171 results on '"congenital heart block"'
Search Results
2. Home monitoring of fetal heart rhythm: Lived experiences of women with anti-SSA/Ro52 autoantibodies and their co-parents.
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Tingström, Joanna, Öst, Elin, Bergman, Gunnar, and Burström, Åsa
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FETAL heart , *AUTOANTIBODIES , *HEART block , *MEDICAL personnel , *CHILDBIRTH , *CHILDBIRTH at home - Abstract
Objective: The aim of this study was to explore the parents' experiences of home monitoring of the fetal heart rhythm. Women with anti-SSA/Ro52 autoantibodies carry a 2%–3% risk of giving birth to a child with congenital heart block (CHB), following transplacental transfer and antibody-mediated inflammation in the fetal conduction system during 18th to 24th gestational week. Early detection and subsequent treatment have been reported to decrease morbidity and mortality. Therefore, home monitoring of the fetal heart rhythm by Doppler has been offered at our fetal cardiology center. This study was undertaken to explore the lived experience of the routine. Methods: Participants were recruited from a single fetal cardiology center. Consecutive sampling was used. The inclusion criteria were women with SSA/Ro52 antibodies who had undergone Doppler examinations within the last two and a half years at the hospital and had monitored the fetal heartbeat at home. A semi-structured questionnaire was created, and the participants were interviewed individually. The interviews were transcribed verbatim and analyzed according to qualitative content analysis. Results: The overall theme was defined as "walking on thin ice," with six underlying categories: reality, different strategies, gain and loss, healthcare providers, underlying tension, and conducting the examinations again, all with a focus on how to handle the home monitoring during the risk period. Conclusion: Both the mother and the co-parent expressed confidence in their own abilities and that the monitoring provided them with the advantage of growing a bond with the expected child. However, all the participants described a feeling of underlying tension during the risk period. The results show that home monitoring is not experienced as complicated or a burden for the parents-to-be and should be considered a vital part of the chain of care for mothers at risk for giving birth to a child with CHB. However, explaining the teamwork between the different caregivers, for the patients involved, their areas of expertise, and how they collaborate with the patient continues to be a pedagogic challenge and should be developed further. [ABSTRACT FROM AUTHOR]
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- 2024
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3. Dual challenge inside the womb: a case report of concomitant fetal atrio-ventricular block associated with maternal anti-SSA antibodies and fetal tachyarrhythmia diagnosed as Wolff-Parkinson-White syndrome after birth
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Ana Teodósio Chícharo, Mónica Rebelo, Ana Rita Lopes, Maria João Saavedra, Maria Filipa Paramés, Ana Rita Araújo, Ana Rita Cruz-Machado, Luísa Pinto, and Susana Capela
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case report ,fetal atrio-ventricular block ,congenital heart block ,Wolff-Parkinson-White syndrome ,pregnancy ,anti-SSA/Ro antibodies ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.
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- 2024
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4. Health Outcomes of 215 Mothers of Children With Autoimmune Congenital Heart Block: Analysis of the French Neonatal Lupus Syndrome Registry.
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Miniaoui, Imene, Morel, Nathalie, Lévesque, Kateri, Maltret, Alice, Driessen, Marine, Masseau, Agathe, Orquevaux, Pauline, Piette, Jean-Charles, Barriere, Francois, Le Bidois, Jérome, Georgin-Lavialle, Sophie, Guettrot-Imbert, Gaëlle, Le Guern, Véronique, Mouthon, Luc, Jallouli, Moez, Deligny, Christophe, Hachulla, Eric, Romefort, Bénédicte, Bonnet, Damien, and Costedoat-Chalumeau, Nathalie
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SYSTEMIC lupus erythematosus diagnosis ,AUTOIMMUNE diseases ,ACQUISITION of data ,RETROSPECTIVE studies - Abstract
Objective: Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).Methods: This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.Results: We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (P < 0.001), dry syndrome (P = 0.01), and parotid swelling (P = 0.05).Conclusion: One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required. [ABSTRACT FROM AUTHOR]- Published
- 2022
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5. Management of fetal congenital heart block in pregnancies with anti-Ro antibodies
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Anca Maria Panaitescu, Gheorghe Peltecu, and Nicolae Gica
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fetus ,pregnancy ,congenital heart block ,anti-ro antibodies ,Medicine ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Anti-Ro antibodies are detected frequently in the general population, but more so in patients with autoimmune conditions as Sjögren’s syndrome and systemic lupus erythematous (SLE). During pregnancy, anti-Ro antibodies can cross the placenta by hijacking physiological mechanisms and can have deleterious effects on the fetus. Administration of hydroxychloroquine (HCQ) to pregnant women with documented anti-Ro antibodies has been shown to prevent congenital heart block (CHB ). Serial fetal ultrasound scans and echocardiograms are controversial in pregnant women with anti-Ro antibodies. When complete CHB is diagnosed, this is irreversible and can lead to fetal heart failure, hydrops, and death. After delivery, babies with complete CHB require pacemaker. In the presence of maternal anti-Ro antibodies, there is a high risk of recurrence of CHB for future pregnancies, if there is a previously affected child. Adequate counselling and prophylactic treatment with HCQ should be encouraged.
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- 2021
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6. Management of Sjögren’s Syndrome During Pregnancy
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Sandhya, Pulukool and Sharma, Shefali Khanna, editor
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- 2020
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7. Pregnancy counseling in rheumatic diseases: Where science meets the steps
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Anu Balakrishnan, Pankti Mehta, and Latika Gupta
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congenital heart block ,lupus ,myositis ,pregnancy ,rheumatology ,scleroderma ,vasculitis ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes.
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- 2021
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8. Management of fetal congenital heart block in pregnancies with anti-Ro antibodies.
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Panaitescu, Anca Maria, Peltecu, Gheorghe, and Gică, Nicolae
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HEART block , *FETAL heart , *SJOGREN'S syndrome , *PHYSIOLOGY , *IMMUNOGLOBULINS , *FETAL ultrasonic imaging - Abstract
Anti-Ro antibodies are detected frequently in the general population, but more so in patients with autoimmune conditions as Sjögren’s syndrome and systemic lupus erythematous (SLE). During pregnancy, anti-Ro antibodies can cross the placenta by hijacking physiological mechanisms and can have deleterious effects on the fetus. Administration of hydroxychloroquine (HCQ) to pregnant women with documented anti-Ro antibodies has been shown to prevent congenital heart block (CHB ). Serial fetal ultrasound scans and echocardiograms are controversial in pregnant women with anti-Ro antibodies. When complete CHB is diagnosed, this is irreversible and can lead to fetal heart failure, hydrops, and death. After delivery, babies with complete CHB require pacemaker. In the presence of maternal anti-Ro antibodies, there is a high risk of recurrence of CHB for future pregnancies, if there is a previously affected child. Adequate counselling and prophylactic treatment with HCQ should be encouraged. [ABSTRACT FROM AUTHOR]
- Published
- 2021
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9. Ethnicity and Neonatal Lupus Erythematosus Manifestations Risk in a Large Multiethnic Cohort.
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Diaz, Talia, Dominguez, Daniela, Jaeggi, Edgar, Knight, Andrea M., Laskin, Carl A., Ng, Lawrence, Silverio, Franklin, Silverman, Earl D., and Hiraki, Linda T.
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LUPUS erythematosus treatment ,LUPUS erythematosus complications ,NEONATAL diseases ,SICK children ,ETHNICITY - Abstract
Objective: To evaluate the association between ethnicity and neonatal lupus erythematosus (NLE), as well as specific NLE manifestations in a large multiethnic population.Methods: We conducted a cohort study of the children (≤ 1 yr of age) seen in the NLE clinic at The Hospital for Sick Children (SickKids), between January 2011 and April 2019. The cohort was divided into European, non-European, and mixed European-non-European groups according to parent-reported child's ethnicity (Canada Census categories). Outcomes were NLE and specific NLE manifestations (cardiac, cutaneous, cytopenias, transaminitis, and macrocephaly). The frequency of NLE and specific manifestations were compared between ethnic groups (Fisher exact test). We tested the association between ethnicity and (1) NLE risk, and (2) specific NLE manifestations with logistic regression models, including covariates for child's sex, maternal rheumatic disease status during pregnancy, and maternal use of antimalarials during pregnancy (multiple comparisons threshold P < 0.008).Results: We included 324 children born to 270 anti-Ro antibody-positive mothers. Median age at first visit was 1.8 (IQR 1.4-2.3) months, and median follow-up time was 12 (IQR 2-24) months. The majority was non-European (48%), with 34% European, and 18% mixed European-non-European. There was no significant association between non-European ethnicity (OR 1.18, 95% CI 0.71-1.94, P = 0.51), mixed European-non-European ethnicity (OR 1.13, 95% CI 0.59-2.16, P = 0.70), and NLE risk compared with European ethnicity. We also did not find an association between ethnicity and specific NLE manifestations in univariate or multivariable-adjusted models.Conclusion: In a large multiethnic cohort, there was no association between a child's ethnicity and NLE risk or specific NLE manifestations. [ABSTRACT FROM AUTHOR]- Published
- 2021
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10. Pregnancy counseling in rheumatic diseases: Where science meets the steps.
- Author
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Balakrishnan, Anu, Mehta, Pankti, and Gupta, Latika
- Abstract
Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes. [ABSTRACT FROM AUTHOR]
- Published
- 2021
- Full Text
- View/download PDF
11. Studies in the Area of Congenital Heart Block Reported from Brigham and Women's Hospital (Expert Perspective On a Clinical Challenge: Lupus and Pregnancy).
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HEART block ,WOMEN'S hospitals ,PREGNANCY ,PREGNANCY complications ,CONGENITAL heart disease ,PREGNANT women - Abstract
A recent report from Brigham and Women's Hospital in Boston, Massachusetts discusses the challenges of managing systemic lupus erythematosus (SLE) during pregnancy. The researchers emphasize the importance of achieving remission or low disease activity before conception to reduce the risk of complications such as pre-eclampsia and preterm birth. They recommend that expectant mothers with a history of antiphospholipid syndrome receive anticoagulant therapy, and that women with certain antibodies undergo additional monitoring due to the increased risk of congenital heart block in their offspring. The researchers also discuss the use of medications during pregnancy and the difficulty of distinguishing between a flare of lupus nephritis and pre-eclampsia. Overall, while outcomes for pregnant women with lupus are improving, there are still gaps in knowledge regarding optimal management strategies. [Extracted from the article]
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- 2024
12. A multicentre study of 244 pregnancies in undifferentiated connective tissue disease: maternal/fetal outcomes and disease evolution.
