949 results on '"congenital heart block"'
Search Results
202. The Diagnostic Significance of Antinuclear Antibodies
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Meurer, M., Ring, Johannes, editor, and Przybilla, Bernhard, editor
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- 1991
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203. Remission of congenital complete heart block without anti-Ro/La antibodies: A case report
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Souvik Mitra, Anindya Kumar Saha, Syamal Kumar Sardar, and Arun Kumarendu Singh
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Anti Ro/La antibody ,congenital heart block ,neonatal bradycardia ,Medicine ,Pediatrics ,RJ1-570 ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Anti-Ro/La negative congenital heart block (CHB) is uncommon. We report one such case of CHB, with no associated structural heart disease or maternal autoantibodies. The heart block reverted to sinus rhythm spontaneously at two weeks of age, and the patient remains in sinus rhythm at a one year followup. Whether patients with antibody negative complete heart block have a different clinical course is conjectural.
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- 2013
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204. Estudo retrospectivo de 76 fetos de mães com lúpus eritematoso sistêmico (LES) Retrospective study of 76 fetus of mother with systemic lupus erythematosus (SLE)
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Marcela Ushida, André Gustavo Cunha Trolezi, and Emilia Inoue Sato
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lúpus neonatal ,anticorpo anti-Ro/SS-A ,anticorpo anti-La/SS-B ,bloqueio cardíaco congênito ,neonatal lupus ,anti-Ro/SS-A antibody ,anti-La/SS-B antibody ,congenital heart block ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
OBJETIVO: avaliar a freqüência de lúpus neonatal, prematuridade, natimortalidade, abortamento e baixo peso em gestações de pacientes com LES atendidas num serviço terciário. MÉTODOS: estudo retrospectivo dos atendimentos a pacientes com LES realizados no centro obstétrico do Hospital São Paulo/Unifesp/ EPM, entre novembro de 1991 e abril de 2003. Os dados clínicos e laboratoriais referentes à mãe e fetos foram obtidos através da revisão de prontuários médicos. RESULTADOS: foram identificadas 60 mulheres com LES que tiveram 75 gestações. A média de idade das mães por ocasião das gestações foi de 27,1± 6,1 anos e a mediana de tempo da doença foi de 48 meses. Duas gestantes necessitaram de diálise peritoneal. Sessenta e três pacientes fizeram uso de prednisona, 4 pacientes receberam pulsoterapia com solumedrol e 2 receberam azatioprina durante a gravidez. No primeiro trimestre da gestação, 6 pacientes haviam feito uso de difosfato de cloroquina e 2 utilizaram hidroxicloroquina. Uma paciente que havia recebido pulso de ciclofosfamida e outra que havia utilizado metotrexato desconhecendo a gravidez evoluíram com abortamento fetal espontâneo. Foram encontrados 13 óbitos intra-uterinos e 7 abordamentos. O parto foi prematuro em 57% das gestações e a média de tempo de gestação foi de 35 semanas. A média de peso ao nascer foi de 2.332±961g (variando de 525 a 3.620g). Foram detectados 5 casos de lúpus neonatal (8,9%), sendo um bloqueio cardíaco congênito (BCC) com óbito intrauterino na 29ª semana e 3 plaquetopenias associadas à anti-Ro/ SSA e 1 neutropenia associada ao anti-RNP. Com exceção do caso com BCC, estes anticorpos foram detectados no soro materno e no sangue do cordão umbilical. Ao final do estudo, foram detectados dois óbitos de recém-nascidos, com 9 e 130 dias de vida, em decorrência de complicações com a prematuridade. CONCLUSÕES: com relação à literatura, este estudo mostrou similar prevalência de perda fetal (aborto e óbito intra-uterino), entretanto, a freqüência de prematuridade foi maior que a média referida em outros trabalhos. Manifestações hematológicas do lúpus neonatal, como leucopenia e plaquetopenia foram observadas em freqüência maior do que a referida na literatura. Por serem transitórias e assintomáticas, estas alterações podem ter sido subdiagnosticadas.OBJECTIVE: to evaluate the frequency of neonatal lupus, prematurity, fetal loss, and low weight in pregnancies of SLE patients attended at a tertiary health service. MEHTODS: it is a retrospective study evaluating all SLE patients attended at obstetric center of Hospital São Paulo/Unifesp/EPM from November, 1991 to April, 2003. The mother and children's clinical and laboratory data were obtained reviewing medical records. RESULTS: sixty women and 75 pregnancies were identified. The mother age average during the pregnancies was 27.1±6.1 years old and the median of disease duration was 48 months. Two patients needed to be submitted to dialysis during the pregnancy. Sixty-three patients used prednisone, 4 received methilprednisolone pulsetherapy and 2 received azathioprine during the pregnancy. During the first trimester of pregnancy, 6 patients received diphosphate chloroquine and 2 received hidroxychloroquine. Two patients unknowing pregnancy received pulse of cyclophosphamide and another one had used methotrexate and both presented spontaneous abortion. It was observed 13 cases of intrauterine death and 7 abortions. Preterm birth occurred in 57% of pregnancies and the average duration of gestation was 35 weeks. The mean weight of the newborns was 2,332±961g (ranging from 525 to 3,620g). Five cases of neonatal lupus were identified (8,9%). One with congenital heart block (CHB) had intrauterine death at the 29th week of gestation, had 3 babies with thrombocytopenia associated with anti-Ro/SSA antibodies and one with neutropenia associated with anti-RNP antibody. Excepting the case of CHB with intrauterine death, these antibodies were detected on maternal and newborn blood sample. At the end of the study, it was detected two deaths of children with 9 and 130 days of life, due to complications related to prematurity. CONCLUSIONS: comparing with published reports this study showed a similar frequency of fetal loss (abortion and intrauterine death), however the frequency of prematurity was higher than the mean reported in the literature. Hematological features of neonatal lupus, as leucopenia and thrombocytopenia were observed at higher frequency than reported in the literature. As transitory and asymptomatic features, they could have been under-diagnosed.
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- 2004
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205. Lupus Erythematosus and Lupus Anticoagulant
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Benirschke, Kurt, Kaufmann, Peter, Benirschke, Kurt, and Kaufmann, Peter
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- 1990
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206. Hydrops Fetalis
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Benirschke, Kurt, Kaufmann, Peter, Benirschke, Kurt, and Kaufmann, Peter
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- 1990
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207. General Physiology and Biochemistry
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Chard, Tim, Lilford, Richard, Chard, Tim, and Lilford, Richard
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- 1990
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208. Researchers from University of California San Francisco (UCSF) Provide Details of New Studies and Findings in the Area of Congenital Heart Block [A Case of Neonatal Lupus Presenting With Myocardial Dysfunction In the Absence of Congenital Heart ...].
