The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices

Abstract

Objective: To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies.

Methods: A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy.

Results: In anti-Ro/SSA positive women, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely. 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. To prevent CHB, HCQ was recommended by 67% of respondents and most would start pre-pregnancy (62%). Respondents were asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. 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.

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.

Fig. 1

Weeks of gestation when respondents reported fetal echocardiograms should be started and stopped in pregnancy (n = 43) Each line is the duration of fetal echocardiograms recommended by a single respondent.

Fig. 2

Treatment practices for first degree, second degree and third degree heart block Treatment for first degree, second degree and third degree heart block in a late-20s woman with SLE and positive anti-Ro and La (SSA and SSB) antibodies who is 20 weeks pregnant. Her lupus has been quiescent for several years and she is not taking any medications (n = 49). Note: respondents were allowed to select multiple responses; therefore, percentages do not add up to 100%.

Fig. 3

Adjustments to dexamethasone treatment for second degree heart block based on follow-up fetal echocardiograms Change in dexamethasone treatment for second degree heart block when the heart block disappears and fetal ECHO is normal (n = 30); the second degree heart block remains (n = 29); and the heart block progresses to complete/third degree heart block (n = 36).

Fig. 4

Management of the anti-Ro ± anti-La pregnancy, as suggested by Jill Buyon, MD