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Fetal autoimmune atrioventricular block (AVB) is a rare but potentially lifethreatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described. [ABSTRACT FROM AUTHOR]

This article presents the case of a 27-year-old female patient with idiopathic congenital complete heart block who does not consent to the implantation of a cardiac pacemaker but was referred by her primary care physician for cardiological evaluation. The conduction disturbance was recognized at the age of 6 and was asymptomatic. The professional disqualification from pacemaker implantation included a detailed history of a patient's symptoms, an echocardiographic assessment of the heart, exercise testing and ECG Holter monitoring. The aid of salbutamol administered orally was also useful. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to explore the parents' experiences of home monitoring of the fetal heart rhythm. Women with anti-SSA/Ro52 autoantibodies carry a 2%–3% risk of giving birth to a child with congenital heart block (CHB), following transplacental transfer and antibody-mediated inflammation in the fetal conduction system during 18th to 24th gestational week. Early detection and subsequent treatment have been reported to decrease morbidity and mortality. Therefore, home monitoring of the fetal heart rhythm by Doppler has been offered at our fetal cardiology center. This study was undertaken to explore the lived experience of the routine. Methods: Participants were recruited from a single fetal cardiology center. Consecutive sampling was used. The inclusion criteria were women with SSA/Ro52 antibodies who had undergone Doppler examinations within the last two and a half years at the hospital and had monitored the fetal heartbeat at home. A semi-structured questionnaire was created, and the participants were interviewed individually. The interviews were transcribed verbatim and analyzed according to qualitative content analysis. Results: The overall theme was defined as "walking on thin ice," with six underlying categories: reality, different strategies, gain and loss, healthcare providers, underlying tension, and conducting the examinations again, all with a focus on how to handle the home monitoring during the risk period. Conclusion: Both the mother and the co-parent expressed confidence in their own abilities and that the monitoring provided them with the advantage of growing a bond with the expected child. However, all the participants described a feeling of underlying tension during the risk period. The results show that home monitoring is not experienced as complicated or a burden for the parents-to-be and should be considered a vital part of the chain of care for mothers at risk for giving birth to a child with CHB. However, explaining the teamwork between the different caregivers, for the patients involved, their areas of expertise, and how they collaborate with the patient continues to be a pedagogic challenge and should be developed further. [ABSTRACT FROM AUTHOR]

This study, conducted by searching keywords such as "maternal lupus", "neonatal lupus", and "congenital heart block" in databases including PubMed and Scopus, provides a detailed narrative review on fetal and neonatal lupus. Autoantibodies like anti-Ro/SSA and anti-La/SSB may cross the placenta and cause complications in neonates, such as congenital heart block (CHB). Management options involve hydroxychloroquine, which is able to counteract some of the adverse events, although the drug needs to be used carefully because of its impact on the QTc interval. Advanced pacing strategies for neonates with CHB, especially in severe forms like hydrops, are also assessed. This review emphasizes the need for interdisciplinary care by rheumatologists, obstetricians, and pediatricians in order to achieve the best maternal and neonatal health in lupus pregnancies. This multidisciplinary approach seeks to improve the outcomes and management of the disease, decreasing the burden on mothers and their infants. [ABSTRACT FROM AUTHOR]

Fetal autoimmune atrioventricular block (AVB) is a rare but potentially life-threatening condition. It results from the passage of maternal anti-SSA/Ro or Anti SSB/La auto-antibodies into the fetal circulation, leading to inflammation and fibrosis of the AV node and often to irreversible damage. Besides AVB, these antibodies can also cause cardiomyopathies, but there is no evidence linking them to tachyarrhythmias. We present the case of a patient with significant risk factors for fetal AVB: a prior history of hydrops fetalis, high anti-SSA/Ro antibody levels and hypothyroidism. In this case, the use of dexamethasone and intravenous immunoglobulin may have contributed to reversing the first-degree atrioventricular block detected at 19 weeks of gestation. Additionally, at 21 weeks, the fetus developed a tachyarrhythmia that needed treatment with flecainide. Soon after the birth, the newborn underwent ECG Holter and Wolff-Parkinson-White Syndrome (WPWS) was diagnosed. To our knowledge, the coexistence of fetal AVB and WPWS has never been described.