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HEART block ,HEART ,ARRHYTHMIA ,CONGENITAL disorders ,HEART diseases - Published
- 2023
209. Congenital second-degree heart block and total anomalous pulmonary venous return associated with microduplication of 1q32.2.
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Puvabanditsin, Surasak, Puthenpura, Vidya, Gueye-Ndiaye, Seyni, Takyi, Michele, Madubuko, Adaora, Walzer, Lauren, and Mehta, Rajeev
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CHROMOSOME abnormalities , *CONGENITAL heart disease , *GENES , *GENETIC polymorphisms , *HEART block , *NUCLEOTIDES , *HEALTH literacy , *OLIGONUCLEOTIDE arrays , *SCIMITAR syndrome - Published
- 2018
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210. Congenital Heart Block in Subsequent Pregnancies of SSA/Ro-Positive Mothers
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Annette Wacker-Gussmann and Janette F. Strasburger
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Pediatrics ,medicine.medical_specialty ,2019-20 coronavirus outbreak ,Coronavirus disease 2019 (COVID-19) ,business.industry ,Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) ,MEDLINE ,Hydroxychloroquine ,Congenital heart block ,medicine ,Cardiology and Cardiovascular Medicine ,business ,Magnetocardiography ,medicine.drug ,Anti-SSA/Ro autoantibodies - Published
- 2020
211. Wide heterogeneity in treatment protocols and inappropiate use of prednisolone for anti-Ro/La associated-congenital heart block: a systematic review of 492 cases
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Benazir Hymabaccus, Yusuf Ziya Şener, Serdar Ceylan, Emre Bilgin, Omer Karadag, Fatih Gürler, Umut Kalyoncu, B. Armagan, Levent Kılıç, Abdulsamet Erden, and Alper Sari
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medicine.medical_specialty ,business.industry ,Internal medicine ,Prednisolone ,medicine ,business ,Congenital heart block ,Rheumatology ,medicine.drug ,Anti-SSA/Ro autoantibodies - Published
- 2019
212. First clinical experience with the Kora pacemaker system in congenital complete heart block in newborn infants
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Gernot Grangl, Ante Burmas, Stefan Kurath-Koller, Sabrina Schweintzger, Martin Koestenberger, and Andreas Gamillscheg
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Male ,Cardiac output ,Pacemaker, Artificial ,medicine.medical_specialty ,Time Factors ,Congenital heart block ,Pacemaker system ,Sampling Studies ,Electrocardiography ,03 medical and health sciences ,Child Development ,Rare Diseases ,0302 clinical medicine ,Heart Rate ,030225 pediatrics ,Internal medicine ,Heart rate ,medicine ,Humans ,030212 general & internal medicine ,Adverse effect ,Children ,business.industry ,Infant, Newborn ,lcsh:RJ1-570 ,lcsh:Pediatrics ,Equipment Design ,Pacemaker ,Heart Block ,Treatment Outcome ,Congenital complete heart block ,Tolerability ,Echocardiography ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Cardiology ,Female ,business ,Respiratory minute volume ,Follow-Up Studies ,Research Article ,Skin traction - Abstract
Background To report first clinical experience on three cases of congenital complete heart block and the use of a pacemaker system with a maximum lower rate interval of 95 beats per minute. Methods We retrospectively analyzed three patients treated with a pacemaker system with a maximum lower rate interval of 95 beats per minute suffering from congenital complete heart block. We report a follow up period of 2.9 years, focusing on the patients’ growth, development, and adverse events, as well as pacemaker function. Results In all three patients pacemaker function was impeccable, including minute ventilation sensor rate adaption. All patients showed limited growths as expected, adequate development, good feeding tolerability and circadiane heart rate adaption. One patient experienced skin traction and revision. All patients showed high aortic velocity time integral values after birth. Conclusion The use of a pacemaker system with a maximum lower rate interval of 95 beats per minute in infants suffering from congenital complete heart block and showing high aortic VTI values seems to be feasible and to result in limited growths but adequate development. Electronic supplementary material The online version of this article (10.1186/s12887-019-1494-7) contains supplementary material, which is available to authorized users.
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- 2019
213. Posterior Spinal Fusion in a Scoliotic Patient With Congenital Heart Block Treated With Pacemaker
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Chris Yin Wei Chan, Weng Hong Chung, Mun Keong Kwan, Jaseemuddeen Abu Bakar, Kok Han Chee, Mohd Shahnaz Hasan, Chee Kidd Chiu, and Tat Seng Wong
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Male ,Pacemaker, Artificial ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Scoliosis ,Thoracic Vertebrae ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,medicine ,Humans ,Lead Dislodgement ,Orthopedics and Sports Medicine ,Thoracotomy ,030222 orthopedics ,business.industry ,medicine.disease ,Surgery ,Heart Block ,Spinal Fusion ,medicine.anatomical_structure ,Congenital complete heart block ,Spinal fusion ,Thoracic vertebrae ,Neurology (clinical) ,Permanent pacemaker ,business ,030217 neurology & neurosurgery - Abstract
Study design Case report. Objective To describe the technical difficulties on performing posterior spinal fusion (PSF) on a pacemaker-dependent patient with complete congenital heart block and right thoracic scoliosis. Summary of background data Congenital complete heart block requires pacemaker implantation at birth through thoracotomy, which can result in scoliosis. Corrective surgery in this patient was challenging. Height gain after corrective surgery may potentially cause lead dislodgement. The usage of monopolar electrocautery may interfere with the function of the implanted cardiac device. Methods A 17-year-old boy was referred to our institution for the treatment of right thoracic scoliosis of 70°. He had underlying complete congenital heart block secondary to maternal systemic lupus erythematosus. Pacemaker was implanted through thoracotomy since birth and later changed for four times. PSF was performed by two attending surgeons with a temporary pacing inserted before the surgery. The monopolar electrocautery device was used throughout the surgery. Results The PSF was successfully performed without any technical issues and complications. Postoperatively, his permanent pacemaker was functioning normally. Three days later, he was recovering well and was discharged home from hospital. Conclusion This case indicates that PSF can be performed successfully with thoughtful anticipation of technical difficulties on a pacemaker-dependent patient with underlying congenital heart block. Level of evidence 5.
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- 2019
214. Neonatal lupus
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T. P. Makarova, Kh. M. Vakhitov, N. N. Firsova, Yu. S. Melnikova, and T. A. Novitskaya
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integumentary system ,children ,congenital heart block ,Pediatrics, Perinatology and Child Health ,skin lesion ,neonatal lupus ,Pediatrics ,RJ1-570 - Abstract
Neonatal lupus is a complex of symptoms diagnosed in newborns whose mothers suffer from autoimmune rheumatic diseases; it is manifested by two main signs: diseases of the skin and heart. A newborn may have classic ring-shaped erythema, telangiectasia, skin atrophy, follicular plugs and scales. This skin lesion is usually combined with a partial or complete heart block due to fibrosis of its pathways. There is a Clinical case of a child with neonatal lupus from own practice.Conflict of interest: The authors of this article confirmed the lack of conflict of interest and financial support, which should be reported.