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Radin, Massimo, Schreiber, Karen, Cecchi, Irene, Bortoluzzi, Alessandra, Crisafulli, Francesca, Freitas, Cristiano M de, Bacco, Beatrice, Rubini, Elena, Foddai, Silvia G, Padovan, Melissa, Cassarino, Silvia Gallo, Franceschini, Franco, Andrade, Danieli, Benedetto, Chiara, Govoni, Marcello, Bertero, Tiziana, Marozio, Luca, Roccatello, Dario, Andreoli, Laura, and Sciascia, Savino
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AUTOANTIBODIES , *AUTOIMMUNE diseases , *CONGENITAL heart disease , *CONNECTIVE tissue diseases , *LONGITUDINAL method , *EVALUATION of medical care , *MEDICAL cooperation , *PREGNANCY , *PREGNANCY complications , *RESEARCH , *SYSTEMIC lupus erythematosus , *DESCRIPTIVE statistics - Abstract
Objectives To investigate fetal/perinatal and maternal outcomes from a large multicentre cohort of women diagnosed with UCTD. Methods This multicentre retrospective cohort study describes the outcomes of 224 pregnancies in 133 consecutive women with a diagnosis of UCTD, positive for ANA and aged <45 years old at study inclusion. Results Of the 224 pregnancies analysed, 177 (79%) resulted in live births, 45 (20.1%) in miscarriages (defined as pregnancy loss before 12 weeks' gestation), 2 (0.9%) in stillbirths (pregnancy loss after 20 weeks' gestation) and 6 (2.7%) cases showed intrauterine growth restriction. Miscarriages and stillbirths were strongly associated with the presence of aPL and ENA antibodies (P < 0.05). Maternal pregnancy complications were as follows: 5 (2.2%) cases developed pre-eclampsia, 11 (4.9%) cases gestational hypertension and 12 (5.4%) cases gestational diabetes. Joint involvement represented the most frequent clinical manifestation of the cohort (57.9%), followed by RP (40.6%), photosensitivity (32.3%) and haematological manifestations (27.1%). The rate of disease evolution of our cohort from a diagnosis of UCTD to a diagnosis of definite CTD was 12% within a mean time of 5.3 ± 2.8 years. With a total follow-up after first pregnancy of 1417 patient-years, we observed the evolution to a defined CTD in one out of every 88 patient- years. Conclusion In our multicentre cohort, women with UCTD had a live birth rate of 79%. Women with UCTD should be referred to specialist follow-up when planning a pregnancy. ENA profiling and aPL testing should be mandatory in this setting, and further therapeutic approaches and management should be planned accordingly. [ABSTRACT FROM AUTHOR]
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- 2020
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13. Pregnancy outcomes in mixed connective tissue disease: a multicentre study.
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Radin, Massimo, Schreiber, Karen, Cuadrado, Maria José, Cecchi, Irene, Andreoli, Laura, Franceschini, Franco, Caleiro, Teresa, Andrade, Danieli, Gibbone, Elena, Khamashta, Munther, Buyon, Jill, Izmirly, Peter, Aguirre, Maria Angeles, Benedetto, Chiara, Roccatello, Dario, Marozio, Luca, and Sciascia, Savino
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HYPERTENSION in pregnancy , *RISK factors of preeclampsia , *ECLAMPSIA , *PERINATAL death , *HEART block , *GESTATIONAL diabetes , *AUTOANTIBODIES , *CHILDBIRTH , *CONGENITAL heart disease , *CONNECTIVE tissue diseases , *ECHOCARDIOGRAPHY , *LONGITUDINAL method , *EVALUATION of medical care , *MEDICAL cooperation , *PREGNANCY , *FIRST trimester of pregnancy , *PREGNANT women , *PRENATAL diagnosis , *RESEARCH , *RISK assessment , *SYSTEMATIC reviews , *RETROSPECTIVE studies , *DISEASE complications , *DISEASE risk factors ,PREGNANCY complication risk factors ,RISK factors in miscarriages - Abstract
Objectives In this study we aimed to investigate foetal and maternal pregnancy outcomes from a large multicentre cohort of women diagnosed with MCTD and anti-U1RNP antibodies. Methods This multicentre retrospective cohort study describes the outcomes of 203 pregnancies in 94 consecutive women ever pregnant who fulfilled the established criteria for MCTD with confirmed U1RNP positivity. Results The foetal outcomes in 203 pregnancies were as follows: 146 (71.9%) live births, 38 (18.7%) miscarriages (first trimester pregnancy loss of <12 weeks gestation), 18 (8.9%) stillbirths (pregnancy loss after 20 weeks gestation) and 11 (5.4%) cases with intrauterine growth restriction. Maternal pregnancy outcomes were as follows: 8 (3.9%) developed pre-eclampsia, 2 (0.9%) developed eclampsia, 31 (15.3%) developed gestational hypertension and 3 (1.5%) developed gestational diabetes. Women with MCTD and aPL and pulmonary or muscular involvement had worse foetal outcomes compared with those without. Moreover, we report a case of complete congenital heart block (0.45%) and a case of cutaneous neonatal lupus, both born to a mother with positive isolated anti-U1RNP and negative anti-Ro/SSA antibodies. Conclusion In our multicentre cohort, women with MCTD had a live birth rate of 72%. While the true frequency of heart block associated with anti-U1RNP remains to be determined, this study might raise the consideration of echocardiographic surveillance in this setting. Pregnancy counselling should be considered in women with MCTD. [ABSTRACT FROM AUTHOR]
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- 2019
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14. First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry)
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Micaela Fredi, Laura Andreoli, Beatrice Bacco, Tiziana Bertero, Alessandra Bortoluzzi, Silvia Breda, Veronica Cappa, Fulvia Ceccarelli, Rolando Cimaz, Salvatore De Vita, Emma Di Poi, Elena Elefante, Franco Franceschini, Maria Gerosa, Marcello Govoni, Ariela Hoxha, Andrea Lojacono, Luca Marozio, Alessandro Mathieu, Pier Luigi Meroni, Antonina Minniti, Marta Mosca, Marina Muscarà, Melissa Padovan, Matteo Piga, Roberta Priori, Véronique Ramoni, Amelia Ruffatti, Chiara Tani, Marta Tonello, Laura Trespidi, Sonia Zatti, Stefano Calza, Angela Tincani, and Antonio Brucato
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pregnancy ,congenital heart block ,neonatal lupus ,outcome ,risk factors ,therapy ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Objective: Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge.Method: Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) in utero or neonatal period (up to 27 days after birth).Result: Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected in utero (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 in utero, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (p = 0.005, OR > 100; CI 95% 2.88->100 and hydrops (p = 0.003, OR = 14.09; CI 95% 2.01–122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins.Conclusion: This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.
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- 2019
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15. Benefits of fetal echocardiographic surveillance in pregnancies at risk of congenital heart block: single-center study of 212 anti-Ro52-positive pregnancies.
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Sonesson, S.‐E., Ambrosi, A., Wahren‐Herlenius, M., Sonesson, S-E, and Wahren-Herlenius, M
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HEART block , *FETAL monitoring , *PREGNANCY , *THERAPEUTICS , *MONEY , *REFERENCE values - Abstract
Objectives: Assuming that autoimmune congenital heart block (CHB) is a progressive disease amenable to therapeutic modulation, we introduced a surveillance program for at-risk pregnancies with the dual aim of investigating if fetal atrioventricular block (AVB) could be detected and treated before becoming complete and irreversible, and to establish the incidence of AVB I, II and III in a large prospective cohort.Methods: This was a prospective study of 212 anti-Ro52 antibody-exposed pregnancies at risk of fetal AVB that were followed weekly between 18 and 24 weeks' gestation at our tertiary fetal cardiology center from 2000 to 2015. A 12-lead electrocardiogram (ECG) was recorded within 1 week after birth. Fetal Doppler atrioventricular (AV) intervals were converted to Z-scores using reference standard values derived from normal pregnancies. Each fetus was represented by the average value of the two recordings, obtained at two consecutive visits, which resulted in the longest AV interval. AV interval values were classified into normal AV conduction (Z-score ≤ 2.0) and three levels of delayed AV conduction: Z-score > 2.0 and ≤ 3.0, Z-score > 3.0 and ≤ 4.0, and Z-score > 4.0.Results: AVB II or III developed in 6/204 (2.9%) pregnancies without a CHB history and 1/8 (12.5%) of those with a CHB history. AV intervals > 2 and ≤ 3, > 3 and ≤ 4, and > 4 were detected in 16.0%, 7.5% and 2.8% of cases, respectively, and were related to the PR interval on 185 available ECGs. Three of the five cases with AVB III and one of two cases with 2:1 AVB II developed within 1 week of AV interval Z-score of 1.0, 1.9, 2.8 and 1.9, respectively. Transplacental treatment with betamethasone was associated with restoration of 1:1 AV conduction in the two fetuses with AVB II, with a better long-term result (normal ECG vs AVB I or II) observed in the case in which treatment was started within 1 week after AVB developed. Betamethasone treatment did not reverse AVB III, although a temporary effect on AV conduction was observed in 1/5 cases. Notably, the three cases in which treatment was started within 1 week after AVB III development responded with a higher ventricular rate than the other two cases and did not require pacemaker implantation until a later age (2-5 years vs 1.5-2 months).Conclusion: Fetal AV interval is a poor predictor of CHB progression, but CHB surveillance still allows detection of fetuses with AVB II or III shortly after its development, allowing for timely treatment initiation and potentially better outcome. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]- Published
- 2019
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16. Síndrome de Sjögren primario y embarazo: reporte de 18 casos.
