 |
 |
|
Home |
Current Issue |
Past Issues |
In the Clinic |
ACP Journal Club |
CME |
Collections |
Audio/Video |
Mobile |
Subscribe |
Tools |
Help |
ACP Online
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 April 1994 | Volume 120 Issue 7 | Pages 544-551
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.
Author and Article Information
From the Hospital for Joint Diseases, New York University School of Medicine, New York, New York.
ARTICLE
Autoantibody-associated Congenital Heart Block: Outcome in Mothers and Children
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).
This article has been cited by other articles:
|
|
 |
|
  M. E. Mangoni and J. Nargeot Genesis and Regulation of the Heart Automaticity Physiol Rev, July 1, 2008; 88(3): 919 - 982. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J.P. Buyon and R.M. Clancy Dying right to live longer: positing apoptosis as a link between maternal autoantibodies and congenital heart block Lupus, February 1, 2008; 17(2): 86 - 90. [Abstract] [PDF]
|
|
|
|
 |
|
 D. M. Friedman, M. Y. Kim, J. A. Copel, C. Davis, C. K.L. Phoon, J. S. Glickstein, J. P. Buyon, and for the PRIDE Investigators Utility of Cardiac Monitoring in Fetuses at Risk for Congenital Heart Block: The PR Interval and Dexamethasone Evaluation (PRIDE) Prospective Study Circulation, January 29, 2008; 117(4): 485 - 493. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P.A. Gordon Review: Congenital heart block: clinical features and therapeutic approaches Lupus, August 1, 2007; 16(8): 642 - 646. [Abstract] [PDF]
|
|
|
|
 |
|
 A. G. Tzioufas and H. M. Moutsopoulos Predicting autoimmune congenital heart block: is it feasible and how? Rheumatology, August 1, 2007; 46(8): 1221 - 1222. [Full Text] [PDF]
|
|
|
|
|
|
L Strandberg, S Salomonsson, K Bremme, S-E Sonesson, and M Wahren-Herlenius Ro52, Ro60 and La IgG autoantibody levels and Ro52 IgG subclass profiles longitudinally throughout pregnancy in congenital heart block risk pregnancies Lupus, June 1, 2006; 15(6): 346 - 353. [Abstract] [PDF]
|
|
|
|
|
|
N Costedoat-Chalumeau, S Georgin-Lavialle, Z Amoura, and J-C Piette Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block Lupus, September 1, 2005; 14(9): 660 - 664. [Abstract] [PDF]
|
|
|
|
|
|
Y. Qu, G. Baroudi, Y. Yue, and M. Boutjdir Novel Molecular Mechanism Involving {alpha}1D (Cav1.3) L-Type Calcium Channel in Autoimmune-Associated Sinus Bradycardia Circulation, June 14, 2005; 111(23): 3034 - 3041. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G E Katsifis and A G Tzioufas Ovarian failure in systemic lupus erythematosus patients treated with pulsed intravenous cyclophosphamide Lupus, September 1, 2004; 13(9): 673 - 678. [Abstract] [PDF]
|
|
|
|
|
|
J P Buyon, A Rupel, and R M Clancy Neonatal lupus syndromes Lupus, September 1, 2004; 13(9): 705 - 712. [Abstract] [PDF]
|
|
|
|
 |
|
 K. Hu, Y. Qu, Y. Yue, and M. Boutjdir Functional Basis of Sinus Bradycardia in Congenital Heart Block Circ. Res., March 5, 2004; 94 (4): e32 - e38. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 N Costedoat-Chalumeau, Z Amoura, D Le Thi Hong, B Wechsler, D Vauthier, P Ghillani, T Papo, O Fain, L Musset, and J-C Piette Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block Ann Rheum Dis, October 1, 2003; 62(10): 1010 - 1012. [Abstract] [Full Text]
