=0.004).  In all ages, decreased LV function associated with lower fetal heart rate at detection of CHB (p=0.012) and nadir rate (p=0.002), as well as fetal DCM (p=0.035) and hydrops (p=0.048).  Abnormal LV function also associated with need for PPM (p=0.029), younger age at PPM implantation (p=0.042), longer period of pacing (p=0.014), atrial septal defect (p=0.012) and black race (p=0.019).  LV end diastolic dimension was enlarged in 21.5% by z score and 20.6% qualitatively.  LV dilation associated with fetal DCM (p=0.04) and black race (p=0.028).  The aortic root and/or ascending aorta were dilated in 27.8%, associated with a younger age (p=0.001) and lower fetal ventricular rate at detection (p=0.04).  Valvular disease was present in 8.7%, associated with older age (p=0.001), fetal DCM (p=0.033), PPM (p=0.042) and longer period of pacing (p<0.001).

Conclusion:

The majority of cardiac NL children have normal heart size and function on follow up, however over 20% have abnormalities.  Depressed LV function is more frequent in the first year of life, possibly due to the ongoing inflammatory insult from anti-Ro exposure in utero.  Decreased function is more common again after age 20, which may be a result of long-term pacing.  Known risk factors for mortality, including race and fetal factors (heart rate, DCM and hydrops) also predict long-term cardiac dysfunction.  Aortic dilation is relatively common, but is noted less frequently in older individuals.  Further studies are required to identify optimal pacing techniques and medical management to prevent complications of cardiac NL.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-outcomes-in-cardiac-neonatal-lupus-and-associated-risk-factors-for-morbidity/