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Demarchi, Julia, Papasidero, Silvia B., Klajn, Diana, Alba, Paula, Babini, Alejandra M., Durigan, Virginia, Gobbi, Carla, and Raiti, Laura
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PREMATURE rupture of fetal membranes , *PREGNANCY complications , *DISEASES - Abstract
Resumen El síndrome de Sjögren primario (SSp) afecta preferentemente al sexo femenino. Los informes sobre la evolución de los embarazos en estas pacientes son pocos y contradictorios. Objetivo Describir las características del embarazo y su desenlace en mujeres con SSp, así como la morbilidad del recién nacido. Material y métodos Se incluyeron pacientes con SSp que quedaron embarazadas luego del comienzo de los síntomas de dicha enfermedad. Se describieron las características clínicas y serológicas, los factores de riesgo y las comorbilidades maternas previas. Se detalló la evolución de cada embarazo, el desenlace y las características del recién nacido. Resultados Se evaluaron 11 pacientes con 18 embarazos posteriores al inicio de síntomas: todas presentaban FAN+; 10 anti-Ro/SSA+ y 7 anti-La/SSB+. El promedio de edad en años al inicio de los síntomas fue de 24,9 (DE 6,9) y al momento del embarazo fue de 30,3 (DE 5,4). Hubo 13 embarazos previos al diagnóstico, reportándose solo un aborto espontáneo. Después de la presentación del SSp, se informaron: 2 partos prematuros, un oligoamnios, 2 roturas prematuras de membranas y 2 recién nacidos con bajo peso al nacer. Se halló un caso con bloqueo cardíaco congénito y otro con lupus cutáneo neonatal. Todas las mujeres con complicaciones obstétricas (6) presentaban Ac anti-Ro/SSA+, 5 con Ac anti-La/SSB+. Quienes no tuvieron complicaciones (5): 2 presentaban anti-Ro/SSA y anti-La/SSB+, y 2 solo anti-Ro/SSA. Conclusiones Casi la mitad de los embarazos de las pacientes con SSp analizados presentaron alguna complicación no atribuible a otro factor distinto de su enfermedad de base. Abstract Primary Sjögren's syndrome (pSS) is a condition that predominantly affects women. Reports of pregnancy outcome in these patients are limited and contradictory. Objective To describe pregnancy characteristics and outcomes and newborn morbidity in women with pSS. Material and methods We included women with pSS who became pregnant after the onset of the symptoms of the disease. Clinical and serological characteristics, risk factors and previous maternal comorbidities are described. For each pregnancy in a woman with pSS, we recorded pregnancy course and outcome and newborn condition. Results We assessed 11 patients with 18 pregnancies after the onset of pSS symptoms. All of them presented FAN +; 10 anti-Ro / SSA + and 7 anti-La / SSB +. The mean age in years at the onset of symptoms was 24.9 (SD 6.9) and at the time of pregnancy was 30.3 (SD 5.4). Thirteen pregnancies happened before the diagnosis, reporting only one miscarriage. Two preterm births, 1 case of oligohydramnios, 2 of premature membrane rupture and 2 low birthweight babies were reported after the onset of pSS symptoms. There was 1 newborn with congenital atrioventricular block and another with neonatal cutaneous lupus. All the women with pregnancy complications (n = 6) had anti-Ro/SSA antibodies. Conclusions Almost half of the pregnancies assessed in women with pSS were associated with complications not attributable to factors other than the disease. [ABSTRACT FROM AUTHOR]
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- 2019
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17. Anesthetic management of patient with Sjogren's syndrome who underwent cesarean section: a case report
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Na Eun Kim, Jae Hyuk Lee, In Sun Chung, and Jun Yong Lee
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anesthesia ,congenital heart block ,pregnancy ,sjogren's syndrome ,Anesthesiology ,RD78.3-87.3 - Abstract
Sjogren's syndrome is one of the most common autoimmune disorders and has a female predominance. Maternal circulating autoantibodies such as anti-Ro/SSA and anti-La/SSB antibodies can cause congenital heart block of fetus, and in severe case, emergency pacemaker implantation may be needed for neonate. Therefore, it is very important to understand maternal and fetal condition and pay attention to the status of the neonate during delivery. In this paper, we present a case of patient with Sjogren's syndrome who underwent cesarean section under spinal anesthesia.
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- 2016
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18. Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period
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Virginie Meau-Petit, William Regan, Eric Rosenthal, Hitarth Bhatt, and Hayley Hernstadt
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Pediatrics ,medicine.medical_specialty ,Neonatal intensive care unit ,Gestational Age ,Infant, Premature, Diseases ,Congenital heart block ,Antenatal steroid ,Cohort Studies ,Neonate ,Pregnancy ,Complete heart block ,Risk of mortality ,Humans ,Medicine ,business.industry ,Infant, Newborn ,Infant ,medicine.disease ,Heart Block ,Intraventricular hemorrhage ,Pediatrics, Perinatology and Child Health ,Necrotizing enterocolitis ,Cohort ,Gestation ,Original Article ,Female ,Prematurity ,business ,Infant, Premature ,Cohort study - Abstract
Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (
- Published
- 2021
19. Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus.
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Morel, Nathalie, Lévesque, Kateri, Maltret, Alice, Baron, Gabriel, Hamidou, Mohamed, Orquevaux, Pauline, Piette, Jean-Charles, Barriere, François, Le Bidois, Jérôme, Fermont, Laurent, Fain, Olivier, Theulin, Arnaud, Sassolas, François, Hauet, Quentin, Guettrot-Imbert, Gaëlle, Georgin-Lavialle, Sophie, Deligny, Christophe, Hachulla, Eric, Mouthon, Luc, and Le Jeunne, Claire
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DILATED cardiomyopathy , *LUPUS erythematosus , *NEONATAL diseases , *STEROID drugs , *HEART block , *THERAPEUTICS , *DISEASE risk factors ,CARDIOVASCULAR disease related mortality - Abstract
Background Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. Methods We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. Results Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7 years [range: birth–36 years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM ( P = 0.0199; HR = 3.13 [95% CI: 1.20–8.16]), non-European origin ( P = 0.0052; HR = 4.10 [95% CI: 1.81–9.28]) and pacemaker implantation ( P = 0.0013; HR = 5.48 [95% CI: 1.94–15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n = 13, diagnosed at a median age of 0 day [birth–4 days]) and late-onset DCM (n = 22, diagnosed at a median age of 15.2 months [3.6 months–22.8 years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM ( P = 0.27; HR = 1.65 [95% CI: 0.63–4.25]). Probability of survival at 10 years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. Conclusion Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB. [ABSTRACT FROM AUTHOR]
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- 2017
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20. Pregnancy control in patients with systemic lupus erythematosus/antiphospholipid syndrome. Part 2: Pregnancy follow-up
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Paloma Vela-Casasempere, Gerard Espinosa, Nuria Martínez Sánchez, Jose L. Bartha, Beatriz Marco, Luis Sáez-Comet, Paloma Delgado, Juan Antonio López, Amaia Ugarte, Manel Casellas, Guillermo Ruiz-Irastorza, María Galindo-Izquierdo, Víctor M. Martínez-Taboada, Esther Rodríguez Almaraz, Angel Robles, and Universidad de Cantabria
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Gynecology ,medicine.medical_specialty ,Embarazo ,Lactancia ,Síndrome Antifosfolípido ,business.industry ,Lupus Eritematoso Sistémico ,Puerperium ,Antiphospholipid Syndrome ,medicine.disease ,Systemic Lupus Erythematosus ,Congenital heart block ,Anticoncepción ,Contraception ,Rheumatology ,Pregnancy ,Antiphospholipid syndrome ,Lactation ,Medicine ,In patient ,business ,Puerperio - Abstract
Objective: In order to agree on the fundamental aspects related to the management of pregnancy in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS), the Spanish Societies of Gynaecology and Obstetrics, Internal Medicine and Rheumatology set up a working group for the preparation of three consensus documents. Methods: Each of the Scientific Societies involved proposed five representatives based on their experience in the field of pregnancy control in patients with autoimmune diseases. The recommendations were developed following the Delphi methodology. Results: This second document contains the recommendations regarding the management of pregnancy in women with SLE and APS, including complications such as lupus activity, congenital heart block, thrombotic and obstetric manifestations of APS and placental vascular disease. Conclusions: These multidisciplinary recommendations are considered decision-making tools for clinicians involved in the care of patients with SLE/APS during pregnancy. Objetivo: Las sociedades españolas de ginecología y obstetricia, de medicina interna y de reumatología han constituido un grupo de trabajo paritario para la elaboración de 3 documentos de consenso sobre el control del embarazo en mujeres con lupus eritematoso sistémico (LES) y síndrome antifosfolípido (SAF).Métodos: Cada una de las sociedades científicas implicadas propuso 5 representantes en base a su experiencia en el área del control del embarazo en pacientes con enfermedades autoinmunes. Las recomendaciones se elaboraron siguiendo la metodología Delphi. Resultados: En este segundo documento se incluyen las recomendaciones que abordan el manejo del embarazo normal y sus complicaciones en mujeres con LES/SAF. Se presentan las recomendaciones relacionadas con el seguimiento del embarazo, la actividad lúpica, el bloqueo cardíaco congénito, las manifestaciones trombóticas y obstétricas del SAF y los defectos de placentación. Conclusiones: Estas recomendaciones multidisciplinares se consideran herramientas en la toma de decisiones para los clínicos involucrados en la asistencia a pacientes con LES/SAF durante el embarazo.
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- 2021
21. Long-term physical and neurodevelopmental outcomes after antenatal betamethasone administration for congenital heart block prevention
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Atsuko Murashima, Yuka Sano Wada, Mikako Goto, Yushi Ito, and Ikuko Hama
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Pediatrics ,medicine.medical_specialty ,Betamethasone ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Pregnancy ,parasitic diseases ,Humans ,Medicine ,Anti-SSA/Ro Antibody ,Child ,Glucocorticoids ,030203 arthritis & rheumatology ,business.industry ,Mortality rate ,Body Weight ,food and beverages ,Obstetrics and Gynecology ,Heart Block ,Pediatrics, Perinatology and Child Health ,Female ,business ,030217 neurology & neurosurgery ,medicine.drug - Abstract
Congenital heart block (CHB) is associated with a mortality rate of 20% and requires a pacemaker in 70% of cases. Steroids can reduce morbidity and prevent the onset of CHB but may have adverse effects on growth and neurodevelopment. This study aimed to clarify the long-term effects of antenatal betamethasone administration on growth and neurodevelopment.The subjects were children with a high risk of CHB due to a high level of maternal anti-SSA/Ro antibody or a maternal history of a previous delivery of a offspring with CHB to whom antenatal betamethasone was administered. Data on body weight, height, and blood pressure were collected as physical outcomes. The Wechsler Intelligence Scale for Children (fourth edition) or the Kyoto Scale of Psychological Development and the Pervasive Developmental Disorders Autism Society Japan Rating Scale was administered to assess the neurodevelopmental outcome.Fourteen children were enrolled. The body weight and height were within normal range in all children. All children had normal intelligence, and none had autism.Our study suggested that antenatal betamethasone administration has no negative effects on long-term physical and neurodevelopmental outcomes.
