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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 (

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.

An infant with congenital heart block and hemodynamically significant bradycardia underwent therapeutic temporary pacing wires placement. Post-operatively, frequent "asystole" alarms were observed on telemetry causing distress to both the family and the nursing staff. Investigation of these alarms showed that pacemaker malfunction led to monitor pseudo-malfunction. The alarms were alleviated with mindful setting of the pacemaker and telemetry monitor parameters. This case highlights the challenges of pacemaker placement and monitoring of very small infants in the intensive care setting. Awareness of these challenges would help in troubleshooting pacemaker and telemetry monitor issues.

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.

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.

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 )

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.

Our group presents our reflections, based on the current literature, concerning the obstetric and rheumatologic management of the foetus identified with clinical myocardial disease mediated by maternal autoantibodies (MAb). The European League Against Rheumatism (EULAR) have recommended the use of hydroxychloroquine (HCQ) during asymptomatic lupus pregnancies since 2007. Foetal echocardiography is indicated if there is suspected foetal dysrhythmia or myocarditis, especially in the involvement of positive maternal anti-Ro/SSA or anti-La/SSB antibodies weekly from 16 weeks of gestation upwards. The obstetric management should be guided by the degree of cardiac failure on foetal echocardiography. Foetal therapy with steroids, intravenous immunoglobulin (IVIG), and plasmapheresis should be reasonably introduced in the lowest effective doses for the shortest duration of time. The aim of the management should be to reverse incomplete heart block and other MAb-mediated foetal myocardial disease, presumably induced due to ongoing inflammation. In irreversible cases the treatment should be stopped, due to its possible maternal side effects. Delivery of the affected newborn should be performed in a tertiary centre, and pacemaker implantation might be an option for neonates.

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.

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.

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.

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.

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.

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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, …

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.

International audience

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.

Background: Pacemaker implantation is recommended in symptomatic patients with congenital complete heart block (CHB) with either an epicardial (epi) or transvenous (TV) approach depending on patient age / size. However, there is a paucity of data on updated left ventricle (LV) functional indices between the two approaches. Objective: Our aim was to compare LV function using speckle strain imaging in patients with LV Epi vs. best site right (R) TV pacing Methods: This was a single center, retrospective study of patients with isolated CHB …

Congenital heart block (CHB) is a cardiac disease with an overall prevalence of approximately1:20000 live births. Structural heart defects, congenital malformations of the cardiac conduction system, maternal connective tissue disorders and fetal myocarditis are the main entities that can cause CHBs. Here we report three cases of congenital heart blocks due to three different pathologies and their outcomes. In the first case, we could not detect a structural pathology for congenital heart block; however, in second and third cases there were significant structural cardiac defects that could contribute to the fetal bradycardia. Prenatal management of fetal arrhythmia may improve the outcome of an affected fetus or neonate. Precise and timely prenatal diagnosis is critical for the selection of the appropriate prenatal treatments. However, there are many limitations in managing CHB even today and more useful strategies need to be investigated.

Cardiac involvement is the most severe manifestation of neonatal lupus. It is usually diagnosed as an advanced (second or third degree) atrio-ventricular (AV) congenital heart block (CHB) starting around 20 weeks of gestation. At this gestational time, a window of opportunity might exist, and fluorinated corticosteroids (FS) have been attempted as a therapeutic option. Unfortunately the quality of the studies is methodologically low, due to the retrospective design and to the rarity of autoimmune CHB. Some case reports and small case series basically suggested a possible utility of FS, mainly in the context of incomplete CHB, while larger observational retrospective studies did not find any efficacy of FS either on mortality, on the rate of pacemaker implantation, to reduce the degree of incomplete CHB or to reduce the development of cardiomyopathy. Of note the distinction between complete and incomplete AV block may be very difficult and time consuming in utero. Overall FS should not be recommended in CHB, but might be considered for a short time for a recent incomplete CHB.

Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti-Ro-mediated second-degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti-arrhythmic drugs. The 2150-g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months. The newborn has had a good outcome with well-controlled heart rate.

