Outcome and Growth of Infants Fetally Exposed to Heart Block-Associated Maternal Anti-Ro52/SSA Autoantibodies

Skog, Amanda; Wahren‐Herlenius, Marie; Sundström, Birgitta; Bremme, Katarina; Sonesson, Sven‐Erik

doi:10.1542/peds.2007-1659

Cited by 34 publications

(25 citation statements)

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“…Hydroxychloroquine, which was used in two women, has been not been found to affect fetal growth (20). The present study also confirms our previous observation that transient mid-trimester prolongation of AV-conduction is not a marker of a milder fetal disease affecting pregnancy outcome or fetal growth (12).…”

Section: Discussionsupporting

confidence: 90%

Outcome in 212 anti‐Ro/SSA‐positive pregnancies and population‐based incidence of congenital heart block

Skog

Lagnefeldt

Conner

et al. 2015

Acta Obstet Gynecol Scand

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Section: Discussionsupporting

confidence: 90%

Outcome in 212 anti‐Ro/SSA‐positive pregnancies and population‐based incidence of congenital heart block

Skog

Lagnefeldt

Conner

et al. 2015

Acta Obstet Gynecol Scand

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“…A trend towards higher maternal age for mothers who give birth to babies with heart block, compared to mothers who give birth to healthy babies, was previously reported by Skog et al 24. In the present study, we found that the risk of fetal heart block in Ro/La-positive pregnancies increased significantly with increasing maternal age, but was not influenced by parity.…”

Section: Discussionsupporting

confidence: 86%

Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern

et al. 2011

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Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern, 2012, Annals of the Rheumatic Diseases, (71) Methods. The influence of fetal gender, maternal age, parity and time of birth on heart block development was analyzed in 145 families including Ro/La-positive (n= 190) and Ro/Lanegative (n=165) pregnancies.Results. We observed a recurrence rate of 12.1% in Ro/La-positive women, and no recurrence in Ro/La-negative women. Fetal gender and parity did not influence the development of heart block in either group. Maternal age in Ro/La-positive pregnancies with a child affected by heart block was however significantly higher than in pregnancies resulting in babies without heart block (p<0.01). Further, seasonal timing of pregnancy influenced the outcome. Gestational susceptibility-weeks 18-24 occurring during January-March correlated with a higher proportion of heart block pregnancies and lower vitamin D levels, and a corresponding higher proportion of children with heart block born in the summer (p<0.02). Maternal age or seasonal timing of pregnancy did not affect the outcome in Ro/La-negative pregnancies.Conclusion. This study identifies maternal age and seasonal timing of pregnancy as novel risk factors for congenital heart block development in Ro/La positive pregnancy. These observations indicate that the risk can be modified, and will be important for counseling when a pregnancy is considered. 4Congenital complete heart block without cardiac malformation is a rare disease, affecting 1 in 15,000 to 20,000 births in the general population. An association with the presence of maternal autoantibodies to Ro/SSA and/or La/SSB is however well established [1,2], and the risk of complete congenital heart block is 1-2% in Ro/SSA-positive pregnancies [3][4][5][6]. Furthermore, the reported risk of giving birth to a second child with complete heart block for anti-Ro/SSA positive mothers ranges from 12 to 20% [7][8][9], despite the persistence of the maternal autoantibodies [10].This indicates that additional factors are critical for establishing the heart block. Fetal genetic susceptibility has been suggested as a potential risk factor [11,12], and polymorphisms in the gene encoding TGFβ have been implicated in the development of heart block [13,14]. Variations in the intrauterine environment between pregnancies have also been suggested to contribute to the penetrance of the disease. Maternal disease severity has been investigated as such a potential risk factor, but was not found to contribute to the development of congenital heart block [15].Given the rarity of congenital heart block occurrence in the general population, it is difficult to investigate potential risk factors associated with the disease. In particular, very little information is available on the influence of maternal age and parity on pregnancy outcome in anti-Ro/La positive mothers. In an effort to address these questions in a reasonable cohort we identified heart ...