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- 2020
22. Pulmonary hypertension associated with congenital heart block and neonatal lupus syndrome: A series of four cases
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Nathalie Morel, Sophie Malekzadeh-Milani, Patrick Barbet, Damien Bonnet, Martina Evangelista, Marilyne Levy, Nathalie Costedoat-Chalumeau, and Alice Maltret
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Male ,Cardiac Catheterization ,Pediatrics ,medicine.medical_specialty ,Hypertension, Pulmonary ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,medicine ,Humans ,Lupus Erythematosus, Systemic ,030203 arthritis & rheumatology ,Pregnancy ,Systemic lupus erythematosus ,business.industry ,Hemodynamics ,Infant, Newborn ,medicine.disease ,Pulmonary hypertension ,Neonatal lupus syndrome ,Heart Block ,Echocardiography ,Female ,Presentation (obstetrics) ,Tomography, X-Ray Computed ,business ,Immunosuppressive Agents - Abstract
Objective Neonatal lupus syndrome has multisystemic manifestations among which pulmonary involvement has been rarely reported. We describe the clinical presentation, management, and outcome of a series of four neonates who developed reversible pulmonary hypertension associated with auto-immune congenital complete heart block. Method Data from the French registry of neonatal lupus syndrome were retrospectively reviewed. Results Between 2000 and March 2020, 231 children were included in the French registry, four/73 followed in our institution developed pulmonary hypertension. Diagnosis was suspected on transthoracic echocardiography at a median age of 42 days [range 10-58], and confirmed by right heart catheterization in all; 2 of them where paced at time of diagnosis and 2 were not. All had some degree of hypoxemia and respiratory distress. Hypoxemia was always reversible under O2 et NO. Lung CT demonstrated ground glass anomalies in all. One patient had a lung biopsy consistent with pulmonary hypertension secondary to lung disease. Management included immunosuppressive therapy in 3 associated with sildenafil in 2. Pulmonary hypertension resolved in all at a median age of 4 weeks [range 3-6] after treatment initiation and after one year for the one child who did not receive specific treatment. Conclusion Clinical, hemodynamical, imaging and histological findings advocate for pulmonary hypertension associated with respiratory disease as a rare manifestation of neonatal lupus syndrome.
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- 2020
23. Prenatal exposure to antimalarials decreases the risk of cardiac but not non-cardiac neonatal lupus: a single-centre cohort study.
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Barsalou, Julie, Jaeggi, Edgar, Laskin, Carl A., Brown, Patrick, Tian, Simon Y., Hamilton, Robert M., and Silverman, Earl D.
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SYSTEMIC lupus erythematosus , *ANTIMALARIALS , *HEART block , *LONGITUDINAL method , *TREATMENT effectiveness , *RETROSPECTIVE studies , *DATA analysis software , *DESCRIPTIVE statistics , *ODDS ratio , *MATERNAL exposure , *CHILDREN , *PREGNANCY , *THERAPEUTICS , *DISEASE risk factors - Abstract
Objective. Recent studies have suggested that prenatal exposure to HCQ reduces the risk of cardiac neonatal lupus. The aim of this study is to assess if maternal intake of antimalarials (AMs) throughout pregnancy lowered the risk of cardiac and non-cardiac neonatal lupus. Methods. Consecutive children seen between 1 January 1984 to 1 October 2013 born to women with a CTD and positive anti-Ro and/or anti-La antibodies were eligible for this single-centre retrospective cohort study. A total of 315 individuals were screened and 268 participants were included. Exposure to AMs was defined as HCQ or chloroquine throughout pregnancy. Outcomes were cardiac and non-cardiac neonatal lupus. Frequentist and Bayesian analyses were performed. We hypothesized that prenatal AM exposure would decrease the risk of cardiac but not non-cardiac neonatal lupus. Results. A total of 268 pregnancies were included; 73 were exposed to AMs throughout pregnancy. Ninety-nine children developed neonatal lupus, 117 remained unaffected and 52 children did not develop cardiac neonatal lupus but could not be categorized as unaffected since their full non-cardiac neonatal lupus status was unknown. Logistic regression suggested a protective effect of AM on cardiac neonatal lupus, but results were not statistically significant [odds ratio (OR) 0.21; P = 0.07]. Bayesian analysis showed that the probability of obtaining a protective effect (OR<1.0) for cardiac neonatal lupus was significant (98.7%). The effect of AMs on non-cardiac neonatal lupus was not significant (OR 0.78; P = 0.21). Conclusion. In this large single-centre cohort study, exposure to AMs throughout pregnancy was asso- ciated with a decreased probability of developing cardiac but not non-cardiac neonatal lupus. [ABSTRACT FROM AUTHOR]
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- 2017
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24. Obstetric and perinatal outcome in anti-Ro/SSA-positive pregnant women: a prospective cohort study.
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Martínez-Sánchez, Nuria, Pérez-Pinto, Sergio, Robles-Marhuenda, Ángel, Arnalich-Fernández, Francisco, Martín Cameán, María, Hueso Zalvide, Edurne, and Bartha, Jose
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Anti-Ro/SS-A is one specific type of antinuclear antibodies. They are in the majority of cases associated with primary Sjögren syndrome (SS) but also in Systemic Lupus Erythematosus (SLE), rheumatoid arthritis (RA), and in healthy people. During pregnancy, they are mainly associated to congenital heart block (CHB) and neonatal lupus (NL). The aim of this study was to compare the rate of maternal and fetal complications between a series of anti-Ro/SS-A positive pregnant women prospectively followed. Forty-two anti-Ro/SSA antibodies positive pregnant women that were referred to our hospital between 2011 and 2015. Data about pregnancy follow-up and outcomes were prospectively recorded from electronic databases. Data included demographic characteristics of the patients and their diseases (type, treatments, profile of anti-Ro/SSA, and antiphospholipid antibodies), pregnancy complications (CHB, preeclampsia, preterm delivery), ultrasound examinations and conditions, and mode of delivery. Maternal age was 35.22 ± 3.42 years and most of them were either SLE ( n = 16, 40%) or Sjögren syndrome ( n = 15, 37.5%). The rest of them were asymptomatic carriers ( n = 8; 20%), and there was only one case of rheumatoid arthritis ( n = 1; 2.5%). The incidence of anti-Ro52 and anti-Ro60 positive was n = 13, 82.4% and n = 16, 100%, respectively. Anti-La/SSB antibodies were present in n = 17, 48,6% of the patients. Half of the patients were taking hydroxycloroquine ( n = 18, 45%). Seven pregnancies were complicated by fetal anti-Ro-related cardiac disease (17.9%) including four cases (57.1%) of second-degree heart block, two cases of third degree heart block (28.6%) and one case (14.3%) of intense and diffuse hyperechogenicity in atrioventricular valves without heart block. Gestational age at diagnosis of these conditions was 23.2 ± 3.5 weeks. One of the 18 patients having hydroxychloroquine (5.6%) compared with the six of them in women not having this medication (6/22, 27.3%) ( p = 0.10). Concerning about Doppler evaluation, the Z score of umbilical pulsatility index (PI) was significantly higher in the SLE patients ( p = 0.02). There were no cases of preeclampsia. Labor was induced in 21 cases (52.5%) and cesarean section rate was 45%. Gestational age at birth was 39 (37-40) weeks, and the general prematurity rate was 20% ( n = 8). Birthweight was 2985 g (2425-3185 g) and 2850 (12.25-52.50) centiles for gestational age. The rate of small for gestational age (SGA) infants was 31.3% for SLE patients (5/16), 13.3% for Sjögren syndrome (2/15), and 12.5% for asymptomatic women (1/8). The rate of neonatal acidosis (pH < 7.20) was 20% (8/34) and it was higher in the SLE cases (6/15, 40%) when delivered after 38 weeks. The main pregnancy complication associated to anti-Ro/SS-A antibodies is CHB. The prevalence of CHB in patients taking hydroxychloriquine is lower without distinguishing between high or low risk patients. Preterm delivery occurs in anti-Ro/SS-A patients at the same rate as in the general population if no complications such as CHB or intrauterine growth restriction (IUGR) occur. The SGA rate also is higher probably because of SLE not because anti-Ro/SS-A antibodies. Finally, the finding of high umbilical artery PI will allow to predict fetus at risk of adverse pregnancy outcomes. Highlights: •Anti-Ro/SS-A and anti-La/SS-B are clinically very relevant during pregnancy mainly because of their association to congenital heart block and neonatal lupus. •In our cohort, the prevalence of congenital heart block detected in patients taking hydroxycloroquine is much lower than in patients not taking it without distinguishing between high and low risk patients. •High umbilical artery pulsatility index in Doppler scans studies has been detected in our anti-Ro/SSA population (basely in SLE patients) demonstrated this measurement as a predictor of SGA and adverse pregnancy outcomes in general population such as cesarean section for fetal distress. The small for gestational age rate is higher probably because of SLE not because anti-Ro/SS-A •Preterm delivery happens in anti-Ro/SS-A patients at the same rate as in the general population if no complications such as congenital heart block or intrauterine growth restriction occur. [ABSTRACT FROM AUTHOR]
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- 2017
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25. Hydroxychloroquine to Prevent Recurrent Congenital Heart Block in Fetuses of Anti-SSA/Ro-Positive Mothers
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Robert R. Clancy, Jill P. Buyon, Noël Zahr, Mimi Y. Kim, Rebecca E. Cohen, Joshua A. Copel, Mala Masson, Colin K.L. Phoon, Peter M. Izmirly, Kimberly Robins, Amit Saxena, Bettina F. Cuneo, Benjamin J. Wainwright, Nathalie Costedoat-Chalumeau, and Deborah M. Friedman
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Adult ,Male ,medicine.medical_specialty ,hydroxychloroquine ,anti-SSA/Ro antibodies ,Administration, Oral ,030204 cardiovascular system & hematology ,Article ,03 medical and health sciences ,0302 clinical medicine ,prevention ,Pregnancy ,Internal medicine ,Secondary Prevention ,medicine ,Humans ,Prospective Studies ,030212 general & internal medicine ,Enzyme Inhibitors ,Autoantibodies ,Systemic lupus erythematosus ,Dose-Response Relationship, Drug ,congenital heart block ,business.industry ,Infant, Newborn ,Hydroxychloroquine ,Endocardial fibroelastosis ,neonatal lupus ,medicine.disease ,Rash ,Clinical trial ,Fetal Diseases ,Heart Block ,magnetocardiography ,Gestation ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies ,medicine.drug ,Anti-SSA/Ro autoantibodies - Abstract
