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Autoantibody-Associated Congenital Heart Block: Outcome in Mothers and Children

Jonathan Waltuck, MD; and Jill P. Buyon, MD
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From the Hospital for Joint Diseases, New York University School of Medicine, New York, New York. Requests for Reprints: Jill P. Buyon, MD, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003. Acknowledgments: The authors thank the following physicians who generously provided serum samples and clinical histories on their patients: Drs. Carol Aitcheson, Frank Arnett, Balu Athreya, Laxmi Baxi, Seth Berney, Joshua Copel, Patricia Fraser, Richard Furie, Patricia Hopkins, Angela Horsfall, Paul Howard, Leslie Kahl, Jeffrey Korotkin, Bernhard Lang, Thomas Lehman, Michael Lockshin, Janice Lysiak, Joseph Miller, Renee Norberg, Ann Parke, Michelle Petri, Lisa Rider, Lisa Sammaritano, Philip Saul, Sandra Sessoms, Earl Silverman, Eng Tan, Patience White, Robert Winchester, and Samuel Zwillich. Grant Support: In part by a grant from the Arthritis Foundation, New York Affiliate (Dr. Buyon).

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1994;120(7):544-551. doi:10.7326/0003-4819-120-7-199404010-00003
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Objective: To determine the initial clinical status and the long-term outcome of mothers and their children with autoantibody-associated congenital heart block.

Design: Dynamic, longitudinal cohort study.

Patients: 55 children with isolated congenital heart block, their 52 mothers, and 5 other women currently carrying fetuses with congenital heart block. All maternal sera contained antibodies to SSA/Ro alone or to both SSA/Ro and SSB/La.

Measurements: Clinical information obtained from mailed questionnaires, telephone interviews, primary physicians, and chart reviews.

Results: When congenital heart block was identified in the children, 23 women were asymptomatic, 15 had systemic lupus erythematosus, 8 had the Sjogren syndrome, and 11 had an undifferentiated autoimmune syndrome. Follow-up ranged from 1 week to 20 years (median, 3.7 years). Eleven (48%) of the 23 initially asymptomatic mothers developed symptoms of a rheumatic disease (0.15 status changes/patient-year of follow-up; 6 [26%] developed an undifferentiated autoimmune syndrome, 2 (9%) developed the Sjogren syndrome, and 3 (13%) developed systemic lupus erythematosus. One mother with the Sjogren syndrome progressed to systemic lupus erythematosus. Four (16%) of 25 subsequent pregnancies in 22 women were complicated by heart block. Seventeen affected children died, 12 within 1 month of birth. Pacemakers were implanted in 37 (67%) of the 55 children, 27 within 3 months after birth.

Conclusion: The development of rheumatic disease in asymptomatic mothers identified by the birth of a child with congenital heart block is common but not universal. The risk for congenital heart block in subsequent pregnancies is low. One third of the children with autoantibody-associated congenital heart block die in the early neonatal period and, of those who survive, most require pacemakers.


Grahic Jump Location
Figure 1.
Timing of pacemaker implantation and death for 55 children with congenital heart block.

The x-axis represents various time periods from birth, and N represents the number of children remaining alive for any part of these designated periods. Thus, the decrease in N along the x-axis represents children not yet old enough to be included in the next age range and deaths that occurred in the previous age range. The y-axis represents the number of children who died and those who received pacemakers during these designated periods. For example, 12 children were followed for at least 5 years from birth and are included in the age range “5 to 10” years. None died and one had a pacemaker implanted for the first time, although some children needed pacemakers at earlier ages.

Grahic Jump Location




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