AUTOANTIBODY-ASSOCIATED CONGENITAL HEART-BLOCK - OUTCOME IN MOTHERS AND CHILDREN

被引:238
作者
WALTUCK, J [1 ]
BUYON, JP [1 ]
机构
[1] NYU, HOSP JOINT DIS, SCH MED, NEW YORK, NY 10003 USA
关键词
HEART BLOCK; AUTOANTIBODIES; AUTOIMMUNE DISEASES; RHEUMATIC DISEASES; PACEMAKER; ARTIFICIAL;
D O I
10.7326/0003-4819-120-7-199404010-00003
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:544 / 551
页数:8
相关论文
共 54 条
[1]   ANTI-RO SS-A ANTIBODIES IN THE PATHOPHYSIOLOGY OF CONGENITAL HEART-BLOCK IN NEONATAL LUPUS SYNDROME, AN EXPERIMENTAL-MODEL - INVITRO ELECTROPHYSIOLOGIC AND IMMUNOCYTOCHEMICAL STUDIES [J].
ALEXANDER, E ;
BUYON, JP ;
PROVOST, TT ;
GUARNIERI, T .
ARTHRITIS AND RHEUMATISM, 1992, 35 (02) :176-189
[2]   THE IMMUNOGENETIC RELATIONSHIP BETWEEN ANTI-RO(SS-A)/LA(SS-B) ANTIBODY POSITIVE SJOGREN LUPUS-ERYTHEMATOSUS OVERLAP SYNDROME AND THE NEONATAL LUPUS SYNDROME [J].
ALEXANDER, EL ;
MCNICHOLL, J ;
WATSON, RM ;
BIAS, W ;
REICHLIN, M ;
PROVOST, TT .
JOURNAL OF INVESTIGATIVE DERMATOLOGY, 1989, 93 (06) :751-756
[3]  
[Anonymous], ARCH MED ENFANTS
[4]  
BABOONIAN C, 1989, CLIN EXP IMMUNOL, V78, P454
[5]   TRANSPLACENTAL PASSAGE OF PREDNISONE AND PREDNISOLONE IN PREGNANCY NEAR TERM [J].
BEITINS, IZ ;
BAYARD, F ;
ANCES, IG ;
KOWARSKI, A ;
MIGEON, CJ .
JOURNAL OF PEDIATRICS, 1972, 81 (05) :936-+
[6]   FETAL HYDROPS AND CONGENITAL COMPLETE HEART-BLOCK - RESPONSE TO MATERNAL STEROID-THERAPY [J].
BIERMAN, FZ ;
BAXI, L ;
JAFFE, I ;
DRISCOLL, J .
JOURNAL OF PEDIATRICS, 1988, 112 (04) :646-648
[7]   INVITRO METABOLISM OF PREDNISOLONE, DEXAMETHASONE, BETAMETHASONE, AND CORTISOL BY HUMAN PLACENTA [J].
BLANFORD, AT ;
MURPHY, BEP .
AMERICAN JOURNAL OF OBSTETRICS AND GYNECOLOGY, 1977, 127 (03) :264-267
[8]   CONGENITAL COMPLETE HEART-BLOCK - A HUMAN-MODEL OF PASSIVELY ACQUIRED AUTOIMMUNE INJURY [J].
BUYON, JP ;
WINCHESTER, R .
ARTHRITIS AND RHEUMATISM, 1990, 33 (05) :609-614
[9]   ACQUIRED CONGENITAL HEART-BLOCK - PATTERN OF MATERNAL ANTIBODY-RESPONSE TO BIOCHEMICALLY DEFINED ANTIGENS OF THE SSA/RO-SSB/LA SYSTEM IN NEONATAL LUPUS [J].
BUYON, JP ;
BENCHETRIT, E ;
KARP, S ;
ROUBEY, RAS ;
POMPEO, L ;
REEVES, WH ;
TAN, EM ;
WINCHESTER, R .
JOURNAL OF CLINICAL INVESTIGATION, 1989, 84 (02) :627-634
[10]   IDENTIFICATION OF MOTHERS AT RISK FOR CONGENITAL HEART-BLOCK AND OTHER NEONATAL LUPUS SYNDROMES IN THEIR CHILDREN - COMPARISON OF ENZYME-LINKED-IMMUNOSORBENT-ASSAY AND IMMUNOBLOT FOR MEASUREMENT OF ANTI-SS-A RO AND ANTI-SS-B LA ANTIBODIES [J].
BUYON, JP ;
WINCHESTER, RJ ;
SLADE, SG ;
ARNETT, F ;
COPEL, J ;
FRIEDMAN, D ;
LOCKSHIN, MD .
ARTHRITIS AND RHEUMATISM, 1993, 36 (09) :1263-1273