Background Experimental and clinical evidence support the role of macrophage Toll-like receptor signaling in maternal anti-SSA/Ro–mediated congenital heart block (CHB). Objectives Hydroxychloroquine (HCQ), an orally administered Toll-like receptor antagonist widely used in lupus including during pregnancy, was evaluated for efficacy in reducing the historical 18% recurrence rate of CHB. Methods This multicenter, open-label, single-arm, 2-stage clinical trial was designed using Simon’s optimal approach. Anti-SSA/Ro–positive mothers with a previous pregnancy complicated by CHB were recruited (n = 19 Stage 1; n = 35 Stage 2). Patients received 400 mg daily of HCQ prior to completion of gestational week 10, which was maintained through pregnancy. The primary outcome was 2° or 3° CHB any time during pregnancy, and secondary outcomes included isolated endocardial fibroelastosis, 1° CHB at birth and skin rash. Results By intention-to-treat (ITT) analysis, 4 of 54 evaluable pregnancies resulted in a primary outcome (7.4%; 90% confidence interval: 3.4% to 15.9%). Because 9 mothers took potentially confounding medications (fluorinated glucocorticoids and/or intravenous immunoglobulin) after enrollment but prior to a primary outcome, to evaluate HCQ alone, 9 additional mothers were recruited and followed the identical protocol. In the per-protocol analysis restricted to pregnancies exposed to HCQ alone, 4 of 54 (7.4%) fetuses developed a primary outcome as in the ITT. Secondary outcomes included mild endocardial fibroelastosis (n = 1) and cutaneous neonatal lupus (n = 4). Conclusions These prospective data support that HCQ significantly reduces the recurrence of CHB below the historical rate by >50%, suggesting that this drug should be prescribed for secondary prevention of fetal cardiac disease in anti-SSA/Ro-exposed pregnancies. (Preventive Approach to Congenital Heart Block With Hydroxychloroquine [PATCH]; NCT01379573 )
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- 2020
26. Autoimmune-mediated congenital heart block
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Catherine Trad, Rebecca E. Cohen, Peter M. Izmirly, Rohit Bhan, Amit Saxena, Jill P. Buyon, and Benjamin J. Wainwright
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Adult ,medicine.medical_specialty ,Inflammation ,Disease ,030204 cardiovascular system & hematology ,Autoantigens ,Congenital heart block ,Autoimmune Diseases ,Pathogenesis ,03 medical and health sciences ,Fetal Heart ,0302 clinical medicine ,Pregnancy ,Fibrosis ,Internal medicine ,medicine ,Humans ,Autoantibodies ,030203 arthritis & rheumatology ,Lupus Vulgaris ,business.industry ,Autoantibody ,Obstetrics and Gynecology ,Dilated cardiomyopathy ,Endocardial fibroelastosis ,General Medicine ,medicine.disease ,Pregnancy Complications ,Heart Block ,Cardiology ,Female ,medicine.symptom ,business - Abstract
Autoimmune-mediated congenital heart block (CHB) is a severe manifestation of neonatal lupus in which conduction tissues of the fetal heart are damaged. This occurs due to passive transference of maternal anti-SSA/Ro and anti-SSB/La autoantibodies and subsequent inflammation and fibrosis of the atrioventricular (AV) node. Notably, the disease manifests after the fetal heart has structurally developed, ruling out other anatomical abnormalities that could otherwise contribute to the block of conduction. Complete AV block is irreversible and the most common manifestation of CHB, although other cardiac complications such as endocardial fibroelastosis (EFE), dilated cardiomyopathy, and valvular insufficiency have been observed. In this review, we detail the classification, prevalence, pathogenesis, and clinical management recommendations for autoimmune CHB.
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- 2020
27. Fetal Congenital Heart Block Associated With Maternal Primary Systemic Lupus Erythematosus and Sjogren’s Syndrome
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Syed R Ali, Priyanka Anvekar, Petras Lohana, Sameera Khan, and Mohammed Sheeraz Alam
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medicine.medical_specialty ,Pregnancy ,Fetus ,sjogren's disease ,congenital heart block ,Obstetrics ,Heart block ,business.industry ,medicine.medical_treatment ,General Engineering ,Fetal Bradycardia ,medicine.disease ,Pediatrics ,Cardiac pacemaker ,Congenital heart block ,Polycystic ovarian disease ,internal medicine and rheumatology ,Rheumatology ,medicine ,Internal Medicine ,Gestation ,systemic lupus erythromatosus ,business ,skin and connective tissue diseases ,cardiac pacemaker - Abstract
Congenital heart block is a grave condition reported in 0.5% of 100 live births. Systemic lupus erythematosus (SLE) and Sjogren's syndrome (SS) are chronic autoimmune and inflammatory condition, which affects multiple systems. The association of SLE and SS with pregnancy has been seen in the past. Usually, it shows anti-Ro/SSA and anti-Ro/SSB auto-antibodies in maternal serum, which is proportional to fetal Outcome. In this report, we present a case of a 29-year-old female gravida 4, para one and aborta 3, with a history of polycystic ovarian disease and multiple abortions. At 20 weeks of gestation, her antenatal examination revealed fetal bradycardia and heart block, which further led to SLE and SS diagnosis in her. She was treated with steroids to prevent further fetal complications. The patient delivered a healthy neonate at 38 weeks of gestation. The neonate eventually received a cardiac pacemaker and is now on regular follow-up.
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- 2021
28. Maternal predictive factors for fetal congenital heart block in pregnant mothers positive for anti-SS-A antibodies.
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Hiroto Tsuboi, Takayuki Sumida, Hisashi Noma, Kazumasa Yamagishi, Ai Anami, Kotaro Fukushima, Hitoshi Horigome, Yasuki Maeno, Mitsumasa Kishimoto, Yoshinari Takasaki, Masahiro Nakayama, Masako Waguri, Haruhiko Sago, and Atsuko Murashima
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CONGENITAL heart disease , *PREGNANCY , *PREDNISOLONE , *CORTICOSTEROIDS , *IMMUNOGLOBULINS - Abstract
Objective: To determine the maternal predictive factors for fetal congenital heart block (CHB) in pregnancy in mothers positive for anti-SS-A antibodies. Methods: The Research Team for Surveillance of Autoantibody-Exposed Fetuses and Treatment of Neonatal Lupus Erythematosus, the Research Program of the Japan Ministry of Health, Labor and Welfare, performed a national survey on pregnancy of mothers positive for anti-SS-A antibodies. We analyzed 635 pregnant mothers who tested positive for anti-SS-A antibodies before conception but had no previous history of fetal CHB. We performed univariate and multivariate analysis (models 1, 2, and 3 using different set of independent variables) investigated the relation between risk of fetal CHB and maternal clinical features. Results: Of the 635 pregnant mothers, fetal CHB was detected in 16. Univariate analysis showed that fetal CHB associated with use of corticosteroids before conception (OR 3.72, p=0.04), and negatively with use of corticosteroids (equivalent doses of prednisolone (PSL), at -10 mg/day) after conception before 16-week gestation (OR 0.17, p=0.03). In multivariate analysis, model 1 identified the use of corticosteroids before conception (OR 4.28, p=0.04) and high titer of anti-SS-A antibodies (OR 3.58, p=0.02) as independent and significant risk factors, and model 3 identified use of corticosteroids (equivalent doses of PSL, at -10 mg/day) after conception before 16-week gestation as independent protective factor against the development of fetal CHB (OR 0.16, p=0.03). Other maternal clinical features did not influence the development of fetal CHB. Conclusion: The results identified high titers of anti-SS-A antibodies and use of corticosteroids before conception as independent risk factors, and use of corticosteroids (equivalent doses of PSL, at -10 mg/day) after conception before 16-week gestation as an independent protective factor for fetal CHB. [ABSTRACT FROM AUTHOR]
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- 2016
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29. Anti-Ro/SSA autoantibody-positive women’s experience of information given on the risk of congenital heart block.
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Tingström, J., Hjelmstedt, A., Welin Henriksson, E., Ambrosi, A., Sonesson, S-E, and Wahren-Herlenius, M.
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HEART block , *AUTOANTIBODIES , *WOMEN'S health , *CONGENITAL heart disease , *HEART disease related mortality , *DISEASE risk factors - Abstract
Objective Congenital heart block (CHB) may develop in fetuses of women with anti-Ro/SSA autoantibodies, and carries substantial morbidity and mortality. The aim was to evaluate how information on CHB is imparted and identify areas of improvement. Methods A questionnaire was distributed to anti-Ro/SSA antibody-positive women who had either participated in a surveillance programme but whose expected child did not develop CHB (n = 100, denoted Doppler-Assessed Pregnancies (DAP) group) or given birth to a child with CHB (n = 88, denoted CHB-Affected Pregnancies (CAP) group). Results The response rate was 83% (157/188). Most women received the information on CHB when they were already pregnant (DAP group 60%, CAP group 83%). However, a majority of them would have wanted the information before pregnancy (DAP group 52%, CAP group 56%), and most stated that it would not have influenced their decision to have a child (DAP group 77%, CAP group 58%). The ability to both understand the information and to perceive the information as sufficient were significantly higher when someone trained in paediatric cardiology gave the information. Conclusions Our findings indicate that information on CHB should be given to women before pregnancy. The data further highlight the importance of having specific knowledge for giving relevant and understandable, yet sufficient information. [ABSTRACT FROM AUTHOR]
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- 2016
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30. Outcome in 212 anti-Ro/SSA-positive pregnancies and population-based incidence of congenital heart block.