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- 2018
215. Case series of late lead dislodgement of Medtronic SelectSecure 3830 pacing leads in growing paediatric patients
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Sarah Gallagher, Kevin Walsh, and Li Yen Ng
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Bradycardia ,medicine.medical_specialty ,Atrium (architecture) ,business.industry ,Late lead dislodgement ,Congenital heart block ,Surgery ,law.invention ,Transvenous pacing ,Congenital complete heart block ,law ,Case report ,Paediatric pacing ,Pacemaker lead failure ,medicine ,Lead Dislodgement ,Artificial cardiac pacemaker ,Case Series ,AcademicSubjects/MED00200 ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Lead (electronics) ,Paediatric patients - Abstract
Background The SelectSecure lumenless 3830 pacing lead is often considered to be the pacing lead of choice for transvenous pacing in children because of its small diameter, lead strength, and reliable long-term sensing and pacing characteristics. One of the potential long-term pitfalls of a sturdy pacing lead is relative retraction with growth in children resulting in late lead dislodgement.Case summary We report two cases of late SelectSecure 3830 lead dislodgement at 11.8 years (Case 1) and 8.8 years (Case 2), respectively, post the initial implantation. Case 1 was diagnosed with congenital complete heart block (CHB) at 9 months old when he presented with unconfirmed diphtheria infection. Case 2 was diagnosed with CHB at 14 weeks of age with positive maternal anti-Ro antibodies. Both patients underwent implantation of a transvenous permanent pacemaker implantation with Medtronic SelectSecure 3830 lead due to symptomatic bradycardia. Apart from a pulse generator change at 8.5 years (Case 1) and 7 years (Case 2), respectively, post-implant due to normal battery depletion, both patients are well in the interim.Discussion As part of the pacemaker follow-up for rapidly growing children, we recommend more frequent surveillance of lead ‘tautness’ by chest radiography especially in children with CHB with no underlying heart rhythm.
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- 2021
216. Neonatal lupus erythematosus: a review of 123 cases in China.
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Li, Yi ‐ qun, Wang, Qian, Luo, Yan, and Zhao, Yan
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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]
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- 2015
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217. Extraction of SelectSecure leads compared to conventional pacing leads in patients with congenital heart disease and congenital atrioventricular block.
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Shepherd, Emma, Stuart, Graham, Martin, Rob, and Walsh, Mark A.
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Background SelectSecure™ pacing leads (Medtronic Inc) are increasingly being used in pediatric patients and adults with structural congenital heart disease. The 4Fr lead is ideal for patients who may require lifelong pacing and can be advantageous for patients with complex anatomy. Objective The purpose of this study was to compare the extraction of SelectSecure leads with conventional (stylette-driven) pacing leads in patients with structural congenital heart disease and congenital atrioventricular block. Methods The data on lead extractions from pediatric and adult congenital heart disease (ACHD) patients from August 2004 to July 2014 at Bristol Royal Hospital for Children and the Bristol Heart Institute were reviewed. Multivariable regression analysis was used to determine whether conventional pacing leads were associated with a more difficult extraction process. Results A total of 57 patients underwent pacemaker lead extractions (22 SelectSecure, 35 conventional). No deaths occurred. Mean age at the time of extraction was 17.6 ± 10.5 years, mean weight was 47 ± 18 kg, and mean lead age was 5.6 ± 2.6 years (range 1–11 years). Complex extraction (partial extraction/femoral extraction) was more common in patients with conventional pacing leads at univariate ( P < .01) and multivariate ( P = .04) levels. Lead age was also a significant predictor of complex extraction ( P < .01). Conclusion SelectSecure leads can be successfully extracted using techniques that are used for conventional pacing leads. They are less likely to be partially extracted and are less likely to require extraction using a femoral approach compared with conventional pacing leads. [ABSTRACT FROM AUTHOR]
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- 2015
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218. The effect of a triple therapy on maternal anti-Ro/SS-A levels associated to fetal cardiac manifestations.
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Martínez-Sánchez, Nuria, Robles-Marhuenda, Ángel, Álvarez-Doforno, Rita, Viejo, Aurora, Antolín-Alvarado, Eugenia, Deirós-Bronte, Lucía, and Bartha, Jose Luis
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- *
HEART block , *COMBINATION drug therapy , *AUTOANTIBODIES , *CONGENITAL heart disease , *THERAPEUTIC use of immunoglobulins , *STEROID drugs , *MATERNAL health , *THERAPEUTICS - Abstract
Objective To evaluate the efficacy and safety of the combination of steroids, plasmapheresis and intravenous immunoglobulins (IVIG) on maternal anti Ro/SS-A antibody levels in cases of fetal cardiac involvement. Material and methods A series of three cases of positive anti-Ro/SS-A mothers with fetuses showing mild cardiac involvement were treated with a triple therapy composed of steroids, plasmapheresis and IVIG. Maternal antibody levels were measured several times before and after the application of each cycle of therapy. The effect of the treatment on fetal cardiac manifestations was also evaluated. Results Maternal anti-Ro/SS-A levels significantly decreased after each cycle of either plasmapheresis or IVIG therapy. The most significant decrease occurred after the first cycle. The natural evolution of the disease was stopped by this therapy in two of these cases, signs of cardiac inflammation decrease and none of the newborns needed neonatal pacemaker. Conclusions A triple therapy combining plasmapheresis, IVIG and glucocorticoids may stop the natural evolution of the fetal cardiac affectation in positive anti-Ro/SS-A antibody patients. Further studies are needed in order to validate clinical applications of this treatment approach. [ABSTRACT FROM AUTHOR]
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- 2015
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219. « Lupus néonatal » : revue de la littérature.
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Morel, N., Georgin-Lavialle, S., Levesque, K., Guettrot-Imbert, G., Le Guern, V., Le Bidois, J., Bessières, B., Brouzes, C., Le Mercier, D., Villain, E., Maltret, A., and Costedoat-Chalumeau, N.