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

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

not availableBangladesh J Child Health 2016; VOL 40 (2) :124-128

Background: Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). Objective: This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Methods: Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. Results: All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Conclusion: Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.

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.

Treatment options in patients with complete congenital heart block (atrioventricular block of III degree) may be controversial. Major obstacles are faced in pregnant women with newly detected heart block. Type of arrhythmia, presence of hemodynamic instability or syncope, anatomy of heart chambers and vessels, gestational age significantly influence the treatment. This article presents substantial information on etiology, pathogenesis, diagnostics, and possible treatment options in pregnant women with complete congenital heart block.

We report a term female infant with congenital heart block and total anomalous of pulmonary venous return. The results of single nucleotide polymorphism oligonucleotide microarray analysis showed an interstitial duplication of approximately 818 Kb, which involved 11 genes, including the entire LAMB3 gene which is known to associate with cardiac conduction defect. Our report adds to the collective knowledge that the cardiac conduction defect is a clinical feature of chromosome 1q32.2 duplication.

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%

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.

Aims Investigating human heart development and applying this to deviations resulting in disease is incomplete without molecular characterization of the cell types required for normal functioning. We investigated foetal human heart single-cell transcriptomes from mid-gestational healthy and anti-SSA/Ro associated congenital heart block (CHB) samples. Methods and results Three healthy foetal human hearts (19th to 22nd week of gestation) and one foetal heart affected by autoimmune-associated CHB (21st week of gestation) were subjected to enzymatic dissociation using the Langendorff preparation to obtain single-cell suspensions followed by 10× Genomics- and Illumina-based single-cell RNA-sequencing (scRNA-seq). In addition to the myocytes, fibroblasts, immune cells, and other minor cell types, previously uncharacterized diverse sub-populations of endothelial cells were identified in the human heart. Differential gene expression analysis revealed increased and heterogeneous interferon responses in varied cell types of the CHB heart compared with the healthy controls. In addition, we also identified matrisome transcripts enriched in CHB stromal cells that potentially contribute to extracellular matrix deposition and subsequent fibrosis. Conclusion These data provide an information-rich resource to further our understanding of human heart development, which, as illustrated by comparison to a heart exposed to a maternal autoimmune environment, can be leveraged to provide insight into the pathogenesis of disease., Graphical Abstract Graphical Abstract

We thank Satis and colleagues for opening the discussion on a potential influence of maternal immunomodulatory treatment on the long-term outcome of congenital heart block (CHB)1 and welcome their questions to help clarify some aspects of our study.2 Whether immunomodulatory treatment administered to the mother—as part of her own rheumatic disease treatment or intended to ameliorate the fetal cardiac inflammation in CHB—influences any outcome in CHB is a current matter of debate. Satis and colleagues cite several studies supporting a role …

Objective: To evaluate outcomes of fluorinated corticosteroids, with or without other medications, for treatment of congenital heart block in-utero. Study design: A search was conducted through MEDLINE, EMBASE, WEB OF SCIENCE and SCOPUS from inception to October 2017. Only comparative studies are considered eligible. Outcomes include fetal death, downgrade of heart block, neonatal death, need for neonatal pacing, fetal and maternal complications. Random effects model was used. Results: Out of 923 articles, 12 studies were eligible. Compared to no treatment, there was no significant difference in incidence of fetal death (OR 1.10, 95%CI 0.65–1.84), neonatal death (OR 0.98, 95%CI 0.41–2.33), or need for pacing (OR 1.46, 95%CI 0.78–2.74). Heart block downgrade was significantly higher in treatment group (9.48%vs.1.76%, OR 3.27, 95%CI 1.23–8.71). Conclusion: antenatal fluorinated corticosteroids do not improve fetal/neonatal morbidity or mortality of congenital heart block and are associated with higher incidence of fetal and maternal complications. Keywords: Congenital heart disease, Fetal diagnosis, Fetal therapy, Prenatal treatment