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Skog, Amanda, Lagnefeldt, Linda, Conner, Peter, Wahren‐Herlenius, Marie, and Sonesson, Sven‐Erik
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CONGENITAL heart disease , *FETAL abnormalities , *DISEASE incidence , *AUTOIMMUNE diseases , *PREGNANCY , *AUTOANTIBODIES , *SJOGREN'S syndrome , *SYSTEMIC lupus erythematosus , *CESAREAN section , *FETAL growth retardation , *HEART block , *PREMATURE infants , *LONGITUDINAL method , *DISEASE prevalence - Abstract
Introduction: We investigated the effects of maternal autoimmune disease and fetal congenital heart block (CHB) on pregnancy outcomes in anti-Ro/SSA-positive women and assessed the population-based incidence of isolated CHB.Material and Methods: One hundred and ninety nine anti-Ro/SSA-positive pregnancies were prospectively followed at our center (2000-2013). Seven fetuses developed atrioventricular block (AVB) II-III. In this period, another 13 anti-Ro/SSA-positive pregnancies were referred for fetal bradycardia, subsequently diagnosed with AVB II-III. Cesarean section rates, gestational age, body measurements at birth, and the incidence of CHB in these 212 pregnancies were analyzed in relation to fetal atrioventricular conduction and maternal diagnosis and compared with data from the Medical Birth Registry on 352,104 pregnancies in the Stockholm County.Results: The prevalence of maternal systemic lupus erythematosus (SLE) and primary Sjögren's syndrome and the outcomes at birth were similar in normal conduction and AVB I cases. Only 1/20 AVB II-III cases (0/7 in the surveillance group) had a mother diagnosed with SLE, compared with 73/192 in cases with normal conduction or AVB I. Excluding cases with AVB II-III, SLE mothers more frequently delivered by cesarean section (31% vs. 20%, p < 0.05) and had a higher incidence of preterm birth (13% vs. 5.8%, p < 0.05) than the county population. Both SLE and primary Sjögren's syndrome mothers had a fourfold greater rate of growth-retarded babies (10.11% vs. 2.2%, p < 0.001). The incidence of autoantibody-related AVB II-III in Stockholm County was 1/23 300.Conclusion: This study of CHB provides new information on the incidence of CHB and outcome of pregnancy in anti-Ro/SSA-positive women, which has clinical relevance when counseling rheumatic patients considering pregnancy. [ABSTRACT FROM AUTHOR]- Published
- 2016
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31. Description of 214 cases of autoimmune congenital heart block: Results of the French neonatal lupus syndrome.
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Levesque, Kateri, Morel, Nathalie, Maltret, Alice, Baron, Gabriel, Masseau, Agathe, Orquevaux, Pauline, Piette, Jean-Charles, Barriere, Francois, Le Bidois, Jérome, Fermont, Laurent, Fain, Olivier, Theulin, Arnaud, Sassolas, Francois, Pezard, Philippe, Amoura, Zahir, Guettrot-Imbert, Gaëlle, Le Mercier, Delphine, Georgin-Lavialle, Sophie, Deligny, Christophe, and Hachulla, Eric
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AUTOIMMUNE diseases , *HEART block , *NEONATAL diseases , *DILATED cardiomyopathy , *CONFIDENCE intervals , *STEROIDS - Abstract
Background Cardiac neonatal lupus syndrome is due to anti-SSA or SSB antibodies and mainly includes congenital heart block (CHB) and dilated cardiomyopathy (DCM). Its optimal management is still debated. We report a large series of autoimmune high degree CHB. Methods Inclusion criteria in this retrospective study were fetuses or neonates with high-degree CHB associated with maternal anti-SSA/SSB antibodies. Results 214 CHB were included: 202 detected in utero at a median term of 23 weeks' gestation (WG) [range 16 to 39 WG] and 12 neonatal cases diagnosed at a median age of 0 days [range birth to 8 days]. The 214 cases of CHB included 202 (94.4%) third-degree CHB, 8 (3.7%) second-degree CHB, and 4 (1.9%) intermittent CHB. In multivariate analysis, the factors associated with feto-neonatal deaths (15.7%) were hydrops (p < 0.001; hazard ratio [HR] 12.4 [95% confidence interval (95%CI) 4.7–32.7]) and prematurity (p = 0.002; HR 17.1 [95%CI 2.8–103.1]). During a median follow-up of 7 years [birth to 36 years], 148 of 187 children born alive (79.1%) had a pacemaker, 35 (18.8%, one missing data) had DCM, and 22 (11.8%) died. In multivariate analysis, factors associated with child death were in utero DCM (p = 0.0157; HR 6.37 [95%CI: 1.25–32.44]), postnatal DCM (p < 0.0001; HR 227.58[95%CI: 24.33–2128.46]) and pacemaker implantation (p = 0.0035; HR 0.11[95%CI: 0.02–0.51]). The use of fluorinated steroids was neither associated with survival nor with regression of 2nd degree CHB. Conclusion In this second largest series of CHB, we confirm some of the previous results. We were unable to find data supporting the routine use of in utero fluorinated steroids. [ABSTRACT FROM AUTHOR]
- Published
- 2015
- Full Text
- View/download PDF
32. Ro/SSA autoantibody-positive pregnancy: reactions to serial fetal Doppler echocardiographic surveillance.
- Author
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Tingström, J., Hjelmstedt, A., Welin Henriksson, E., Sonesson, S-E, and Wahren-Herlenius, M.
- Subjects
- *
HEART block , *SJOGREN'S syndrome , *AUTOANTIBODIES , *PREGNANCY complications , *DOPPLER echocardiography - Abstract
Objective The risk for congenital heart block (CHB) associated with maternal Ro/SSA autoantibodies is low, but the possibility of treating early stages of disease has seen the introduction of Doppler echocardiographic surveillance programs with serial examinations during the CHB susceptibility weeks of pregnancy. The aim of the present study was to understand how Ro/SSA autoantibody-positive women having undergone Doppler echocardiographic surveillance programs and giving birth to children without CHB experienced their pregnancy and frequent ultrasound examinations. Methods A validated questionnaire based on data from an interview-study was distributed to Ro/SSA-positive women supervised with Doppler examinations during their pregnancy (n = 100). Results The response rate was 79%. The majority of the women (61%) reported that the increased number of ultrasound examinations influenced their pregnancy, but in a positive way, with qualified information and additional support from health care personnel in conjunction with the examinations. Further, the visits to the clinic provided opportunities to see the ultrasound picture of the expected infant. However, one-third of the women also reported stress in relation to the examinations. Conclusions Fetal echocardiographic surveillance holds many and predominantly positive effects for Ro/SSA-positive women during pregnancy in addition to the medical advantages. [ABSTRACT FROM AUTHOR]
- Published
- 2015
- Full Text
- View/download PDF
33. Neonatal lupus erythematosus: a review of 123 cases in China.
- Author
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Li, Yi ‐ qun, Wang, Qian, Luo, Yan, and Zhao, Yan
- Subjects
- *
LUPUS erythematosus , *DOMINANCE (Genetics) , *TISSUE wounds , *PREGNANCY - Abstract
Aim To analyze the clinical features, outcomes and prognosis of neonatal lupus erythematosus ( NLE) in China. Method We reviewed 12 NLE cases at the Peking Union Medical College Hospital and compared the data with 111 cases reported in China between 1990 and 2014. The Chinese medical journal search engines used in this study were Wanfang.data and Science China. Results No gender dominance in NLE incidence was found. Cutaneous lesions were present in more than 96% of patients, while cardiac, hematological and hepatobiliary manifestations were seen in 12.61%, 45.53% and 17.89% of cases, respectively. Congenital heart block ( CHB) tended to be more persistent, with two cases showing CHB for 1 year and three cases persisting for 7-10 years. In this study more than 90% of mothers were anti-Sjögren's syndrome A positive, and 65.04% were asymptomatic prior to the pregnancy. Conclusion These results indicate that clinicians, especially dermatologists, in China should improve their recognition of this disease to avoid misdiagnosis, and more attention should be paid to the follow-up of NLE patients and their asymptomatic mothers. [ABSTRACT FROM AUTHOR]
- Published
- 2015
- Full Text
- View/download PDF
34. Doppler echocardiographic isovolumetric time intervals in diagnosis of fetal blocked atrial bigeminy and 2:1 atrioventricular block.
- Author
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Sonesson, S.‐E., Eliasson, H., Conner, P., and Wahren‐Herlenius, M.
