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
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页码:544 / 551
页数:8
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