Congenital Heart Block from Neonatal Lupus: Prevention, Screening, and Treatment

Congenital heart block (CHB) is a rare occurrence, affecting about 1-to-5% babies born to women with systemic lupus erythematosus (SLE) or Sjögrens syndrome who have autoantibodies to the cellular proteins Ro and La. In a study published in Rheumatology, researchers conducted a survey of an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to these types of antibodies.  

the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases developed a survey, which was then sent to attendants of the conference and authors of recent publications or abstracts at the American College of Rheumatology (ACR) 2012, 2013or 2014 on rheumatic diseases and pregnancy.

According to the survey results, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), in anti-Ro/SSA positive women. The study results showed that for women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs; for women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. 

Respondents were then asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. Many respondents were consistent in their suggested treatments: 

  • For first degree, respondents recommended starting dexamethasone (53%) or HCQ (43%) 
  • For second degree, respondents recommended starting dexamethasone (88%) 
  • For third degree, respondents recommended starting dexamethasone (55%) or IVIg (33%) – although 27% would not start treatment 

“Despite the absence of official guidelines, many physicians with a focus on pregnancy and rheumatic disease have developed similar patterns in the screening, prevention and treatment of CHB,” the researchers concluded. 

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SOURCE: Rheumatology