Congenital heart block associated with Sjögren syndrome: Case report

Cancer Sciences Division, School of Medicine, University of Southampton, Southampton SO16 6YD, UK.
International Archives of Medicine (Impact Factor: 1.08). 08/2009; 2(1):21. DOI: 10.1186/1755-7682-2-21
Source: PubMed


Congenital heart block is a rare complication of pregnancy associated with Sjögren Syndrome that may result in the death of the foetus or infant, or the need for pacing in the newborn or at a later stage.
The case is presented of a 64-year-old patient with primary Sjögren Syndrome and a history of having given birth to two sons with congenital heart block, both of whom required pacing several years later.
The literature relating to this association is discussed including the suggested mechanism, long-term outcome of mothers of children with congenital heart block and preventive treatment strategies.

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    • "Gestational impairments are important factors to be investigated [1,2], since it affects children development [3-5]. The hydatidiform mole (HM) is a variant of gestational trophoblastic disease, a generic term that brings together different conditions of proliferation of trophoblastic epithelium, with abnormal functioning tissue producing human chorionic gonadotropin (hCG) [6]. "
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    ABSTRACT: OBJECTIVE: congenital heart block may develop in the fetus of women with Ro/SSA autoantibodies. The aim of this study was to investigate how women expecting a child with congenital heart block (CHB) experienced their pregnancy and post-partum period. DESIGN, SETTING AND PARTICIPANTS: women giving birth to a child with CHB in Sweden during 2000-2009 were identified in a population-based manner and individually interviewed post-pregnancy using a semi-structured interview guide. The interviews (n=21) were audiotaped, transcribed verbatim and analysed by qualitative content analysis. FINDINGS: three categories emerged from the responses: learning, suspense and facing. Learning contained both learning about the child's heart block, but frequently also about autoantibody-positivity and a potential rheumatic diagnosis in the mother (16/21). The medical procedures and information differed considerably depending on the area of residence and who was encountered in the health-care system. In many cases, ignorance about this rare condition caused a delay in treatment and surveillance. Suspense described the women's struggle to cope with the feeling of guilt and that the child had a serious heart condition and might not survive the pregnancy. Facing included the post-partum period, leaving the hospital and adjusting to everyday life. The women had tended to put their pregnancies 'on hold', and some described that they needed prolonged time to bond with their newborn child. CONCLUSION: increased awareness and knowledge of CHB are needed to provide adequate care. Offering psychological support by a health-care professional to facilitate early bonding with the child should be considered. IMPLICATIONS FOR PRACTICE: there is a need for structured programs for surveillance of the pregnancies. Such programme should implement guidelines for the involved personnel in the chain of care and make relevant information accessible for the women and families.
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