- Abstract
Résumé Le lupus érythémateux néonatal se manifeste par une atteinte cutanée, hématologique, hépatique ou cardiaque incluant un éventuel bloc auriculo-ventriculaire congénital (BAVc) survenant sur un cœur indemne de cardiopathie malformative, une cardiomyopathie dilatée et une fibroélastose endocardique. Ce syndrome rare est constamment associé à la présence chez la mère d’anticorps anti-SSA/Ro et/ou anti-SSB/La. La fréquence du BAVc chez les femmes ayant une connectivite associée à un anticorps anti-SSA/Ro est estimée entre 1 et 2 %, et le risque de récurrence est évalué à environ 19 % lors d’une grossesse ultérieure. Les atteintes cutanées et systémiques sont transitoires, contrairement aux atteintes cardiaques et notamment au BAVc qui est définitif, associé à une morbidité et à une mortalité d’environ 18 %. La mise en place d’un pacemaker est nécessaire dans 2/3 des cas. Une myocardite peut être associée d’emblée ou apparaître plus tardivement. Les mères d’enfants ayant un BAVc sont le plus souvent asymptomatiques au moment de la découverte du BAVc. La physiopathologie n’est pas complètement élucidée à ce jour. Lorsqu’une femme enceinte est porteuse d’un anticorps anti-SSA/Ro, une surveillance échographique obstétricale sera réalisée tous les 15 jours entre 16 et 24 semaines d’aménorrhée et un électrocardiogramme sera fait de principe chez l’enfant. L’intérêt d’un traitement curatif in utero du BAVc par corticoïdes fluorés n’est à ce jour pas démontré. Un traitement préventif par hydroxychloroquine semble diminuer le risque de récidive du BAVc chez les femmes ayant déjà eu un enfant atteint. Une étude prospective est en cours pour confirmer ces résultats. Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings. [ABSTRACT FROM AUTHOR]
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- 2015
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220. Fluorinated steroids are not superior to any treatment to ameliorate the outcome of autoimmune mediated congenital heart block: a systematic review of the literature and meta-analysis
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Objectives.Fluorinated steroids are largely the therapeutic approach of autoimmune mediated congenital heart block (CHB). We performed a meta-analysis to assess the efficacy of fluorinated steroids for the treatment of CHB.Methods.Studies evaluating the efficacy of fluorinated steroids versus no treatment in CHB patients were identified in electronic databases. Random-effects model was used to pool odds ratio (OR) (with 95% CI) of live births as the primary outcome. ORs of CHB progression, pacemaker implantation and extranodal disease were the secondary outcome. Subgroup analysis according to CHB grade and study type was performed.Results.Data from nine studies involving 747 patients were analysed. The overall live birth rates were 86.8% and 86.7%, respectively, in the fluorinated steroids exposed foetuses and in the non-exposed ones. Fluorinated steroids did not ameliorate overall survival in CHB (OR 1.02; 95% CI: 0.65-1.61) with any significant statistical heterogeneity between studies (I-2 0%, p=0.45). No significant differences for the progression of CHB, the pacing and the presence of extranodal disease were observed. Subgroup analysis revealed a significant protective role of fluorinated steroids for survival in 3rd degree CHB and for pacing in monocentric studies, OR 4.07; 95% CI: 1.10-15.08 and OR 0.15; 95% CI: 0.02-0.99, respectively.Conclusions.This meta-analysis shows that fluorinated steroids are not superior to any treatment in patients with CHB in terms of live birth, prevention of progression of incomplete CHB, pacemaker implantation and extranodal disease. Thus, considering their side effects, their use in CHB patients should be discouraged.
- Published
- 2020
221. Lúpus materno evidenciado por bloqueio cardíaco congênito.
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Modesto Santos, Vitorino, Montes Araújo, Monique Chiovatto, Medeiros Santos, Uliana, Rita Fachinelli, Leticia, Gomes Gebrim, Daniela, and Montes Araujo, Marcella Chiovatto
- Abstract
Copyright of Revista de Medicina e Saúde de Brasília is the property of Revista de Medicina e Saude de Brasilia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Published
- 2015
222. Absence of an association between anti-Ro antibodies and prolonged QTc interval in systemic sclerosis: A multicenter study of 689 patients.
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Massie, Charles, Hudson, Marie, Tatibouet, Solène, Steele, Russell, Huynh, Thao, Fritzler, Marvin J., Baron, Murray, and Pineau, Christian A.
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Objective To examine the association between anti-Ro antibodies, namely anti-Ro60/SS-A and anti-Ro52/TRIM21, together and separately, and a prolonged QT interval corrected for heart rate (QTc) in systemic sclerosis (SSc) patients. Methods A total of 689 SSc patients enrolled in a multicenter cohort study underwent a 12-lead resting EKG at baseline. The QTc interval was measured, and a QTc ≥ 440 ms was considered prolonged. Detailed clinical data and sera of these patients were collected and positivity for anti-Ro60/SS-A and anti-Ro52/TRIM21 antibodies was determined using an addressable laser bead immunoassay (ALBIA). Results QTc prolongation was common in this SSc cohort (25%). In a univariate analysis, Ro antibodies, together or separately, were not associated with prolongation of the QTc interval [mean difference in QTc in anti-Ro antibody positive versus negative subjects was −2.2 ms ( p = 0.5748), in anti-Ro60/SS-A antibody positive versus negative subjects was 1.3 ms ( p = 0.8616), and in anti-Ro52/TRIM21 antibody positive versus negative subjects was −3.3 ms ( p = 0.4106)]. In a multivariate logistic regression analysis adjusting for possible confounders, there was no association between prolonged QTc and anti-Ro antibodies [odds ratio (OR) = 0.74, 95% confidence interval (CI): 0.45, 1.22], anti-Ro60/SS-A antibodies (OR = 1.57, 95% CI: 0.72, 3.41), and anti-Ro52/TRIM21 antibodies (OR = 0.76, 95% CI: 0.46, 1.26). However, in both univariate and multivariate analyses, QTc prolongation was associated with longer disease duration, greater disease severity, and the presence of anti-RNA polymerase III antibodies. Conclusions QTc prolongation is common in SSc, although anti-Ro antibodies do not seem to be associated with it as is the case in systemic lupus erythematosus. The reasons for this difference as well as the cause of abnormalities in cardiac repolarization in SSc will require additional studies. [ABSTRACT FROM AUTHOR]
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- 2014
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223. Histological finding of maternal antibody-associated congenital heart block accompanied by large atrial septal defect and severe cardiac dysfunction.
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Endo Y, Kawai Y, and Yamagishi M
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- Female, Humans, Child, Preschool, Heart Block diagnosis, Heart Block etiology, Heart Block therapy, Heart Septal Defects, Atrial complications, Heart Septal Defects, Atrial diagnosis, Heart Septal Defects, Atrial surgery, Pacemaker, Artificial adverse effects
- Abstract
Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.
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- 2022
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224. Ventricular pacing and myocardial function in patients with congenital heart block: Is it time to consider de novo biventricular pacing?
