The presence of maternal
autoantibodies to SS-A/Ro and/or SS-B/La is associated with the
development of fetal heart block. There are data suggesting that
maternal treatment with steroids might reverse heart block. We report on
a pregnancy in a mother with secondary Sjögren syndrome and systemic
lupus erythematosus with presence of autoantibodies to SS-A/Ro and
SS-B/La, which was complicated by the development of incomplete fetal
heart block. Oral dexamethasone treatment could not prevent progression
to complete heart block and was associated with a number of
complications.
A review of the literature
revealed 19 studies (including ours) in which 93 cases of fetal heart
block were treated with maternal steroid therapy. Complete heart block
proved irreversible in all cases; and of 13 fetuses with incomplete
heart block which received maternal steroid therapy, three had a
reduction in their degree of block and one reverted to sinus rhythm.
Maternal steroid therapy, initiated early in pregnancy and potentially
preventing the onset of heart block, did not decrease the incidence of
heart block in nine studies with 43 cases. Furthermore, the literature
review revealed numerous serious side effects of maternal steroid
administration during pregnancy. Data on these potential side effects
are lacking in the 28 studies discussed in this review.
Maternal
dexamethasone therapy to prevent or treat fetal heart block remains, in
our opinion, a questionable intervention and can as yet not be
recommended in the clinical situation. Copyright © 2004 ISUOG. Published
by John Wiley & Sons, Ltd.
Introduction
Fetal heart block occurs in 1 in 15 000–22 000 live births1.
Associated structural heart defects are responsible for the conduction
disturbance in 50% of all cases. In the other half, fetal heart block
occurs as an isolated lesion in a structurally normal heart. In these
cases, maternal autoantibodies conducted to SS-A/Ro and/or SS-B/La
proteins cross the placenta from as early as 16 weeks' gestational age
(GA) and initiate inflammatory damage to the fetal conduction system and
the myocardium. These passively acquired maternal autoantibodies may
also cause skin lesions: cutaneous neonatal lupus erythematosus (NLE).
NLE and isolated fetal heart block together compose the neonatal lupus
syndrome2.
Autoantibodies to SS-A/Ro and SS-B/La are mainly found in the sera of
patients with Sjögren syndrome and patients with systemic lupus
erythematosus (SLE)3.
Women with these autoantibodies have a risk of 1–5% of a pregnancy
complicated by heart block. Recurrence risks have been reported from 10%
to as high as 40%4–6.
Maternal steroid therapy has widely been used in anti-SS-A/Ro- and/or
anti-SS-B/La-positive pregnancies at risk of developing heart block. In
the case of treatment of underlying maternal disease or in pregnancies
at high risk for the development of heart block, maternal steroid
therapy may potentially prevent the occurrence of heart block when
initiated early during pregnancy in an asymptomatic fetus. Once
established, heart block may respond to maternal steroid therapy with
lessening of the degree of block7–11.
We
describe a case of isolated incomplete fetal heart block treated with
maternal steroid therapy in which therapy did not result in resolution
of heart block but was associated with a number of complications. The
literature is reviewed, with special emphasis on efficacy and safety of
maternal steroid therapy.
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Read More = http://onlinelibrary.wiley.com/doi/10.1002/uog.1713/full
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