LONG-TERM OUTCOME OF MOTHERS OF CHILDREN WITH COMPLETE CONGENITAL HEART-BLOCK

OBJECTIVES:To determine the health of mothers of offspring with comple te congenital heart block (CHB) both at the time of delivery of the af fected child and in the long-term, and the percentage of mothers of ch ildren with CHB who had anti-SSA/Ro and/or SSB/La antibodies. PATIENTS AND METHODS: Sixty-four mothers of 64 children with CHB (seen between 1964 and 1993) were identified through the Cardiology database of The Hospital for Sick Children, Toronto, Canada. Medical information from these of children with CHB was evaluated. Data were obtained from the mothers by mailed questionnaire, telephone interview, and/or from the attending physicians. The presence of anti-Re antibodies and anti-La antibodies were evaluated by ELISA assay. RESULTS: The mean age at the time of delivery of the first child with CHB was 28 +/- 6 years. At t he time of delivery 42 (66%) mothers were healthy, 2 (3%) had systemic lupus erythematosus (SLE), 2 (3%) had linear scleroderma, 2 (3%) had rheumatoid arthritis; 3 (5%) had a history of rheumatic fever (but wer e otherwise well), 1 (2%) had Sjogren's syndrome (SS), and 12 (19%) ha d an undifferentiated autoimmune syndrome (UAS) (arthralgia, myalgia, photosensitivity, skin vasculitis, Raynaud's phenomenon). The mean tim e to follow-up from delivery to study was 121 +/- 88 months. The mean maternal age at study was 38 +/- 9 years. Three of 12 mothers who init ially had a UAS progressed to SLE (average follow-up time of 80 months , median 96), and 2 developed SS (with average follow-up time 140 mont hs, median 132) and 1 went into remission. The mean follow-up time for the other mothers who did not develop an autoimmune disease was 150 /- 102 months. Thirty-six of the 42 initially healthy mothers remained well. One mother developed SLE; 1 developed hyperthyroidism; 1 develo ped ankylosing spondylitis; and 3 developed an UAS. The mean follow-up time of the 36 mothers who remained healthy was similar (123 +/- 97 m onths) to the 6 initially healthy mothers who developed an autoimmune disease (121 +/- 36 months). Anti-Ro and/or anti-La antibodies were po sitive in 32 of 53 (60%) mothers tested. Fourteen of the 53 mothers we re symptomatic at the time of delivery and 39 were asymptomatic. Anti- Ro and/or anti-La antibodies were positive in 12 of 13 mothers tested at the time of delivery. CONCLUSIONS: The long-term maternal outcome i n our cohort was very good as most of the initially healthy mothers re mained well at follow-up. Twenty-five percent of the mothers with a UA S and only 2% of the initially healthy mothers developed SLE. The deve lopment of an autoimmune disease in an asymptomatic mother identified by the birth of a child with CHB was less common in our study than in previous studies. However, close follow-up of mothers with UAS is warr anted.