Abstract Background It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. Case presentation A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring. Physical examination revealed a bluish-purple discoloration around the lips and an irregular heartbeat. EGG showed the presence of isolated P (142 bpm) and QRS (78 bpm) waves, ventricular escape beats, and a diagnosis of NL-CHB. To reverse the condition, transcutaneous pacing and five sessions of plasmapheresis were performed. At a 1.5-year follow-up, the baby exhibited well-developed cardiac structure and normal neurodevelopment. Conclusions Transcutaneous pacing and repeated plasmapheresis might be possible to reverse CHB in NL.

Background: It has been reported that the complete heart block (CHB) in neonatal lupus (NL) cannot be reversed. This study reported a case of NL-CHB that was reversed by transcutaneous pacing and repeated plasmapheresis. Case presentation: A 35+ 6-week male preterm baby was transferred to the neonatal intensive care unit of the Army Medical Center in May 2020 for slight cyanosis around the lips and nose. Two days after birth, a sudden decrease in heart rate was observed during electrocardiogram (EGG) monitoring. Physical examination revealed a bluish-purple discoloration around the lips and an irregular heartbeat. EGG showed the presence of isolated P (142 bpm) and QRS (78 bpm) waves, ventricular escape beats, and a diagnosis of NL-CHB. To reverse the condition, transcutaneous pacing and five sessions of plasmapheresis were performed. At a 1.5-year follow-up, the baby exhibited well-developed cardiac structure and normal neurodevelopment. Conclusions: Transcutaneous pacing and repeated plasmapheresis might be possible to reverse CHB in NL. [ABSTRACT FROM AUTHOR]

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This article presents the case of a 27-year-old female patient with idiopathic congenital complete heart block who does not consent to the implantation of a cardiac pacemaker but was referred by her primary care physician for cardiological evaluation. The conduction disturbance was recognized at the age of 6 and was asymptomatic. The professional disqualification from pacemaker implantation included a detailed history of a patient’s symptoms, an echocardiographic assessment of the heart, exercise testing and ECG Holter monitoring. The aid of salbutamol administered orally was also useful.

This study, conducted by searching keywords such as “maternal lupus”, “neonatal lupus”, and “congenital heart block” in databases including PubMed and Scopus, provides a detailed narrative review on fetal and neonatal lupus. Autoantibodies like anti-Ro/SSA and anti-La/SSB may cross the placenta and cause complications in neonates, such as congenital heart block (CHB). Management options involve hydroxychloroquine, which is able to counteract some of the adverse events, although the drug needs to be used carefully because of its impact on the QTc interval. Advanced pacing strategies for neonates with CHB, especially in severe forms like hydrops, are also assessed. This review emphasizes the need for interdisciplinary care by rheumatologists, obstetricians, and pediatricians in order to achieve the best maternal and neonatal health in lupus pregnancies. This multidisciplinary approach seeks to improve the outcomes and management of the disease, decreasing the burden on mothers and their infants.

• A common cause of congenital heart block is autoimmune in mothers with lupus. • Symptoms range in severity going from asymptomatic to syncope and cyanosis. • Congenital heart block may occur in a structurally normal heart. • Research focused on preventing fetal heart effects in pregnant women with lupus. Congenital heart block is a rare disease characterized by electrical conduction abnormalities of the heart in patients with both structural and without structural abnormalities. The most common cause of congenital heart block is autoimmune related, but other potential causes exist. Treatment for congenital heart block is determined by the severity of the disease and presenting symptoms. Primary care providers are in the optimal position to support patients and families. Although common causes and treatments of congenital heart block are known, much research is still to be done on the cause, optimal treatments, and potential long-term side effects. [ABSTRACT FROM AUTHOR]

An 8-year-old girl with a history of single-chamber epicardial pacemaker during infancy and cardiac resynchronization therapy with a His bundle pacing lead implantation six months earlier, presented with congenital complete heart block. At the follow-up visit, we found atrial pacing lead protrusion with probable insulation in the computed tomography scan. We have shown late pacemaker lead perforation management under fluoroscopic guidance in a pediatric patient. A serious complication associated with cardiac implantable electronic devices is lead perforation. In the pediatric age group, limited data exist on this complication and its challenging management. We present a case of atrial pacing lead protrusion in an 8-year-old girl. The lead was extracted under fluoroscopic guidance without any complications. [ABSTRACT FROM AUTHOR]