- Subjects
- *
DOPPLER echocardiography , *ISOCHORIC processes , *PREGNANCY , *FETAL development , *ATRIOVENTRICULAR node , *LONG QT syndrome - Abstract
ABSTRACT Objective To distinguish between blocked atrial bigeminy ( BB) and incomplete atrioventricular block with 2:1 conduction (2:1 AVB) can be very difficult, especially in the mid-term fetus. Making a correct diagnosis has important clinical implications, as their prognosis and management differ markedly. Our objective was to investigate whether analysis of isovolumetric time intervals could improve Doppler echocardiography in differentiating these conditions. Methods Sixteen fetuses with sustained BB or isolated 2:1 AVB, diagnosed at our tertiary center from 2002 to 2012, were reviewed retrospectively. Doppler recordings of left ventricular in- and outflow, including mitral and aortic valve movements, were used to measure isovolumetric contraction ( ICT) and relaxation ( IRT) time intervals. ICT reference values obtained from 104 normal pregnancies were used for comparison. Results Ten fetuses had BB and six 2:1 AVB. Five of the AVB cases were anti-Ro antibody positive and one had long QT syndrome ( LQTS). ICT was systematically shorter in BB than in antibody-mediated 2:1 AVB. Nine of 10 cases with BB had an ICT below −2 SD and the five with antibody-mediated 2:1 AVB had values at or above +2 SD. All 15 fetuses with either BB or antibody-mediated AVB had an IRT of < 70 ms, as opposed to a markedly prolonged IRT (105 ms) in the LQTS case. Conclusion Measurement of ICT can improve the differential diagnosis between BB and antibody-mediated 2:1 AVB. Fetuses with BB or antibody-mediated AVB are unlikely to have IRT measurements exceeding 70 ms and, when this is observed, LQTS should be considered a more likely diagnosis. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd [ABSTRACT FROM AUTHOR]
- Published
- 2014
- Full Text
- View/download PDF
35. Successful perinatal management and pacemaker stimulation during the first hour of life in a 1.6 kg newborn with autoimmune congenital complete heart block diagnosed prenatally
- Author
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Anna Wójtowicz, Tomasz Mroczek, Hubert Huras, Janusz Skalski, and Agata Włoch
- Subjects
Adult ,medicine.medical_specialty ,Pacemaker, Artificial ,business.industry ,Autoantibody ,Infant, Newborn ,Parturition ,Obstetrics and Gynecology ,Stimulation ,Congenital heart block ,Heart Block ,Congenital complete heart block ,Echocardiography ,Pregnancy ,Internal medicine ,Prenatal Diagnosis ,medicine ,Cardiology ,Humans ,Female ,business - Published
- 2020
36. A multicentre study of 244 pregnancies in undifferentiated connective tissue disease: maternal/fetal outcomes and disease evolution
- Author
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Cristiano M. de Freitas, Silvia Gallo Cassarino, Marcello Govoni, Elena Rubini, Silvia Grazietta Foddai, Massimo Radin, Irene Cecchi, Chiara Benedetto, Luca Marozio, Laura Andreoli, Francesca Crisafulli, Karen Schreiber, Dario Roccatello, Beatrice Bacco, Franco Franceschini, Melissa Padovan, Savino Sciascia, Danieli Andrade, Tiziana Bertero, and Alessandra Bortoluzzi
- Subjects
Adult ,Gestational hypertension ,medicine.medical_specialty ,undifferentiated connective tissue disease ,autoantibodies ,Intrauterine growth restriction ,autoimmune disease ,anti-nuclear antibodies ,congenital heart block ,neonatal lupus ,pregnancy ,pregnancy complications ,NO ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,medicine ,Humans ,Pharmacology (medical) ,030212 general & internal medicine ,Undifferentiated Connective Tissue Diseases ,Retrospective Studies ,030203 arthritis & rheumatology ,Pregnancy ,Fetal Growth Retardation ,Obstetrics ,business.industry ,Pregnancy Outcome ,LS6_12 ,Retrospective cohort study ,Stillbirth ,medicine.disease ,Abortion, Spontaneous ,Gestational diabetes ,Antibodies, Antinuclear ,Cohort ,Antibodies, Antiphospholipid ,Gestation ,Female ,Live birth ,business ,Live Birth - Abstract
Objectives To investigate fetal/perinatal and maternal outcomes from a large multicentre cohort of women diagnosed with UCTD. Methods This multicentre retrospective cohort study describes the outcomes of 224 pregnancies in 133 consecutive women with a diagnosis of UCTD, positive for ANA and aged Results Of the 224 pregnancies analysed, 177 (79%) resulted in live births, 45 (20.1%) in miscarriages (defined as pregnancy loss before 12 weeks’ gestation), 2 (0.9%) in stillbirths (pregnancy loss after 20 weeks’ gestation) and 6 (2.7%) cases showed intrauterine growth restriction. Miscarriages and stillbirths were strongly associated with the presence of aPL and ENA antibodies (P Conclusion In our multicentre cohort, women with UCTD had a live birth rate of 79%. Women with UCTD should be referred to specialist follow-up when planning a pregnancy. ENA profiling and aPL testing should be mandatory in this setting, and further therapeutic approaches and management should be planned accordingly.
- Published
- 2020
37. Could Timing Be Everything for Antibody-Mediated Congenital Heart Block?
- Author
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Shreya S Sheth and Carolyn A. Altman
- Subjects
medicine.medical_specialty ,biology ,business.industry ,030204 cardiovascular system & hematology ,Antibodies ,Congenital heart block ,Fetal Diseases ,03 medical and health sciences ,Fetal Heart ,Heart Block ,0302 clinical medicine ,Pregnancy ,Internal medicine ,medicine ,Cardiology ,biology.protein ,Humans ,Female ,030212 general & internal medicine ,Antibody ,Cardiology and Cardiovascular Medicine ,business - Published
- 2018
38. Preventing congenital neonatal heart block in offspring of mothers with anti-SSA/Ro and SSB/La antibodies: A review of published literature and registered clinical trials.
- Author
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Gleicher, Norbert and Elkayam, Uri
- Subjects
- *
HEART block , *NEONATAL diseases , *AUTOANTIBODIES , *GENETIC disorders , *CLINICAL trials ,MEDICAL literature reviews - Abstract
Abstract: Offspring of women with anti-SSA/Ro–SSB/La antibodies are believed to be at risk for congenital heart block (CHB). Whether this risk can be reduced, and what constitutes standard of care treatment is, however, unclear. The objective of this review therefore was to determine whether currently proposed standard of care treatments to avoid CHB in offspring of mothers at risk are evidence-based. To do so, we conducted a review of the literature under appropriate keywords and phrases in Medline/PubMed and Google Scholar for the years 2000–2013. Reference lists were further reviewed, and relevant manuscripts were pulled. We also reviewed www.clinicaltrials.gov for registered studies. In the absence of randomized prospective clinical trials, a meta-analysis was not feasible. We, therefore, reviewed lower evidence level studies individually. Risk of CHB actually appears more closely associated with general autoimmunity than, specifically, with SSA/Ro–SSB/La antibodies. This and other observations raise questions whether CHB is caused by passively transferred maternal autoimmunity, as is currently widely believed. Observational studies suggest the possible effectiveness of intravenous gamma globulin (IV-Ig) and hydroxychloroquine (Plaquenil) in reducing CHB-risk. Evidence for both is, however, inconclusive, and studies are biased in favor of hydroxychloroquine and against IV-Ig. Based on the review of the literature, current evidence of effectiveness for any treatment has to be judged as insufficient. Among the available treatment options, some considerations favor IV-Ig over hydroxychloroquine or, alternatively, suggest treatment with IV-Ig periconceptionally and into early gestation, with hydroxychloroquine added or replacing IV-Ig at approximately 10weeks gestational age. Benefits for the utilization of steroid drugs are unclear. Since no treatment can be considered as established, prevention of CHB in offspring should be considered experimental, and performed under appropriate study conditions. [Copyright &y& Elsevier]
- Published
- 2013
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39. Ro52 autoantibody-positive women's experience of being pregnant and giving birth to a child with congenital heart block.
- Author
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Tingström, Joanna, Welin Henriksson, Elisabet, Sonesson, Sven-Erik, and Wahren-Herlenius, Marie
- Abstract
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. [Copyright &y& Elsevier]
- Published
- 2013
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40. A combination therapy to treat second-degree anti-Ro/La-related congenital heart block. A strategy to avoid stable third-degree heart block?
- Author
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Ruffatti, A, Milanesi, O, Chiandetti, L, Cerutti, A, Gervasi, MT, De Silvestro, G, Pengo, V, and Punzi, L
- Subjects
- *
HEART block , *IMMUNOGLOBULINS , *PLASMAPHERESIS , *RIBONUCLEASES - Abstract
While mainly based on the use of fluorinated steroids, there is no standard management of anti-Ro/La-related congenital heart block (CHB). This is a report concerning two consecutive cases of anti-Ro/La-related second-degree block treated with betamethasone (4 mg/day), weekly plasmapheresis, and intravenous immunoglobulins (IVIGs; 1 g/kg) administered every 15 days, a therapy that was begun shortly after CHB was detected and continued until delivery. The newborns were also treated with IVIG (1 g/kg) soon after birth and continued fortnightly until the anti-Ro/La antibody levels became undetectable. In both cases second-degree AV block reverted to a stable sinus rhythm with a first-degree atrioventricular (AV) block. Moreover, there was no recurrence of CHB when therapy was suspended, as confirmed by a 29 month and an eight month follow-up, respectively. [ABSTRACT FROM AUTHOR]
- Published
- 2012
- Full Text
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41. Challenges of lupus pregnancies.
- Author
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Doria, A., Tincani, A., and Lockshin, M.
- Published
- 2008
- Full Text
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42. PEDIATRICS ELECTRONIC PAGES.
- Subjects
- *
PEDIATRIC research , *LENGTH of stay in hospitals , *TERMINAL care , *SMOKING cessation - Abstract
The article presents abstracts of several pediatric research, including "Impact of Admission-Day Crowding on the Length of Stay of Pediatric Hospitalization," "Pediatric Residents Clinical and Educational Experiences With End-of-Life Care" and "Effect of a Pediatric Practice-Based Smoking Prevention and Cessation Intervention for Adolescents: A Randomized, Controlled Trial."
- Published
- 2008
43. Th2 immune deviation induced by pregnancy: The two faces of autoimmune rheumatic diseases
- Author
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Doria, Andrea, Iaccarino, Luca, Arienti, Silvia, Ghirardello, Anna, Zampieri, Sandra, Rampudda, Maria Elisa, Cutolo, Maurizio, Tincani, Angela, and Todesco, Silvano
- Subjects
- *
PREGNANCY , *ARTHRITIS , *CONCEPTION , *PREGNANT women - Abstract
Abstract: One of the most important immunological modifications during pregnancy is the Th1/Th2 shift, due to the progressive increase of progesterone and estrogens during pregnancy, which reach their peak-level in the third trimester of gestation. At high levels, estrogens seem mainly to suppress Th1 cytokines and stimulate Th2-mediated immunological responses as well as antibody production. For this reason Th1-mediated diseases, like rheumatoid arthritis (RA), tend to improve and Th2-mediated disease, like systemic lupus erythematosus (SLE), tend to worsen during pregnancy. SLE is the autoimmune rheumatic disease in which pregnancy most frequently occurs because it predominantly affects young females in their childbearing age. Other autoimmune rheumatic diseases, including RA, are less frequently observed during pregnancy due to their low female-to-male ratio and peak onset after the age of 40. This review is focused on the disease course, gestational outcome and management of patients with SLE and RA during pregnancy. [Copyright &y& Elsevier]
- Published
- 2006
- Full Text
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44. Ro52, Ro60 and La IgG autoantibody levels and Ro52 IgG subclass profiles longitudinally throughout pregnancy in congenital heart block risk pregnancies.
- Author
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Strandberg, L., Salomonsson, S., Bremme, K., Sonesson, S.-E., and Wahren-Herlenius, M.