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Seshadri Balaji and Anjan S. Batra
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medicine.medical_specialty ,business.industry ,Heart Ventricles ,medicine.medical_treatment ,Cardiac resynchronization therapy ,Ventricular pacing ,Myocardial function ,Congenital heart block ,Cardiac Resynchronization Therapy ,Heart Block ,Physiology (medical) ,Internal medicine ,Cardiology ,Humans ,Medicine ,In patient ,Cardiology and Cardiovascular Medicine ,business - Published
- 2021
225. B-PO02-159 IMPACT OF PACING ON LEFT VENTRICULAR FUNCTION IN PATIENTS WITH CONGENITAL HEART BLOCK
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Aditya Bhonsale, Sandeep Jain, Floyd Thoma, Samir Saba, Suresh Mulukutla, Gautam Rangavajla, Krishna Kancharla, and Mark Estes
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medicine.medical_specialty ,Ventricular function ,business.industry ,Physiology (medical) ,Internal medicine ,Cardiology ,Medicine ,In patient ,Cardiology and Cardiovascular Medicine ,business ,Congenital heart block - Published
- 2021
226. Successful 2-Stage Pacemaker Implantation for Atrioventricular Block in a Low-Birth-Weight Infant with Congenital Heart Disease: Case Report
- Author
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Matthew A. Hazle, Leigh Kline, and Yoshio Ootaki
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Heart Defects, Congenital ,medicine.medical_specialty ,Pacemaker, Artificial ,Heart disease ,Pacemaker system ,Congenital heart block ,Pacemaker implantation ,Electrocardiography ,Heart Rate ,Internal medicine ,medicine ,Humans ,Stage (cooking) ,Atrioventricular Block ,business.industry ,Infant, Newborn ,General Medicine ,Infant, Low Birth Weight ,medicine.disease ,Low birth weight ,medicine.anatomical_structure ,Ventricle ,cardiovascular system ,Cardiology ,Surgery ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Atrioventricular block - Abstract
Congenital heart block is a potentially life-threatening condition with high morbidity and mortality, especially in the presence of congenital heart disease. We present the case of a low-body-weight premature infant with complex single ventricle congenital heart disease and high-grade atrioventricular block. A 2-staged pacing approach provided atrio-ventricular synchrony and allowed her to grow until a permanent dual-chamber pacemaker system could be implanted.
- Published
- 2020
227. Mortality in congenital heart block – Authors' reply
- Author
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Nathalie Morel, N. Costedoat-Chalumeau, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Conservatoire National des Arts et Métiers [CNAM] (CNAM), HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-HESAM Université - Communauté d'universités et d'établissements Hautes écoles Sorbonne Arts et métiers université (HESAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Serva, Camille, and Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,[SDV.MHEP.RSOA] Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,business.industry ,Immunology ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,Internal medicine ,medicine ,Cardiology ,Immunology and Allergy ,business ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
- Published
- 2020
228. Apoptosis
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Rovenský, Jozef, editor and Payer, Juraj, editor
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- 2009
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229. Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.
- Author
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Kasar, Taner, Saygı, Murat, Özyılmaz, İsa, and Ergül, Yakup
- Subjects
- *
HEART block , *CONGENITAL heart disease , *TWINS , *DISEASE remission , *DIAGNOSIS - Abstract
Background: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. Case Report: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin's physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute. The patient was confirmed to have congenital complete atrioventricular block. Conclusion: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants. [ABSTRACT FROM AUTHOR]
- Published
- 2017
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230. Successful Preventive Treatment of Congenital Heart Block During Pregnancy in a Woman with Systemic Lupus Erythematosus with Anti-La/Ro Antibody.
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Pratibha, Vishwakarma, Kundavi, Shankar, Thangam, Varma, and Ramakrishnan, S.
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- 2016
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231. 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.
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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
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232. Molecular mechanisms of congenital heart block.
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Ambrosi, Aurélie, Sonesson, Sven-Erik, and Wahren-Herlenius, Marie
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- *
FETAL heart , *MOLECULAR biology , *CONGENITAL heart disease , *HEART block , *AUTOANTIBODIES , *PREGNANCY complications , *DISEASE relapse , *DISEASES - Abstract
Abstract: Autoantibody-associated congenital heart block (CHB) is a passively acquired autoimmune condition associated with maternal anti-Ro/SSA antibodies and primarily affecting electric signal conduction at the atrioventricular node in the fetal heart. CHB occurs in 1–2% of anti-Ro/SSA antibody-positive pregancies and has a recurrence rate of 12–20% in a subsequent pregnancy. Despite the long-recognized association between maternal anti-Ro/SSA autoantibodies and CHB, the molecular mechanisms underlying CHB pathogenesis are not fully understood, but several targets for the maternal autoantibodies in the fetal heart have been suggested. Recent studies also indicate that fetal susceptibility genes determine whether an autoantibody-exposed fetus will develop CHB or not, and begin to identify such genes. In this article, we review the different lines of investigation undertaken to elucidate the molecular pathways involved in CHB development and reflect on the hypotheses put forward to explain CHB pathogenesis as well as on the questions left unanswered and that should guide future studies. [Copyright &y& Elsevier]
- Published
- 2014
- Full Text
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233. Overview of Neonatal Lupus.
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Johnson, Benay
- Abstract
Abstract: Neonatal lupus (NL) is defined by the presentation of the fetus and the newborn who possess autoantibodies received from the mother. It is the dysfunction of the maternal immune system that leads to the production of autoantibodies to anti–Sjögren syndrome–A, anti–Sjögren syndrome–B, and anti-ribonuclear protein antigens. These antibodies are shared through the placenta and produce bodily changes in the fetal skin and heart, as well as potential changes in other body systems. Congenital complete heart block is the most dangerous manifestation of NL that can occur in utero or after birth. This article will provide an overview the presentation of NL and current therapies. Prenatal steroids have been the mainstay of therapy to try to reverse first- and second-degree congenital heart block and to prevent progression to a more advanced stage. New therapies are combining steroids with intravenous immunoglobulin and plasmapheresis. This article will provide guidelines for practitioners so they can consider NL as a differential diagnosis when presented with cutaneous lesions, congenital heart block, or abnormal findings in the hematologic, hepatobiliary, neurologic, and musculoskeletal systems. [Copyright &y& Elsevier]
- Published
- 2014
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234. The HLA locus contains novel foetal susceptibility alleles for congenital heart block with significant paternal influence.
- Author
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Meisgen, S., Östberg, T., Salomonsson, S., Ding, B., Eliasson, H., Mälarstig, A., Alfredsson, L., Klareskog, L., Hamsten, A., Olsson, T., Axelsson, T., Gadler, F., Jonzon, A., Sonesson, S. ‐ E., Kockum, I., and Wahren ‐ Herlenius, M.