Background: Chronic right ventricular (RV) apical pacing in patients with congenital complete atrioventricular block (CCAVB) is associated with left ventricle (LV) dyssynchrony and dysfunction. Hence, alternative pacing sites are advocated. The aim of this study was to compare LV function using STE in selected patients with LV epicardial pacing (LVEp) vs. RV transvenous pacing (RVSp). Methods: This was a single-center, retrospective study in patients with CCAVB who underwent permanent pacemaker implant at age ≤ 18 years. Age- and gender-matched patients with a normal heart anatomy and function served as the control group. LV function was comprehensively assessed by conventional 2D Echocardiography and speckle-tracking echocardiography (STE). Results: We included 24 patients in the pacemaker group [27.6% male, mean age of 17.1 at last follow-up, follow-up duration of 8.7 years, RVSp (n = 9; 62.5%)] compared to 48 matched healthy controls. Shortening fraction (SF) and ejection fraction (EF) were normal and similar between cases and controls. However, STE detected abnormal LV function in the pacemaker group compared to controls. The former demonstrated lower/abnormal, Peak Longitudinal Strain myocardial (PLS Myo) [− 12.0 ± 3.3 vs. − 18.1 ± 1.9, p < 0.001] and Peak Longitudinal Strain endocardial (PLS endo) [− 16.1 ± 4.1 vs. 1.7 ± 1.7, p < 0.001]. STE parameters of LV function were significantly more abnormal in LVEp vs. RVSp subgroup as demonstrated by lower values for PLS Myo (− 10.1 ± 3.2 vs. − 13.1 ± 2.9, p = 0.03) and PLS Endo (− 13.8 ± 4.4 vs. − 17.5 ± 3.3, p = 0.03). Conclusion: STE was more sensitive in detecting subtle differences in LV function relative to standard conventional 2D echocardiography (SF and EF) in selected patients with CCAVB and a permanent pacemaker. Furthermore, STE demonstrated that transvenous RV septal pacing was associated with better LV systolic function preservation than LV epicardial pacing for comparable post-implant intervals. [ABSTRACT FROM AUTHOR]

About 2% of mothers with Sjögren’s syndrome and about 1% of mothers with systemic lupus erythematosus deliver a baby with a congenital heart block (CHB). This is thought to be as a result of the maternal autoantibodies that cross the placenta and cause congenital lupus in the fetus/neonate. Among patients with a 2nd or 3rd degree atrioventricular block, the mortality rate in the neonatal period is about 10%, and most neonates who survive require a pacemaker into adulthood. Despite the compelling mortality and morbidity, the data on the optimal preventive treatments are meager and not well-established. In addition to pharmaceutical therapy, one potentially effective therapy is plasmapheresis. Plasmapheresis is safe in pregnancy, well tolerated, and is effective in removing the offending substances in the serum which may cause disease. We review this literature, in order to educate the reader and to motivate interest in studying this condition in the future.

Neonatal lupus erythematosus (NLE) is a congenital autoimmune condition in which the transplacental passage of immunoglobulin G (IgG) directed against auto-antigens causes clinical symptoms in the foetus or neonate. Anti-Ro/SS-A, anti-La/SS-B, and to a lesser extent, anti-U1RNP autoantibodies (aAbs) have the strongest association with NLE. However, ~ 50% of affected mothers are asymptomatic despite carrying those aAbs. The clinical picture of the disease is very diverse. Cardiac manifestations are the most severe, including congenital heart block (CHB), with a mortality rate of ~18%. Preventative therapy with hydroxychloroquine (HCQ) reduces the recurrence rate of CHB in subsequent pregnancies by ~50%.

Cardiac electrical stimulation in children usually is needed in the setting of complete congenital atrioventricular block, atrioventricular block after heart surgery, and bradycardia associated with some specific channelopathies. In cases of atrioventricular block, the high percentage of ventricular stimulation raises concern on the deleterious effects of chronic stimulation of the right ventricle. In recent years, physiologic stimulation has developed as a valid approach for adult patients and a great interest has risen in offering conduction system pacing also to the pediatric population. We present three pediatric cases of stimulation of the conduction system (His bundle or left bundle branch), in order to show the intrinsic particularities and challenges implied in these new techniques.