|
|
|
|
 |
|
 R. M. Clancy, C. B. Backer, X. Yin, R. P. Kapur, Y. Molad, and J. P. Buyon Cytokine Polymorphisms and Histologic Expression in Autopsy Studies: Contribution of TNF-{alpha} and TGF-{beta}1 to the Pathogenesis of Autoimmune-Associated Congenital Heart Block J. Immunol., September 15, 2003; 171(6): 3253 - 3261. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A Brucato, A Jonzon, D Friedman, L D Allan, G Vignati, M Gasparini, J I Stein, S Montella, M Michaelsson, and J Buyon Proposal for a new definition of congenital complete atrioventricular block Lupus, June 1, 2003; 12(6): 427 - 435. [Abstract] [PDF]
|
|
|
|
 |
|
 L. E. Nield, E. D. Silverman, J. F. Smallhorn, G. P. Taylor, J. Brendan, M. Mullen, L. N. Benson, and L. K. Hornberger Endocardial fibroelastosis associated with maternal anti-Ro and anti-La antibodies in the absence of atrioventricular block J. Am. Coll. Cardiol., August 21, 2002; 40(4): 796 - 802. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E Rosenthal Spectrum of conduction abnormalities associated with maternal anti-Ro antibodies Lupus, March 1, 2002; 11(3): 135 - 136. [PDF]
|
|
|
|
|
|
E. T. Jaeggi, R. M. Hamilton, E. D. Silverman, S. A. Zamora, and L. K. Hornberger Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block: A single institution's experience of 30 years J. Am. Coll. Cardiol., January 2, 2002; 39(1): 130 - 137. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G.-Q. XIAO, Y. QU, K. HU, and M. BOUTJDIR Down-regulation of L-type calcium channel in pups born to 52 kDa SSA/Ro immunized rabbits FASEB J, July 1, 2001; 15(9): 1539 - 1545. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B Maisch and A.D Ristic Immunological basis of the cardiac conduction and rhythm disorders Eur. Heart J., May 2, 2001; 22(10): 813 - 824. [PDF]
|
|
|
|
 |
|
 M. Restivo, D. O. Kozhevnikov, and M. Boutjdir Optical mapping of activation patterns in an animal model of congenital heart block Am J Physiol Heart Circ Physiol, April 1, 2001; 280(4): H1889 - H1895. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G.-Q. Xiao, K. Hu, and M. Boutjdir Direct Inhibition of Expressed Cardiac L- and T-Type Calcium Channels by IgG From Mothers Whose Children Have Congenital Heart Block Circulation, March 20, 2001; 103(11): 1599 - 1604. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
F. E. A. Udink ten Cate, J. M. P. J. Breur, M. I. Cohen, N. Boramanand, L. Kapusta, J. E. Crosson, J. I. Brenner, L. J. Lubbers, A. H. Friedman, V. L. Vetter, et al. Dilated cardiomyopathy in isolated congenital complete atrioventricular block: early and long-term risk in children J. Am. Coll. Cardiol., March 15, 2001; 37(4): 1129 - 1134. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. P. Moak, K. S. Barron, T. J. Hougen, H. B. Wiles, S. Balaji, N. Sreeram, M. H. Cohen, A. Nordenberg, G. F. Van Hare, R. A. Friedman, et al. Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela J. Am. Coll. Cardiol., January 1, 2001; 37(1): 238 - 242. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M.-E. Miranda-Carus, A. D. Askanase, R. M. Clancy, F. Di Donato, T.-M. Chou, M. R. Libera, E. K. L. Chan, and J. P. Buyon Anti-SSA/Ro and Anti-SSB/La Autoantibodies Bind the Surface of Apoptotic Fetal Cardiocytes and Promote Secretion of TNF-{alpha} by Macrophages J. Immunol., November 1, 2000; 165(9): 5345 - 5351. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Eronen, M.-K. Sirèn, H. Ekblad, T. Tikanoja, H. Julkunen, and T. Paavilainen Short- and Long-Term Outcome of Children With Congenital Complete Heart Block Diagnosed In Utero or as a Newborn Pediatrics, July 1, 2000; 106(1): 86 - 91. [Abstract] [Full Text]
