Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center
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 autoa...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2019-11-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fped.2019.00465/full |
| _version_ | 1811295255449829376 |
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| author | Benzhen Wang Benzhen Wang Sujuan Hu Degong Shi Zhen Bing Zipu Li Zipu Li |
| author_facet | Benzhen Wang Benzhen Wang Sujuan Hu Degong Shi Zhen Bing Zipu Li Zipu Li |
| author_sort | Benzhen Wang |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T05:30:40Z |
| format | Article |
| id | doaj.art-dc39f6d183c84af5b031ff7f61afa474 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-04-13T05:30:40Z |
| publishDate | 2019-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-dc39f6d183c84af5b031ff7f61afa4742022-12-22T03:00:28ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602019-11-01710.3389/fped.2019.00465477456Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single CenterBenzhen Wang0Benzhen Wang1Sujuan Hu2Degong Shi3Zhen Bing4Zipu Li5Zipu Li6School of Medicine, Shandong University, Jinan, ChinaHeart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, ChinaDepartment of Pediatrics, The Affiliated Hospital of Qingdao University, Qingdao, ChinaDepartment of Pediatrics, The Traditional Chinese Medical Hospital of Huangdao District, Qingdao, ChinaHeart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, ChinaSchool of Medicine, Shandong University, Jinan, ChinaHeart Center, Qingdao Women and Children's Hospital, Affiliated to Qingdao University, Qingdao, ChinaObjective: 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.https://www.frontiersin.org/article/10.3389/fped.2019.00465/fullautoantibodycongenital heart blockcardiomyopathysteroidneonatal lupus |
| spellingShingle | Benzhen Wang Benzhen Wang Sujuan Hu Degong Shi Zhen Bing Zipu Li Zipu Li Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center Frontiers in Pediatrics autoantibody congenital heart block cardiomyopathy steroid neonatal lupus |
| title | Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center |
| title_full | Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center |
| title_fullStr | Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center |
| title_full_unstemmed | Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center |
| title_short | Arrhythmia and/or Cardiomyopathy Related to Maternal Autoantibodies: Descriptive Analysis of a Series of 16 Cases From a Single Center |
| title_sort | arrhythmia and or cardiomyopathy related to maternal autoantibodies descriptive analysis of a series of 16 cases from a single center |
| topic | autoantibody congenital heart block cardiomyopathy steroid neonatal lupus |
| url | https://www.frontiersin.org/article/10.3389/fped.2019.00465/full |
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