Objective Maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies are a risk factor for congenital complete heart block (CHB). Because detailed analysis of the incidence of CHB after 24 weeks of gestational age (GA) is lacking, we aimed to ascertain the risk of “later-onset” CHB among offspring of SSA/SSB-positive mothers in the published literature. Study Design Using search terms “neonatal lupus heart block” and “autoimmune congenital heart block” on PubMed and Ovid, we gathered prospective studies of SSA/SSB-positive mothers with fetal echo surveillance starting from before CHB diagnosis and retrospective cases of fetal CHB diagnosis after 24 weeks of GA (if there was prior normal heart rate) or after birth. Results Ten prospective studies included 1,248 SSA/SSB-positive pregnancies with 24 cases of CHB diagnosed during pregnancy (1.9%). Among these, three (12.5%) were after 24 weeks—at weeks 25, 26, and 28. Our retrospective studies revealed 50 patients with CHB diagnosis in late fetal life and neonatal period and 34 in the nonneonatal childhood period. An additional four cases were diagnosed after age 18 years. Conclusion Later-onset autoimmune CHB in offspring of SSA/SSB-positive mothers does occur. Our analysis suggests that prenatal surveillance should continue beyond 24 weeks of GA but is limited by inconsistent published surveillance data.

Background: Complete atrioventricular block (CAVB) is a complication of maternal antibody positivity and treatment of fetal disease is controversial in terms of efficacy and safety. We hypothesized that dexamethasone treatment for fetal anti-Ro/SSA antibody-mediated cardiac disease leads to better pregnancy outcomes than expectant management. Methods: A retrospective multi-center cohort study of anti-Ro/SSA antibody positive pregnancies with fetal conduction disease reported by participating North American Fetal Therapy Network (NAFTNet) centers between January 2010 and December 2018. The primary outcomes included: fetal death, oligohydramnios, growth restriction, preterm delivery, and new maternal comorbidities. Secondary outcomes included: pacemaker prior to 28 days, transplantation, and neonatal death in maternal/fetal dyads treated with dexamethasone versus not. Results: In 127 anti-Ro/SSA positive pregnancies, 98 were treated with dexamethasone and 29 were not. Of those treated, 61/96 (63.5%) met the primary outcome including 45/91 (49.4%) premature deliveries; 20 mothers developed comorbidities during treatment (fetal death 5, 10 growth restriction, 14 oligohydramnios, two new/worsening gestational diabetes). In the untreated group, 15/25 (60%) met the primary outcome including 11/22 (50%) premature deliveries and four mothers developing comorbidities during their pregnancy (fetal death 3, one growth restriction, one new onset maternal hypertension). Regarding secondary outcomes, 37/ 96 (43%) treated fetuses required a pacemaker or died by 28 days, while untreated 13/25 (52%) required pacemaker placement, died prior to 28 days or required listing for transplantation. Excluding terminations, survival without transplant was 17 (68%) in untreated and 85 (89%) in treated patients (p<.01). Conclusions: While the use of dexamethasone in anti-Ro/SSA positive pregnancies is associated with a high rate of poor pregnancy outcomes, there was an unexpected similarly high rate in untreated positive pregnancies. This suggests that the maternal disease itself is influencing pregnancy complications independent of dexamethasone. Our data, which show that treatment decreases neonatal morbidity and overall mortality without increasing overall pregnancy complications, warrant further study. [ABSTRACT FROM AUTHOR]

Neonatal lupus erythematosus (NLE) is a congenital autoimmune condition in which the transplacental passage of immunoglobulin G (IgG) directed against auto-antigens causes clinical symptoms in the foetus or neonate. Anti-Ro/SS-A, anti-La/SS-B, and to a lesser extent, anti-U1RNP autoantibodies (aAbs) have the strongest association with NLE. However, ~ 50% of affected mothers are asymptomatic despite carrying those aAbs. The clinical picture of the disease is very diverse. Cardiac manifestations are the most severe, including congenital heart block (CHB), with a mortality rate of ~18%. Preventative therapy with hydroxychloroquine (HCQ) reduces the recurrence rate of CHB in subsequent pregnancies by ~50%. [ABSTRACT FROM AUTHOR]

