Background/Purpose: There are presently no official guidelines about the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro antibodies. The objective of this study was to survey an international sample of providers to determine their current practices.

Methods:  A survey was designed by the organizing committee of the 9^thInternational Conference or Reproduction, Pregnancy and Rheumatic Diseases. The survey was sent to 330 people who had attended prior conferences or were registered for this conference, were authors of recent publications on rheumatic diseases and pregnancy, or were authors of abstracts on rheumatic diseases and pregnancy from the ACR meetings in 2012, 2013, and 2014. Respondents who did not provide demographic information were excluded from the final analysis (n=11).

Results:   There were 48 respondents. Most (55%) follow >15 pregnancies in rheumatic patients per year, and 33% were practicing rheumatologists for >15 years. Most were university-based physicians (88%) and from North America (42%) or Europe (42%). Screening: For anti-Ro/SSA positive women, 80% recommended serial fetal ECHOs, with most starting at gestational week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely between weeks 22 and 34. For women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs, and 5% of respondents preferred a plan of two fetal echoes. For women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. Prevention: Hydroxychloroquine was recommended by 67% of respondents to prevent CHB and most would recommend starting the drug prior to pregnancy (62%). Treatment: Respondents were asked about medications for varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient.Respondents recommended dexamethasone (53%) or HCQ (43%) for 1st degree HB; dexamethasone (88%) for 2nd degree HB; and dexamethasone (55%), IVIg (33%), or no therapy (27%) for complete HB. When dexamethasone was started for 2nd degree CHB, 58% would stop dexamethasone if it progressed to complete heart block, 47% would stop if heart block disappeared, and 24% would stop if the 2nd degree CHB remained.

Conclusion:  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. These include serial fetal ECHOs, preventive HCQ, and treatment of early heart block with dexamethasone. These practices are not uniform, however, and have not been formally tested in prospective trials. The next step in this field must include testing of these approaches to identify the most cost effective and efficacious plan for these pregnancies.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-prevention-screening-and-treatment-of-congenital-heart-block-from-neonatal-lupus-a-survey-of-provider-practices/