- Subjects
- *
HLA histocompatibility antigens , *DISEASE susceptibility , *ALLELES , *HEART block , *CONGENITAL heart disease , *SINGLE nucleotide polymorphisms - Abstract
Objective The main aim of this study was to identify foetal susceptibility genes on chromosome six for Ro/ SSA autoantibody-mediated congenital heart block. Subjects and Design Single nucleotide polymorphism ( SNP) genotyping of individuals in the Swedish Congenital Heart Block ( CHB) study population was performed. Low-resolution HLA-A, -Cw and - DRB1 allele typing was carried out in 86 families comprising 339 individuals (86 Ro/ SSA autoantibody-positive mothers, 71 fathers, 87 CHB index cases and 95 unaffected siblings). Results A case-control comparison between index cases and population-based out-of-study controls ( n = 1710) revealed association of CHB with 15 SNPs in the 6p21.3 MHC locus at a chromosome-wide significance of P < 2.59 × 10−6 ( OR 2.21-3.12). In a family-based analysis of association of SNP markers as well as distinct MHC class I and II alleles with CHB, HLA- DRB1*04 and HLA-Cw*05 variants were significantly more frequently transmitted to affected individuals ( P < 0.03 and P < 0.05, respectively), whilst HLA- DRB1*13 and HLA-Cw*06 variants were significantly less often transmitted to affected children ( P < 0.04 and P < 0.03). We further observed marked association of increased paternal (but not maternal) HLA- DRB1*04 transmission to affected offspring ( P < 0.02). Conclusions HLA- DRB1*04 and HLA-Cw*05 were identified as novel foetal HLA allele variants that confer susceptibility to CHB in response to Ro/ SSA autoantibody exposure, whilst DRB1*13 and Cw*06 emerged as protective alleles. Additionally, we demonstrated a paternal contribution to foetal susceptibility to CHB for the first time. [ABSTRACT FROM AUTHOR]
- Published
- 2014
- Full Text
- View/download PDF
235. Lupus eritematoso neonatal: revisión de casos en los últimos 5 anos.
- Author
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Porcel Chacón, Rocío, Tapia Ceballos, Leopoldo, Díaz Cabrera, Rocío, and Gutiérrez Perandones, María Teresa
- Abstract
Neonatal lupus erythematosus is an infrequent disease seen in newborns. It is caused by transplacental maternal autoantibody passage. Cutaneous involvement and congenital heart block (CHB) are the most common affections, although it may involve multiple organs like the liver, lungs, blood, nervous or digestive systems. This article present a review of the four cases diagnosed in the past five years in a Neonatal Unit, which shows the different clinical spectrum which can develop around this disease (CHB, multisystemic affection and two cutaneous cases), different autoantibodies (specially anti-SSA) with an early negativization during the first year of life and the possibility of future collagen vascular disease as occurred in one case. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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236. CNS vasculitis and stroke in neonatal lupus erythematosus: A case report and review of literature.
- Author
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Saini, Arushi G., Sankhyan, Naveen, Bhattad, Sagar, Vyas, Sameer, Saikia, Biman, and Singhi, Pratibha
- Abstract
Abstract: Neonatal lupus erythematosus refers to the clinical spectrum of cardiac, cutaneous and other systemic abnormalities in neonates born to mothers with autoantibodies against Ro/SSA and La/SSB antigens. Isolated central nervous system involvement is very rare and has been described as transient vasculopathy only. We describe a 2-months-old girl who presented with acute ischemic stroke secondary to central nervous system vasculitis without any cardiac, cutaneous or hematological manifestations. The mother was pauci-symptomatic with raised anti-Ro autoantibody titers; the baby was positive for autoantibodies against Ro-antigen. Angiography confirmed vasculitis in cerebral vasculature. Our case highlights that neonatal lupus erythematosus can present with isolated nervous system manifestations and the vascular damage can be permanent in the form of vasculitis. Early recognition will help pediatricians identify such possible permanent complications in newborns with neonatal lupus erythematosus. A review of previously reported central nervous system manifestations of neonatal lupus is also presented. [Copyright &y& Elsevier]
- Published
- 2014
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237. Towards computational modelling of the human foetal electrocardiogram: normal sinus rhythm and congenital heart block.
- Author
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Pervolaraki, Eleftheria, Hodgson, Sam, Holden, Arun V., and Benson, Alan P.
- Abstract
Aims We aim to engineer a computational model of propagation during normal sinus rhythm in the foetal human heart, by modifying models for adult cardiac tissue to match foetal electrocardiogram (fECG) characteristics. The model will be partially validated by fECG data, and applied to explore possible mechanisms of arrhythmogenesis in the foetal heart. Methods and results Foetal electrocardiograms have been recorded during pregnancy, with P- and T-waves, and the QRS complex, identified by averaging and signal processing. Intervals of the fECG are extracted and used to modify currently available human adult cardiomyocyte models. RR intervals inform models of the pacemaking cells by constraining their rate, the QT interval and its rate dependence constrain models of ventricular cells, and the width of the P-wave, the QR and PR intervals constrain propagation times, conduction velocities, and intercellular coupling. These cell models are coupled into a one-dimensional (1D) model of propagation during normal sinus rhythm in the human foetal heart. We constructed a modular, heterogeneous 1D model for propagation in the foetal heart, and predicted the effects of reduction in L-type Ca++ current. These include bradycardia and atrioventricular conduction blocks. These may account quantitatively for congenital heart block produced by positive IgG antibodies. Conclusion The fECG can be interpreted mechanistically and quantitatively by using a simple computational model for propagation. After further validation, by clinical recordings of the fECG and the electrophysiological experiments on foetal cardiac cells and tissues, the model may be used to predict the effects of maternally administered pharmaceuticals on the fECG. [ABSTRACT FROM PUBLISHER]
- Published
- 2014
- Full Text
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238. The unique neonatal NK cells: a critical component required for neonatal autoimmune disease induction by maternal autoantibody.
- Author
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Rival, Claudia, Setiady, Yulius, Samy, Eileen T., Harakal, Jessica, and Tung, Kenneth S. K.