Objective: Transplacental passage of maternal anti-SSA and anti-SSB antibodies, potentially associated with maternal autoimmune diseases, can cause neonatal lupus syndrome. Given the paucity of data in this setting, we report short- and long-term outcomes of mothers of offspring with congenital heart block (CHB).Methods: This retrospective study included anti-SSA/SSB antibody-positive mothers of fetuses with high-degree CHB and focused on their health status before pregnancy, at CHB diagnosis, and thereafter.Results: We analyzed 215 women with at least 1 pregnancy with CHB. Prior to this diagnosis, only 52 (24%) mothers had been diagnosed with an autoimmune disease, mainly systemic lupus erythematosus (SLE; n = 26, 12%) and Sjögren syndrome (SS; n = 16, 7%). Six more were diagnosed with an autoimmune disease during the index pregnancy. Of the 157 mothers (73%) with no such diagnosis at childbirth, 77 (49%) developed one after a median follow-up of 11 years (range: 21 days to 54 years). By the end of follow-up, 135 women (63%) had an autoimmune disease diagnosis, mainly SLE (n = 54, 25%) and SS (n = 72, 33%). Three patients with SLE had renal involvement, and only 6 (3%) had required an immunosuppressive drug at any point. The symptoms best predicting autoimmune disease development were arthralgia and myalgia (P < 0.001), dry syndrome (P = 0.01), and parotid swelling (P = 0.05).Conclusion: One-quarter of the patients had an autoimmune disease diagnosis at the time of the fetal CHB diagnosis. Nearly half of those without an initial diagnosis progressed during follow-up, most without severe manifestations. Severe diseases such as lupus nephritis were rarely seen, and immunosuppressive drugs were rarely required. [ABSTRACT FROM AUTHOR]

Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease caused by transplacental passage of maternal circulating anti-Ro/SSA and anti-La/SSB antibodies which can bind to fetal cardiac tissue, damaging conduction tissues by inflammation and fibrosis. Approximately 2% of pregnancies with positive anti-Ro antibodies will be complicated by fetal atrioventricular block and the risk of recurrence in subsequent pregnancies is 10 times higher. We report a case of a clinically asymptomatic patient diagnosed with anti-Ro antibodies who had two pregnancies complicated by CHB with different outcomes. Despite preventive treatment with hydroxychloroquine (HCQ) from 6 weeks of pregnancy onward, the fetus developed second to third degree CHB. Dexamethasone was added. The pregnancy evolved to near-term with persistent intermittent CHB. It is not clear how pregnancies with recurrent fetal CHB despite prophylaxis with HCQ should be managed and there is a need for controlled studies to answer the remaining questions in relation to this subject.

Anti-Ro antibodies are detected frequently in the general population, but more so in patients with autoimmune conditions as Sjögren’s syndrome and systemic lupus erythematous (SLE). During pregnancy, anti-Ro antibodies can cross the placenta by hijacking physiological mechanisms and can have deleterious effects on the fetus. Administration of hydroxychloroquine (HCQ) to pregnant women with documented anti-Ro antibodies has been shown to prevent congenital heart block (CHB ). Serial fetal ultrasound scans and echocardiograms are controversial in pregnant women with anti-Ro antibodies. When complete CHB is diagnosed, this is irreversible and can lead to fetal heart failure, hydrops, and death. After delivery, babies with complete CHB require pacemaker. In the presence of maternal anti-Ro antibodies, there is a high risk of recurrence of CHB for future pregnancies, if there is a previously affected child. Adequate counselling and prophylactic treatment with HCQ should be encouraged.

One of the most successful achievements of fetal intervention is the pharmacologic management of fetal arrhythmias. This management usually takes place during the second or third trimester. While most arrhythmias in the fetus are benign, both tachy‐ and bradyarrhythmias can lead to fetal hydrops or cardiac dysfunction and require treatment under certain conditions. This review will highlight precise diagnosis by fetal echocardiography and magnetocardiography, the 2 primary means of diagnosing fetuses with arrhythmia. Additionally, transient or hidden arrhythmias such as bundle branch block, QT prolongation, and torsades de pointes, which can lead to cardiomyopathy and sudden unexplained death in the fetus, may also need pharmacologic treatment. The review will address the types of drug therapies; current knowledge of drug usage, efficacy, and precautions; and the transition to neonatal treatments when indicated. Finally, we will highlight new assessments, including the role of the nurse in the care of fetal arrhythmias. The prognosis for the human fetus with arrhythmias continues to improve as we expand our ability to provide intensive care unit–like monitoring, to better understand drug treatments, to optimize subsequent pregnancy monitoring, to effectively predict timing for delivery, and to follow up these conditions into the neonatal period and into childhood. Coordinated initiatives that facilitate clinical fetal research are needed to address gaps in knowledge and to facilitate fetal drug and device development. [ABSTRACT FROM AUTHOR]