- Subjects
- *
CONGENITAL heart disease , *AUTOANTIBODIES , *SYSTEMIC lupus erythematosus , *HEART block , *PREGNANCY , *IMMUNOGLOBULIN G - Abstract
Congenital heart block occurs in fetuses of Ro/SSA and La/SSB positive women. To investigate the stability of maternal autoantibody levels during pregnancy, we followed Ro52, Ro60 and La autoantibody IgG level variation and Ro52 subclass profiles longitudinally in selected congenital heart block risk pregnancies. Serum samples were obtained from 12 Ro/La positive women diagnosed with a systemic rheumatic disease and followed on average 60 months (range two to 84) which included 13 pregnancies. Seven children were affected by neonatal lupus, whereof four developed complete congenital heart block. Serum was also collected from the babies at birth. Ro52, Ro60 and La IgG as well as subclass antibodies were analysed by ELISA using recombinant antigens. Six Ro/La negative rheumatic patients were included as controls for antibody levels during pregnancy. Ro52, Ro60 and La IgG levels decreased progressively from early to late pregnancy, significantly for Ro52 and Ro60 (P < 0.01). No peaks or persistent elevation of antibody levels were noted in any of the CHB risk pregnancies. Ro52 IgG1 antibody levels were significantly higher than IgG2 (P < 0.01), IgG3 (P < 0.01) and IgG4 (P < 0.05) levels in the mothers during pregnancy. Ro52 IgG1 and IgG4 levels decreased significantly from early to late pregnancy (P = 0.02), while levels of IgG2 and IgG3 were low and the decrease was not significant. All IgG subclasses were transferred to the children. We conclude that maternal levels of Ro52, Ro60 and La autoantibodies tended rather to decrease than to increase during pregnancy. [ABSTRACT FROM AUTHOR]
- Published
- 2006
- Full Text
- View/download PDF
45. Near-zero fluoroscopy implantation of dual-chamber pacemaker in pregnancy using electroanatomic mapping
- Author
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Hakan Paydak, Monica Lo, Jason Payne, and Waddah Maskoun
- Subjects
Electroanatomic mapping ,medicine.medical_specialty ,Heart block ,Case Report ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,medicine ,Complete heart block ,Fluoroscopy ,Diseases of the circulatory (Cardiovascular) system ,030212 general & internal medicine ,Dual Chamber Pacemaker ,Fetus ,Pregnancy ,medicine.diagnostic_test ,business.industry ,Gestational age ,medicine.disease ,RC666-701 ,Cardiology ,Pacemaker, Pregnancy ,Radiology ,3D electroanatomic mapping ,Cardiology and Cardiovascular Medicine ,business ,Near-zero fluoroscopy - Abstract
Background: Complete Heart Block (CHB) is uncommon in pregnancy and challenging to manage due to risks of radiation to the fetus. Methods: A 27-year-old with known 1st degree AV block at 11 weeks gestational age presents with a 5-week history of fatigue, dizziness, and severe dyspnea. BP was 110/60
- Published
- 2017
46. Low titer, isolated anti Ro/SSA 60 kd antibodies is correlated with positive pregnancy outcomes in women at risk of congenital heart block
- Author
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Anna Ghirardello, Marta Tonello, Ornella Milanesi, Silvia Visentin, Alessandra Zambon, Amelia Ruffatti, Elena Mattia, Alessia Cerutti, and Ariela Hoxha
- Subjects
0301 basic medicine ,Time Factors ,Congenital heart block ,Gastroenterology ,0302 clinical medicine ,Pregnancy ,Prospective Studies ,biology ,Incidence ,Pregnancy Outcome ,Antibody titer ,General Medicine ,Anti-Ro/SSA 52kd ,Titer ,Italy ,Antibodies, Antinuclear ,Anti-La/SSB ,Anti-p200 ,Anti-Ro/SSA 60 kd ,Isolated autoantibodies ,Rheumatology ,Female ,Antibody ,Adult ,medicine.medical_specialty ,Pregnancy Complications, Cardiovascular ,Enzyme-Linked Immunosorbent Assay ,03 medical and health sciences ,stomatognathic system ,Internal medicine ,medicine ,Humans ,Clinical significance ,Autoantibodies ,030203 arthritis & rheumatology ,business.industry ,Infant, Newborn ,Autoantibody ,medicine.disease ,eye diseases ,stomatognathic diseases ,Heart Block ,030104 developmental biology ,Immunology ,biology.protein ,business ,Follow-Up Studies ,Anti-SSA/Ro autoantibodies - Abstract
Congenital heart block (CHB) is an autoantibody mediated disorder presumably caused by placental transmission of maternal autoantibodies to Ro/SSA 52 kd, p200, Ro/SSA 60 kd, La/SSB ribonucleoproteins. This study investigated the clinical significance of isolated anti-Ro/SSA 52 kd, anti-p200, anti-Ro/SSA 60 kd, and anti-La/SSB antibodies in positive pregnant patients. One hundred sixty-three pregnant women positive to anti-Ro/SSA 52 kd and/or anti-Ro/SSA 60 kd and/or anti-La/SSB antibodies were prospectively enrolled in the study. Anti-Ro52, anti-Ro60, anti-p200, and anti-La antibodies were assayed using home-made ELISA assays. Isolated antibody positivity was found in 25 women (15.3%), while multiple antibody positivity in 138 (84.7%). Twenty-four developed CHB, and the 139 had a favorable pregnancy outcome. The prevalence of isolated anti-Ro/SSA 60 kd antibodies was significantly higher (p
- Published
- 2017
47. Autoimmune-associated Congenital Heart Block: A New Insight in Fetal Life
- Author
-
Yimin Hua and Kaiyu Zhou
- Subjects
Pediatrics ,medicine.medical_specialty ,Autoimmune Diseases ,Congenital ,Disease Management ,Heart Block ,High-risk ,Pregnancy ,Prenatal Care ,MEDLINE ,lcsh:Medicine ,Prenatal care ,Review Article ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,030203 arthritis & rheumatology ,Fetus ,business.industry ,lcsh:R ,General Medicine ,medicine.disease ,Research findings ,Female ,business - Abstract
Objective: Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with maternal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder. Data Sources: We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords including “Autoimmune-associated”, “Autoimmune-mediated”, and “Congenital heart block”. Study Selection: Articles about autoimmune-associated CHB were obtained and reviewed. Results: Observational studies consistently reported that transplacental maternal antibodies might recognize fetal or neonatal antigens in various tissues and result in immunological damages, but the molecular mechanisms underlying CHB pathogenesis still need illuminated. Multiple factors were involved in the process of atrioventricular block development and progression. While several susceptibility genes had been successfully defined, how these genes and their protein interact and impact each other remains to be explored. With currently available diagnostic tools, fetal ultrasound cardiography, and fetal magnetocardiography, most of CHB could be successfully diagnosed and comprehensively evaluated prenatally. The efficacy of current approaches for preventing the progression and recurrence of CHB and other autoimmune-mediated damages was still controversial. Conclusions: This review highlighted the relationships between autoimmune injuries and CHB and strengthened the importance of perinatal management and therapy for autoimmune-associated CHB.
- Published
- 2017
48. Anti-SSA/Ro and anti-SSB/La antibody-mediated congenital heart block.
- Author
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Costedoat-Chalumeau, N., Georgin-Lavialle, S., Amoura, Z., and Piette, J. -C.
- Subjects
- *
CONGENITAL heart disease in children , *BLOOD plasma , *CONNECTIVE tissues , *CARDIOMYOPATHIES , *IMMUNOGLOBULINS , *STEROIDS - Abstract
When anti-SSA/Ro antibodies are present in sera of mothers with connective tissue diseases, the incidence of congenital heart block (CHB) has been reported to be 1–2% in live births. The risk of recurrence of CHB in a subsequent child remains limited to 10–16%. CHBs are usually complete but CHB of the first or second degree can also be observed. In some cases, CHB is associated with endocardial fibroelastosis. Late-onset cardiomyopathy may occur later in life in these children. The mortality of CHB, which is predominant in utero and in the first months of life, is an estimated 16–19%. A pacemaker is required in about 66% of cases. Curative treatment of CHB is based on fluorinated steroids (dexamethasone or betamethasone) that do cross the placenta in an active form. Guidelines are available but further studies are needed to optimize treatment. [ABSTRACT FROM AUTHOR]
- Published
- 2005
- Full Text
- View/download PDF
49. Any increased risk of autoimmune disease?
- Author
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Cimaz, R.
- Subjects
- *
AUTOIMMUNE diseases , *SYSTEMIC lupus erythematosus , *SKIN diseases , *IMMUNOLOGIC diseases , *HEART diseases , *AUTOIMMUNITY - Abstract
Autoimmune diseases such as systemic lupus erythematosus (SLE) are known to have a strong genetic component, therefore the risk of autoimmune disease might be increased in family members of patients with SLE. However, there are currently no data that support a higher incidence of autoimmune disorders in the offspring of SLE patients. Babies with neonatal lupus are not at increased risk to develop SLE in later life, but a continued follow-up is suggested, especially prior to adolescence and if the mother herself has an autoimmune disease. [ABSTRACT FROM AUTHOR]
- Published
- 2004
- Full Text
- View/download PDF
50. Neonatal Lupus: Review of Proposed Pathogenesis and Clinical Data from the US-based Research Registry for Neonatal Lupus.
- Author
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Buyon, Jil P. and Clancy, Robert M.
- Subjects
- *
MUSCLE cells , *APOPTOSIS , *FETUS , *AUTOANTIBODIES , *PREGNANCY - Abstract
Congenitial heart block (CHB), a life-threatening manifestation of neonatal lupus, offers a unique opportunity to study the effector arm of immunity and define the pathogenicity of an autoantibody in mediating tissue injury. This review focuses on our recent in vitro model which supports a cascade from antibody insult to unchecked fibrosis. In brief, it is proposed that the fetal cardiac myocyte undergoes apoptosis which facilitates transfer of intracellular Ro and La antigens to the surface where they are bound by circulating maternal autoantibodies (anti-SSA/Ro-SSB/La antibodies). Scavenging macrophages phagocytose these inadvertently "opsonized" cardiocytes, leading to the secretion of pro-inflammatory and pro-fibrotic cytokines, the latter of which transdifferentiate fibroblasts into myofibroblasts and thereby promote scarring. Immunohistologic study of a heart from a neonate dying of CHB supports this model in that macrophages and myofibroblasts were demonstrated. To facilitate both basic and clinical research, a Research Registry for Neonatal Lupus was established in 1994 by the U.S. National Institute of Arthritis, Musculoskeletal and Skin Diseases. Maternal and fetal outcomes are addressed as well as recurrence rates. Laboratory evaluation and management decisions during pregnancy are provided. [ABSTRACT FROM AUTHOR]
- Published
- 2003
- Full Text
- View/download PDF
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