This therapeutic effect reflects the immune-mediated nature of the disease and shows that steroids could actually have the capability to reverse complete heart block. glucocorticoids and immunoglobulin. None of them from the small children received pacemaker implantation. Through the follow-up, one 3-month-old young lady who LIN28 inhibitor LI71 got full CHB, DCM, and Torsades de pointes nearly recovered following the administration of prednisone for ~8 years. Three instances with full CHB got no improvement after 3C5 many years of follow-up. One case with EFE and 3 instances with DCM and CLBBB were in steady condition now. Kids with QTc prolongation and junctional ectopic tachycardia came back to a normal tempo. Conclusions: Autoantibody-mediated arrhythmias and/or cardiomyopathy are serious complications linked to maternal autoantibodies, as well as the administration of steroid could be helpful in reversing complete CHB. or during the first year of life (5). Heart involvement in neonatal lupus is often accompanied by the presence of maternal autoantibodies in the fetal and neonatal circulation (6). The signature cardiac lesion is an atrioventricular block seen as a CHB, but 15C20% of these cases have associated fatal cardiomyopathy (7, 8). Autoimmune congenital heart block (ACHB), a rare condition that occurs in ~1 out of every 20,000 pregnancies (9), is associated with the transplacental passage of maternal autoantibodies such as anti-Ro/SSA and/or anti-La/SSB antibodies in more than 80% of affected neonates (3). ACHB might be detected as a first- or second-degree atrioventricular block (AVB), but the majority have a potentially lethal complete AVB (CAVB) (10). Atrioventricular block most commonly develops during the 18C24 weeks of gestation, and may be found using fetal Doppler echocardiography (11). The disease can continue to develop after birth, even during infancy and early childhood (12). At the same time, ~20% of affected fetuses can develop more diffuse myocardial disease manifested as cardiomyopathy and usually associated with endocardial fibroelastosis (EFE) (1, 2, 13); unfortunately, information relating to these conditions is sparse (2, 13, 14). The majority of studies suggest that autoantibodies damage fetal conduction tissues leading to inflammation, calcification, and fibrosis, which can block signal conduction at the atrioventricular node without the requirement for additional structural abnormalities (2, 3). Indeed, increasing experimental, and clinical evidence has shown how these autoantibodies can critically interfere with cardiac electrical function and promote the development of life-threatening arrhythmic events by affecting the function of cardiac ion channels (7, 15). The current curative CHB treatment is very controversial, and various therapeutic approaches including corticosteroids (especially fluorinated steroids, such as betamethasone or dexamethasone), intravenous immunoglobulin (IVIG), plasmapheresis, and beta-adrenergic agents have been reported (3, 5, 16). However, several studies have cast doubt on the efficacy of these therapies, and ultimately, a pacemaker is required in ~80% of newborns with congenital third-degree AVB (17C19). This study aims to present our single-center experience describing the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy. In addition, we describe one interesting case where the mother had systemic lupus erythematosus (SLE), and the obstetric history CDC42EP1 demonstrated fetal bradycardia, complete AVB, Torsades de pointes (Tdp), and DCM in the third month after birth, but had almost returned to normal following the long-term administration of corticosteroids. Materials and Methods Patient Identification Clinical data on 16 cases were collected by performing a retrospective observational study of the patients at the Heart Center of Qingdao Women and Children’s Hospital from September 2010 to December 2018. This study was approved by the ethics committee of Qingdao Women and Children’s Hospital, and written informed consent was obtained from the parents of the study participants. Inclusion criteria were as follows: The presence of maternal autoantibodies, such as anti-Ro/SSA and/or anti-La/SSB antibodies; and the confirmation of arrhythmia [including second- and third-degree heart block, prolonged QT interval, and complete left bundle branch block.In addition, EFE also occupies a certain proportion of the cardiac manifestation of neonatal lupus (9, 20) and isolated EFE associated with maternal antibodies can be present in the absence of CAVB (13). 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 3C5 years of follow-up. One case with EFE and three cases with LIN28 inhibitor LI71 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. or during the first year of life (5). Heart involvement in neonatal lupus is often accompanied by the presence of maternal autoantibodies in the fetal and neonatal circulation (6). The signature cardiac lesion is an atrioventricular block seen as a CHB, but 15C20% of these cases have associated fatal cardiomyopathy (7, 8). Autoimmune congenital heart block (ACHB), a rare condition that occurs in ~1 out of every 20,000 pregnancies (9), is associated with the transplacental passage of maternal autoantibodies such as anti-Ro/SSA and/or anti-La/SSB antibodies in more than 80% of affected neonates (3). ACHB might be detected as a first- or second-degree atrioventricular block (AVB), but the majority have a potentially lethal complete AVB (CAVB) (10). Atrioventricular block most commonly develops during the 18C24 weeks of gestation, and may be found using fetal Doppler echocardiography (11). The disease can continue to develop after birth, even during infancy and early childhood (12). At the same time, ~20% of affected fetuses can develop more diffuse myocardial disease manifested as cardiomyopathy and usually associated with endocardial fibroelastosis (EFE) (1, 2, 13); unfortunately, information relating to these conditions is sparse (2, 13, 14). The majority of studies suggest that autoantibodies damage fetal conduction tissues leading to inflammation, calcification, and fibrosis, which can block signal conduction at the atrioventricular node without the requirement for additional structural abnormalities (2, 3). Indeed, increasing experimental, and clinical evidence has shown how these autoantibodies can critically interfere with cardiac electrical function and promote the development of life-threatening arrhythmic events by affecting the function of cardiac ion channels (7, 15). The current curative CHB treatment is very controversial, and various therapeutic approaches including corticosteroids (especially fluorinated steroids, such as betamethasone or dexamethasone), intravenous immunoglobulin (IVIG), plasmapheresis, and beta-adrenergic agents have been reported (3, 5, 16). However, several studies have cast doubt on the efficacy of these therapies, and ultimately, a pacemaker is required in ~80% of newborns with congenital third-degree AVB (17C19). This study aims to present our single-center experience describing the clinical characteristics of maternal autoantibody-mediated arrhythmia and/or cardiomyopathy. In addition, LIN28 inhibitor LI71 we describe one interesting case where the mother had systemic lupus erythematosus (SLE), and the obstetric history demonstrated fetal bradycardia, complete AVB, Torsades de pointes (Tdp), and DCM in the third month after birth, but had almost returned to normal following the long-term administration of corticosteroids. Materials and Methods Patient Identification Clinical data on 16 cases were collected by performing a retrospective observational study of the patients at the Heart Center of Qingdao Women and Children’s Hospital from September 2010 to December 2018. This study was approved by the ethics committee of Qingdao Women and Children’s Hospital, and written informed consent was obtained from the parents of the study participants. Inclusion criteria were as follows: The presence of maternal autoantibodies, such as anti-Ro/SSA and/or anti-La/SSB antibodies; and the confirmation of arrhythmia [including second- and third-degree heart block, prolonged QT interval, and complete left bundle branch block (CLBBB)] and/or cardiomyopathy (autoantibody-mediated EFE or DCM) in the patients, as documented by fetal echocardiography, electrocardiogram, or.