Surveillance and Treatment to Prevent Fetal Atrioventricular Block Likely to Occur Quickly (STOP BLOQ)

Description

Fetal complete (i.e., third degree, 3°) atrioventricular block (AVB), identified in the 2nd trimester of pregnancy in an otherwise normally developing heart, is almost universally associated with maternal anti-Ro autoantibodies and results in death in a fifth of cases. To date treatment of 3° AVB has been ineffective in restoring normal rhythm (NR) which may be because current surveillance is limited to once- weekly fetal echocardiograms. It is hypothesized that there may be a vital transition period of several hours in which incomplete block (2° AVB) may be successfully treated avoiding fully advanced irreversible 3° AVB. To optimize the likelihood of timely detection of the transition period this study comprises three steps: 1) to risk stratify for high titer anti-Ro antibodies, which are necessary but not sufficient to develop fetal AVB; 2) to empower mothers to identify 2° AVB by using fetal heart rate and rhythm monitoring (FHRM) at home, and 3) to rapidly treat mothers who detect an abnormality by monitoring with an urgent echocardiogram that confirms 2° AVB with the hope of reversing 2° AVB before it becomes permanent (3° AVB). In addition, it will be determined if FHRM reduces the need for weekly echoes. Although mothers with low titer anti-Ro will not be continued in Step 2 and therefore not followed by FHRM, birth ECGs will be collected to confirm that low titer antibodies do not confer risk. It is anticipated that this study will provide an evidenced based surveillance strategy for those mothers at high risk of having a child with 3° AVB.

Conditions

AVB - Atrioventricular Block, Fetal AVB

Study Overview

Study Details

Study overview

Fetal complete (i.e., third degree, 3°) atrioventricular block (AVB), identified in the 2nd trimester of pregnancy in an otherwise normally developing heart, is almost universally associated with maternal anti-Ro autoantibodies and results in death in a fifth of cases. To date treatment of 3° AVB has been ineffective in restoring normal rhythm (NR) which may be because current surveillance is limited to once- weekly fetal echocardiograms. It is hypothesized that there may be a vital transition period of several hours in which incomplete block (2° AVB) may be successfully treated avoiding fully advanced irreversible 3° AVB. To optimize the likelihood of timely detection of the transition period this study comprises three steps: 1) to risk stratify for high titer anti-Ro antibodies, which are necessary but not sufficient to develop fetal AVB; 2) to empower mothers to identify 2° AVB by using fetal heart rate and rhythm monitoring (FHRM) at home, and 3) to rapidly treat mothers who detect an abnormality by monitoring with an urgent echocardiogram that confirms 2° AVB with the hope of reversing 2° AVB before it becomes permanent (3° AVB). In addition, it will be determined if FHRM reduces the need for weekly echoes. Although mothers with low titer anti-Ro will not be continued in Step 2 and therefore not followed by FHRM, birth ECGs will be collected to confirm that low titer antibodies do not confer risk. It is anticipated that this study will provide an evidenced based surveillance strategy for those mothers at high risk of having a child with 3° AVB.

Surveillance and Treatment to Prevent Fetal Atrioventricular Block Likely to Occur Quickly (STOP BLOQ)

Surveillance and Treatment to Prevent Fetal Atrioventricular Block Likely to Occur Quickly (STOP BLOQ)

Condition
AVB - Atrioventricular Block
Intervention / Treatment

-

Contacts and Locations

Phoenix

Phoenix Children's Hospital/Dignity Health, Phoenix, Arizona, United States, 85016

Los Angeles

University of California - Los Angeles (UCLA), Los Angeles, California, United States, 90095

Palo Alto

Stanford University, Palo Alto, California, United States, 94305

San Francisco

University of California-San Francisco, San Francisco, California, United States, 94143

Aurora

University of Colorado, Denver (UCD), Aurora, Colorado, United States, 80204

New Haven

Yale University School of Medicine, New Haven, Connecticut, United States, 06510

Washington

Children's National Medical Center/George Washington University, Washington, District of Columbia, United States, 20010

Lexington

University of Kentucky / Kentucky Children's Hospital, Lexington, Kentucky, United States, 40536

Louisville

University of Louisville / Norton Children's Hospital, Louisville, Kentucky, United States, 40202

Ann Arbor

University of Michigan / C. S. Mott Children's Hospital, Ann Arbor, Michigan, United States, 48109

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

For general information about clinical research, read Learn About Studies.

Eligibility Criteria

  • 1. Provision of signed and dated informed consent form
  • 2. Stated willingness to comply with all study procedures and availability for the duration of the study
  • 3. Be \<18 weeks pregnant at the time of enrollment
  • 4. Titer of anti-Ro 52 or 60 antibodies ≥1,000 EU
  • 5. Any positive titer of anti-Ro if a history of a previously affected child
  • 6. Ability to take oral medication and be willing to adhere to the dexamethasone and IVIG protocols.
  • 7. Ability to perform Doppler fetal heart rate and rhythm monitoring in the ambulatory setting,
  • 8. Ability to send an audiotext message by cell phone therefore the participant will be informed that they need a phone with texting capabilities. Located within 6 hours drive of the participating pediatric cardiology site
  • 9. Be ≥18 years of age
  • 1. Multi-fetal pregnancy
  • 2. Known allergic reactions to components of IVIG, or dexamethasone or maternal IgA deficiency
  • 3. Fetal conduction system disease already present in the current pregnancy
  • 4. Any women who in the opinion of the investigator cannot understand the consent form or be able to perform thrice daily home monitoring or recognize an abnormal fetal heart rate or rhythm
  • 5. Women prisoners
  • 6. Treatment with \>20 mg/prednisone q day or with any dose of fluorinated steroids at enrollment

Ages Eligible for Study

18 Years to

Sexes Eligible for Study

FEMALE

Accepts Healthy Volunteers

No

Collaborators and Investigators

NYU Langone Health,

Jill Buyon, MD, PRINCIPAL_INVESTIGATOR, NYU Langone Health

Bettina Cuneo, MD, PRINCIPAL_INVESTIGATOR, University of Colorado, Denver

Study Record Dates

2026-10-31