- Subjects
MATERNALLY acquired immunity ,IMMUNE complexes ,OVARIAN diseases ,T cells ,CONGENITAL heart disease ,KILLER cells - Abstract
Human maternal autoantibodies can trigger autoimmune diseases such as congenital heart block (CHB) in the progeny of women with lupus or Sjogren's disease. The pathogenic effect of early autoantibody (autoAb) exposure has been investigated in a murine neonatal autoimmune ovarian disease (nAOD) model triggered by a unique ZP3 antibody. Although immune complexes (IC) are formed in adult and neonatal ovaries, ZP3 antibody triggers severe nAOD only in <7-day-old neonatal mice. Propensity to nAOD is due to the uniquely hyper-responsive neonatal natural killer (NK) cells that lack the inhibitory Ly49C/I receptors. In nAOD, the neonatal NK cells directly mediate ovarian inflammation and oocyte depletion while simultaneously promoting de novo pathogenic ovarian-specific T cell responses. Resistance to nAOD in older mice results from the emergence of the Ly49C/I
+ NK cells that regulate effector NK cells and from CD25+ regulatoryT cell control. In preliminary studies, FcgRIIIC NK cells as well as the ovarian resident FcγRIII+ macrophages and/or dendritic cells were found to be as indispensable players. Activated by ovarian IC, they migrate to lymphoid organs where NK cell priming occurs. Remarkably, the findings in nAOD are very similar to those reported for neonatal responses to a retrovirus and its cognate antibody that lead to long-lasting immunity. Studies on nAOD therefore provide insights into maternal autoAb-mediated neonatal autoimmunity, including CHB, while simultaneously uncovering new properties of the neonatal innate and adaptive responses, lethality of premature infant infection, and novel neonatal antiviral vaccine design. [ABSTRACT FROM AUTHOR]- Published
- 2014
- Full Text
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239. Late development of complete atrioventricular block may be immune mediated and congenital in origin.
- Author
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Bergman, Gunnar, Skog, Amanda, Tingström, Joanna, Ottosson, Vijole, Hoxha, Ariela, Ambrosi, Aurelie, Salomonsson, Stina, and Wahren‐Herlenius, Marie
- Subjects
- *
MEDICAL care , *ATRIOVENTRICULAR node , *NEONATAL diseases , *SEROLOGY , *INFECTION - Abstract
Aim To investigate the correlation between maternal autoantibodies and age at diagnosis of isolated complete atrioventricular ( AV) block ( CAVB) and to study signs of late progression of foetal immune-mediated insults in cases of postnatally diagnosed CAVB. Methods Patients with CAVB (n = 190) identified in a population-based manner were included. Maternal autoantibody profile was correlated with age at CAVB diagnosis. A structured review of medical records was performed if a late CAVB diagnosis (>27 days post-partum) was associated with a sero-positive mother. Results Maternal Ro/La autoantibodies were observed in 88% of cases with a congenital diagnosis. Thirteen cases with a sero-positive mother and late CAVB diagnosis were found (age-range: 4 months-43 years). In two cases, CAVB was diagnosed in conjunction with infections, one case had a family history of cardiomyopathy and two cases had nontypical clinical presentations, indicating alternative pathogenetic mechanisms. In the remaining eight cases, no likely factors inducing CAVB, other than maternal autoantibodies, could be identified. Conclusion Our observations support the hypothesis that late progression to CAVB can be the result of an immune-mediated pathogenetic mechanism during foetal life. An autoantibody-associated diagnosis after the neonatal period is therefore possible, and testing of maternal serology at the time of diagnosis is recommended. [ABSTRACT FROM AUTHOR]
- Published
- 2014
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240. 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
241. Comorbidity and long-term outcome in patients with congenital heart block and their siblings exposed to Ro/SSA autoantibodies in utero
- Author
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Reyhan Bilici Salman, Şeyma Yıldız, Neslihan Kayahan, Hazan Karadeniz, Aslıhan Avanoğlu Güler, and Hasan Satış
- Subjects
030203 arthritis & rheumatology ,0301 basic medicine ,Pediatrics ,medicine.medical_specialty ,education.field_of_study ,business.industry ,Immunology ,Population ,Autoantibody ,medicine.disease ,Comorbidity ,General Biochemistry, Genetics and Molecular Biology ,Congenital heart block ,03 medical and health sciences ,030104 developmental biology ,0302 clinical medicine ,Rheumatology ,In utero ,Immunology and Allergy ,Medicine ,In patient ,business ,education - Abstract
We read with great interest the article ‘Comorbidity and Long-Term Outcome in Patients with Congenital Heart Block and Their Siblings Exposed to Ro/SSA Autoantibodies In Utero’ by Mofors et al .1 A large and long-term follow-up population consisting of patients with congenital heart block (CHB) as a result of in utero Ro/SSA autoantibody exposure were studied. It contributes valuable information to our knowledge; however, …
- Published
- 2020
242. A Broader Perspective on Anti-Ro Antibodies and Their Fetal Consequences-A Case Report and Literature Review
- Author
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Ana-Maria Zagrean, Anca Marina Ciobanu, Mihaela Roxana Popescu, Andreea Dudu, Ciprian Jurcut, and Anca Maria Panaitescu
- Subjects
030203 arthritis & rheumatology ,Risk awareness ,autoimmune channelopathy ,Fetus ,medicine.medical_specialty ,lcsh:R5-920 ,business.industry ,Mortality rate ,Clinical Biochemistry ,Perspective (graphical) ,fetal complete heart block ,apoptosis ,Review ,030204 cardiovascular system & hematology ,Congenital heart block ,03 medical and health sciences ,0302 clinical medicine ,anti Ro/SSA antibodies ,Medicine ,Clinical case ,business ,Intensive care medicine ,lcsh:Medicine (General) ,long-term consequences ,Anti-SSA/Ro autoantibodies - Abstract
The presence of maternal Anti-Ro/Anti-La antibodies causes a passively acquired autoimmunity that may be associated with serious fetal complications. The classic example is the autoimmune-mediated congenital heart block (CHB) which is due in most cases to the transplacental passage of Anti-Ro/Anti-La antibodies. The exact mechanisms through which these pathologic events arise are linked to disturbances in calcium channels function, impairment of calcium homeostasis and ultimately apoptosis, inflammation and fibrosis. CHB still represents a challenging diagnosis and a source of debate regarding the best management. As the third-degree block is usually irreversible, the best strategy is risk awareness and prevention. Although CHB is a rare occurrence, it affects one in 20,000 live births, with a high overall mortality rate (up to 20%, with 70% of in utero deaths). There is also concern over the lifelong consequences, as most babies need a pacemaker. This review aims to offer, apart from the data needed for a better understanding of the issue at hand, a broader perspective of the specialists directly involved in managing this pathology: the rheumatologist, the maternal–fetal specialist and the cardiologist. To better illustrate the theoretical facts presented, we also include a representative clinical case.
