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619 Prospective Evaluation of anti-SSA/Ro Positive Pregnancies to Address Risk Factors for Fetal Cardiac Disease/Adverse Pregnancy Outcomes and Efficacy of Ambulatory Fetal Heart Rate Monitoring (FHRM) and Rapid Treatment of Emergent Block
  1. Jill Buyon1,
  2. Kristina Deonaraine2,
  3. Philip Carlucci1,
  4. Mala Masson1,
  5. Nicola Fraser1,
  6. Colin Phoon1,
  7. Ashley Roman1,
  8. Peter Izmirly1,
  9. Amit Saxena1,
  10. Michael Belmont1,
  11. Christina Penfield1,
  12. Young Mi Lee1,
  13. Julie Nusbaum1,
  14. Bruce Solitar1,
  15. Fardina Malik1,
  16. Paula Rackoff1,
  17. Rebecca Haberman1,
  18. Ruben Acherman3,
  19. Elena Sinkovskaya4,
  20. Alfred Albuhamad4,
  21. Majd Makhoul5,
  22. Gary Satou6,
  23. Nelangi Pinto7,
  24. Anita Moon-Grady8,
  25. Lisa Howley9,
  26. Stephanie Levasseur10,
  27. Jyothi Matta11,
  28. Christopher Lindblade12,
  29. Andrew Rubenstein13,
  30. Caitlin Haxel14,
  31. Katherine Kohari15,
  32. Joshua Copel15,
  33. James Strainic16,
  34. Tam Doan17,
  35. Karla Bermudez-Wagner17,
  36. Shreya Sunil Sheth17,
  37. Stacy Killen18,
  38. Theresa Tacy19,
  39. Michelle Kaplinski19,
  40. Bailey Drewes20,
  41. Robert Clancy1 and
  42. Bettina Cuneo20
  1. 1NYU Langone Health, USA
  2. 2University at Buffalo, USA
  3. 3Children’s Heart Center, USA
  4. 4East Virginia Medical School, USA
  5. 5University of Kentucky, USA
  6. 6University of California, USA, Los Angeles, USA
  7. 7University of Utah, USA
  8. 8University of California, San Francisco, USA
  9. 9Midwest Fetal Care Center, Children’s Minnesota/Allina Health, USA
  10. 10Columbia University, USA
  11. 11University of Louisville, USA
  12. 12Phoenix Children’s Hospital, USA
  13. 13Dignity Health, USA
  14. 14University of Vermont Children’s Hospital, USA
  15. 15Yale University, USA
  16. 16University Hospitals Rainbow Babies, USA
  17. 17Baylor School of Medicine, USA
  18. 18Vanderbilt University, USA
  19. 19Stanford University, USA
  20. 20University of Colorado, Denver, USA


Introduction Fetal cardiac disease is strongly associated with maternal anti-SSA/Ro antibodies, but gaps in our knowledge include the influence of antibody specificity and titer, maternal diagnosis, overall non-cardiac adverse pregnancy outcomes (APOs), optimal surveillance protocols, and efficacy of rapid treatment.

Methods The multi-center Surveillance and Treatment To Prevent Fetal AV Block Likely to Occur Quickly (STOP BLOQ) study recruited pregnant women with commercially positive anti- Ro antibodies and stratified them into high and low titers of anti-Ro60 and Ro52 based on a research ELISA, using a cutoff defined by that obtained for 50 mothers with previous AVB offspring. Mothers with anti-Ro60 and/or 52 antibodies at or above 1,000 I.U. were trained to perform FHRM. From 17- 25 weeks of gestation, FHRM was completed 3x/day in addition to weekly or biweekly fetal echocardiograms (echo). Mothers texted all audio sounds to the coordinating center. Texts deemed abnormal by mothers were immediately sent to an on call pediatric cardiologist who either reassured if FHRM was normal or referred for emergency fetal echo in < 6 hours if abnormal.

Results 250 anti-Ro pregnant women (22% Hispanic, 50% white, 12% Black, 12% Asian, 4% other) have been consented, including 28 whose previous child had AVB. Of mothers tested to date, 153 were provided home monitors given high titer anti-Ro60 and/or 52 antibodies (26 high titer anti-Ro60 alone, 21 high titer anti-Ro52 alone,105 high titer antibodies to both antigens).

The 83 patients with low titers were surveilled with echos per local standard of care. Regarding maternal diagnosis, of 161 assessed to date, 39% were asym/UAS, 11% RA, 31% SS, 19% SLE. Antibody titers did not significantly differ by ethnicity, race or diagnosis (table 1). Non- AVB APOs occurred in 18% and were not predicted by Ro60 or 52 titers but rather SLE diagnosis (table 2). In total, 24,759 FHRM audiotexts were received from 131 patients (90 of whom have delivered) during the monitoring period. Of these, 22 were evaluated by the on-call pediatric cardiologist, who prompted an emergency echo (all completed in < 6 hrs). In 11 cases, the emergency echo was normal. In 9, there were premature atrial contractions, confirming the mother’s perception. In 2 with 2° block on urgent echo (both treated per protocol with IVIG and dexamethasone), 1 reverted to normal sinus rhythm and the other progressed to 3° block. In 2 others, the mother did not perceive abnormal FHRM for > 24 hrs, echo identified 3° block, and retrospective cardiology review of FHRM audio captures identified an abnormality prior to obtaining the echo. All 4 AVB developed in fetuses of mothers with high titer antibodies to both Ro60 and 52 (mean 32,451 and 34,991 respectively). Of the 18 mothers with a previous AVB child who followed the 400mg hydroxychloroquine PATCH protocol, 1 developed AVB in accord with the results of Step 1 in that study.

Conclusion These data support that APOs in this clinically diverse group of mothers are not influenced by anti-Ro titer or specificity, but rather SLE diagnosis. All conduction defects were initially identified by FHRM and in mothers with high titer anti-Ro60 and 52. Hydroxychloroquine continues to show efficacy in reducing the AVB recurrence rate with rapid intervention of emergent block being promising.

Abstract 619 Table 1

Antibody titers by race/ethnicity and maternal diagnosis.

Abstract 619 Table 2

Adverse pregnancy outcomes by maternal diagnosis.

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: .

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