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“…[438][439][440][441][442][443] Important complications of dexamethasone that have been reported include growth restriction, oligohydramios, ductal constriction (conveyed also by the collagen vascular disease itself), maternal DM, and central nervous system side effect. 441,444,445 Despite these potential complications, a trial of dexamethasone for second-degree AV block or first-degree AV block if there are additional cardiac findings of inflammation (echogenicity, valve regurgitation, cardiac dysfunction, effusion, etc) may be considered to prevent progression to CHB, although its usefulness is not well established. Dexamethasone treatment of fetuses with established CHB and no heart failure may also be considered with the goal of improving survival or reducing the incidence of dilated cardiomyopathy, although its usefulness has not been established given that studies to date have been retrospective and nonrandomized and have had incomplete follow-up.…”

Section: Av Blockmentioning

confidence: 99%

Diagnosis and Treatment of Fetal Cardiac Disease

Donofrio¹,

Moon-Grady²,

Hornberger³

et al. 2014

Circulation

976

543

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Background— The goal of this statement is to review available literature and to put forth a scientific statement on the current practice of fetal cardiac medicine, including the diagnosis and management of fetal cardiovascular disease. Methods and Results— A writing group appointed by the American Heart Association reviewed the available literature pertaining to topics relevant to fetal cardiac medicine, including the diagnosis of congenital heart disease and arrhythmias, assessment of cardiac function and the cardiovascular system, and available treatment options. The American College of Cardiology/American Heart Association classification of recommendations and level of evidence for practice guidelines were applied to the current practice of fetal cardiac medicine. Recommendations relating to the specifics of fetal diagnosis, including the timing of referral for study, indications for referral, and experience suggested for performance and interpretation of studies, are presented. The components of a fetal echocardiogram are described in detail, including descriptions of the assessment of cardiac anatomy, cardiac function, and rhythm. Complementary modalities for fetal cardiac assessment are reviewed, including the use of advanced ultrasound techniques, fetal magnetic resonance imaging, and fetal magnetocardiography and electrocardiography for rhythm assessment. Models for parental counseling and a discussion of parental stress and depression assessments are reviewed. Available fetal therapies, including medical management for arrhythmias or heart failure and closed or open intervention for diseases affecting the cardiovascular system such as twin–twin transfusion syndrome, lung masses, and vascular tumors, are highlighted. Catheter-based intervention strategies to prevent the progression of disease in utero are also discussed. Recommendations for delivery planning strategies for fetuses with congenital heart disease including models based on classification of disease severity and delivery room treatment will be highlighted. Outcome assessment is reviewed to show the benefit of prenatal diagnosis and management as they affect outcome for babies with congenital heart disease. Conclusions— Fetal cardiac medicine has evolved considerably over the past 2 decades, predominantly in response to advances in imaging technology and innovations in therapies. The diagnosis of cardiac disease in the fetus is mostly made with ultrasound; however, new technologies, including 3- and 4-dimensional echocardiography, magnetic resonance imaging, and fetal electrocardiography and magnetocardiography, are available. Medical and interventional treatments for select diseases and strategies for delivery room care enable stabilization of high-risk fetuses and contribute to improved outcomes. This statement highlights what is currently known and recommended on the basis of evidence and experience in the rapidly advancing and highly specialized field of fetal cardiac care.

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Outcome and Growth of Infants Fetally Exposed to Heart Block-Associated Maternal Anti-Ro52/SSA Autoantibodies

Cited by 34 publications

References 34 publications

Outcome in 212 anti‐Ro/SSA‐positive pregnancies and population‐based incidence of congenital heart block

Outcome in 212 anti‐Ro/SSA‐positive pregnancies and population‐based incidence of congenital heart block

Development of heart block in children of SSA/SSB-autoantibody-positive women is associated with maternal age and displays a season-of-birth pattern

Diagnosis and Treatment of Fetal Cardiac Disease

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