|
|
|
|
|
|
C. P. Mavragani, J. P. A. Ioannidis, A. G. Tzioufas, I. E. Hantoumi, and H. M. Moutsopoulos Recurrent pregnancy loss and autoantibody profile in autoimmune diseases Rheumatology, December 1, 1999; 38(12): 1228 - 1233. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. SHINOHARA, S. MIYAGAWA, T. FUJITA, T. AONO, and K.-I. KIDOGUCHI Neonatal Lupus Erythematosus: Results of Maternal Corticosteroid Therapy Obstet. Gynecol., June 1, 1999; 93(6): 952 - 957. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. E. Miranda, C.-E Tseng, W. Rashbaum, R. L. Ochs, C. A. Casiano, F. Di Donato, E. K. L. Chan, and J. P. Buyon Accessibility of SSA/Ro and SSB/La Antigens to Maternal Autoantibodies in Apoptotic Human Fetal Cardiac Myocytes J. Immunol., November 1, 1998; 161(9): 5061 - 5069. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E Rosenthal New insights into the pathogenesis of anti-Ro antibody associated congenital complete heart block Lupus, March 1, 1998; 7(3): 135 - 136. [PDF]
|
|
|
|
|
|
E. Taylor-Albert, M. Reichlin, W. H. Toews, E. D. Overholt, and L. A. Lee Delayed Dilated Cardiomyopathy as a Manifestation of Neonatal Lupus: Case Reports, Autoantibody Analysis, and Management Pediatrics, May 1, 1997; 99(5): 733 - 733. [Full Text] [PDF]
|
|
|
|
|
|
A. C Oshiro, S. J Derbes, A. R Stopa, and A. Gedalia Anti-Ro/SS-A and anti-La/SS-B antibodies associated with cardiac involvement in childhood systemic lupus erythematosus Ann Rheum Dis, April 1, 1997; 56(4): 272 - 274. [Abstract] [Full Text]
|
|
|
|
|
|
M. Boutjdir, L. Chen, * Z.-H. Zhang, C.-E Tseng, * F. DiDonato, W. Rashbaum, A. Morris, N. El-Sherif, and J. P. Buyon Arrhythmogenicity of IgG and Anti–52-kD SSA/Ro Affinity-Purified Antibodies From Mothers of Children With Congenital Heart Block Circ. Res., March 1, 1997; 80(3): 354 - 362. [Abstract] [Full Text]
|
|
|
|
|
|
L. Lee and W. Weston Cutaneous lupus erythematosus during the neonatal and childhood periods Lupus, January 1, 1997; 6(2): 132 - 138. [Abstract] [PDF]
|
|
|
|
|
|
C-E. Tseng, F. Di Donato, and J. Buyon Stability of immunoblot profile of anti-SSA/Ro-SSB/La antibodies over time in mothers whose children have neonatal lupus Lupus, June 1, 1996; 5(3): 212 - 215. [Abstract] [PDF]
|
|
|
|
|
|
T. N. James, E. St. Martin, P. W. Willis III, and T. O. Lohr Apoptosis as a Possible Cause of Gradual Development of Complete Heart Block and Fatal Arrhythmias Associated With Absence of the AV Node, Sinus Node, and Internodal Pathways Circulation, April 1, 1996; 93(7): 1424 - 1438. [Abstract] [Full Text]
|
|
|
|
|
|
A. Gorelick, T. Oglesby, W. Rashbaum, J. Atkinson, and J.P. Buyon Ontogeny of membrane cofactor protein: phenotypic divergence in the fetal heart Lupus, August 1, 1995; 4(4): 293 - 296. [Abstract] [PDF]
|
|
|
|
|
|
J. Buyon, J. Waltuck, C. Kleinman, and J. Copel In Utero identification and therapy of congenital heart block Lupus, April 1, 1995; 4(2): 116 - 121. [Abstract] [PDF]
|
|
Article