- Published
- 2020
243. Surveillance of congenital heart block in highly specialised care – Authors' reply
- Author
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Nathalie Morel, N. Costedoat-Chalumeau, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), and Conservatoire National des Arts et Métiers [CNAM] (CNAM)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE)
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,business.industry ,Immunology ,030204 cardiovascular system & hematology ,Congenital heart block ,3. Good health ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,[SDV.MHEP.RSOA]Life Sciences [q-bio]/Human health and pathology/Rhumatology and musculoskeletal system ,medicine ,Immunology and Allergy ,Intensive care medicine ,business ,ComputingMilieux_MISCELLANEOUS - Abstract
International audience
- Published
- 2020
244. Report of Congenital heart block and Atrial Septal Aneurysm in a case of Neonatal Lupus
- Author
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Mohinish S
- Subjects
medicine.medical_specialty ,Atrial septal aneurysm ,business.industry ,Neonatal lupus ,Internal medicine ,Cardiology ,Medicine ,General Medicine ,business ,Congenital heart block - Published
- 2020
245. 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
246. Development and application of an automated extraction algorithm for fetal magnetocardiography - normal data and arrhythmia detection.
- Author
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Stingl, Krunoslav, Paulsen, Henrike, Weiss, Magdalene, Preissl, Hubert, Abele, Harald, Goelz, Rangmar, and Wacker-Gussmann, Annette
- Subjects
- *
ARRHYTHMIA diagnosis , *MAGNETICS , *PRENATAL diagnosis , *REGRESSION analysis , *DATA analysis software , *DESCRIPTIVE statistics - Abstract
Introduction: Current standard methods of monitoring fetal heart function are mainly based on echocardiography, which provides indirect information (through mechanical assessment) of the fetal heart rhythm. Fetal magnetocardiography (fMCG) allows a reliable quantification of the temporal structure of fetal heart signals. However, its application in clinical studies is difficult because extracting the fetal heart signal for most current applications requires user intervention. To overcome this limitation, we developed a completely automated extraction algorithm. Patients and methods: The fMCG recordings were acquired using a 156-channel biomagnetic system. To perform an automated analysis, a combination of orthogonal projection and independent component analysis was used. fMCG recordings from 69 healthy uncomplicated singleton pregnancies with normally developing fetuses were included in the study. Results: The normal values achieved by the automated algorithm were comparable to previously published data. The majority of the cardiac time intervals were positively correlated with gestational age (GA). The ST segment, T wave and QT interval did not show any correlation. Conclusions: The automated detection of fetal heart signals was possible beginning at a GA of 19 weeks. This automated analysis of fMCG recordings might be an objective and easily applicable approach for clinicians to analyze fetal heart signals. [ABSTRACT FROM AUTHOR]
- Published
- 2013
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247. Congenital heart block in neonatal lupus erythematosus
- Author
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Marietta Branson and Richard L. Pullen
- Subjects
030203 arthritis & rheumatology ,medicine.medical_specialty ,business.industry ,030204 cardiovascular system & hematology ,Assessment and Diagnosis ,LPN and LVN ,medicine.disease ,Congenital heart block ,Nurse Assisting ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Cardiology ,Medicine ,Fundamentals and skills ,Neonatal lupus erythematosus ,business - Published
- 2018
248. Bradycardia in a Neonate
- Author
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Farhad Salehzadeh and Ross E. Petty
- Subjects
Bradycardia ,Pediatrics ,medicine.medical_specialty ,business.industry ,medicine.disease ,Asymptomatic ,Congenital heart block ,medicine ,medicine.symptom ,Neonatal lupus erythematosus ,Permanent pacemaker ,skin and connective tissue diseases ,Complication ,business - Abstract
The most important complication of neonatal lupus erythematosus (NLE) is congenital heart block (CHB) Infants with NLE are born to mothers who have antibodies against Ro (SSA) and/or La (SSB) antigens, although the mother may be completely asymptomatic Most children with CHB will eventually require a permanent pacemaker during childhood The risk of having a second baby with congenital heart block is within the range of 15–20%
- Published
- 2019
249. Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center
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Zhen Bing, Zipu Li, Degong Shi, Sujuan Hu, and Benzhen Wang
- Subjects
medicine.medical_specialty ,Cardiomyopathy ,Torsades de pointes ,030204 cardiovascular system & hematology ,QT interval ,Pediatrics ,03 medical and health sciences ,0302 clinical medicine ,Prednisone ,030225 pediatrics ,Internal medicine ,Junctional ectopic tachycardia ,medicine ,cardiovascular diseases ,Original Research ,congenital heart block ,Left bundle branch block ,business.industry ,steroid ,lcsh:RJ1-570 ,Endocardial fibroelastosis ,Dilated cardiomyopathy ,lcsh:Pediatrics ,neonatal lupus ,medicine.disease ,Pediatrics, Perinatology and Child Health ,Cardiology ,cardiovascular system ,business ,cardiomyopathy ,autoantibody ,medicine.drug - Abstract
Objective: To describe the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy, and to explore the therapeutic role of glucocorticoids in these diseases. Methods: This was a retrospective observational study of 2 fetuses and 14 children who presented with autoantibody-mediated arrhythmia and/or cardiomyopathy in our hospital from September 2010 to December 2018. Results: In total, 16 patients were identified, including 2 fetuses, and 14 children. One mother suffered from Sjogren's syndrome, two suffered from systemic lupus erythematosus (SLE), and the remaining 13 were asymptomatic carriers of autoantibodies. Two fetuses were diagnosed with complete congenital heart block (CHB) and had mean heart rates of 45 and 50 bpm. In the 14 surviving children, third-degree CHB was detected in 4 children, second- to third-degree CHB in 4, corrected QT interval (QTc) prolongation in 1, atrioventricular dissociation, and junctional ectopic tachycardia in 1, complete left bundle branch block (CLBBB) with dilated cardiomyopathy (DCM) in 3, and endocardial fibroelastosis (EFE) in 1. All of the 14 surviving babies received intravenous immunoglobulin and glucocorticoids. None of the children received pacemaker implantation. During the follow-up, one 3-month-old girl who had complete CHB, DCM, and Torsades de pointes almost recovered after the administration of prednisone for ~8 years. Three cases with complete CHB had no improvement after 3-5 years of follow-up. One case with EFE and three cases with CLBBB and DCM were in stable condition now. Children with QTc prolongation and junctional ectopic tachycardia returned to a regular rhythm. Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are severe complications related to maternal autoantibodies, and the administration of steroid may be beneficial in reversing complete CHB.
- Published
- 2019
250. VP23.17: Perinatal management of congenital heart block
- Author
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A. Mohd Jamil, Z. Yahaya, N. Abd Malik, V.R.L. Velayudham, M. Itam, and P. Yubbu
- Subjects
medicine.medical_specialty ,Reproductive Medicine ,Radiological and Ultrasound Technology ,business.industry ,Internal medicine ,medicine ,Cardiology ,Obstetrics and Gynecology ,Radiology, Nuclear Medicine and imaging ,General Medicine ,business ,Congenital heart block - Published
- 2021
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