Background: Congenital heart defects are the most common of birth defect, which leads to neonatal death after birth. Early diagnosis during prenatal period would be a benefit for precaution and treatment. Antegrade Late Diastolic Arterial blood Flow (ALDAF) was reported to measure fetal atrioventricular (AV) time intervals (FAVTI) at an early gestational ages (GA) of 6 weeks. There has been no previous studies reporting reference value of fetal atrioventricular time intervals (FAVTI) derive from ALDAF technique. Methods: Using fetal echocardiogram, this cross-sectional study was performed on 528 healthy fetuses between 14 and 40 weeks. Pulsed wave Doppler-derived FAVTI (milliseconds) were measured from ALDAF-AO and ALDAF-PA and left ventricle (LV) In/Out. Correlations between these three Doppler measurement techniques were examined with the Bland-Altman analysis and Pearson correlation coefficient. GA was used as specific reference value and its correlation with FAVTI was examined with linear regression. Results: We establish reference values of fetal atrioventricular (AV) time intervals (FAVTI) from antegrade late diastolic arterial blood flow (ALDAF) aorta (AO) and pulmonary artery (PA) from 14 to 40 weeks of gestation (GA). A positive correlation between FAVTI and GA was identified when using each of the three measurements (ALDAF-AO/ALDAF-PA and LV In/Out) (R2 = 0.177–0.272; P < 0.001). GA had the strongest impact on ALDAF-AO FAVTI, which was estimated to have a predicted FAVTI of 1.02 × GA (weeks) + 87.82. Bland-Altman analysis showed FAVTI of ALDAF-AO and ALDAF-PA were also significantly correlated (R2 = 0.573, P < 0.001). Intra-observer and inter-observer reliability coefficients showed good reproducibility (ICC >0.90) for all methods. Conclusions: This is the first study to establish reference ranges for FAVTI obtained from ALDAF-AO/ALDAF-PA for each week of gestation from 14 to 40 weeks. Our findings inform clinical practice by establishing GA-specific ALDAF-AO/PA cut-off values for the diagnosis of congenital heart block. FAVTI from ALDAF-AO/ALDAF-PA is a more practical measurement to use in the clinical setting because it is easier to investigate than LV In/Out. Good reproducibility in FAVTI measurements and a lack of fetal heart rate influence underpin the strength of our findings.

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.

Epicardial pacing wire (EPW) insertion is an integral part of open heart surgery. However, the use of EPW insertion is also associated with complications such as bleeding, tamponade, arrhythmias, and occasionally even death of the patient. Various techniques have been described for preparing, placing, and removing EPW. We present our simple, yet effective technique of preparing, inserting, and removing EPW where the incidence of complications is nil.

Sjögren's (SHOW'-grenz) syndrome is a systemic autoimmune, rheumatic disease that affects the entire body. The most common problems are dry mouth, dry eyes, fatigue and musculoskeletal pain in adults. Ten times as many women as men are diagnosed with Sjögren's. While most often diagnosed in women during middle age or after menopause. The congenital heart block (CHB) is defined as the heart block that is diagnosed in fetus (in utero) or within the first 28 days after birth (neonatal period). Congenital heart block is a rare disorder that appears to affect males and females in equal numbers. The most common cause of congenital heart block (CHB) is neonatal lupus due to maternal Sjögren's syndrome, an acquired autoimmune disease caused by transplacental transfer of maternal antibodies to the fetus. Several studies have reported an increased rate of spontaneous abortion and fetal loss associated with Sjögren syndrome. Congenital heart block occurs in a frequency of 1 in 20,000 live births. It has been reported to occur in 2% of Ro-positive mothers [2]; 5% of mothers with a diagnosis of mixed connective tissue and/or Sjögren Syndrome [3] and in 8% of Ro-positive mothers. Here we present an early pre-term neonate that was admitted to neonatal intensive care unit for bradycardia with stable haemodynamics. The mother, who showed no clinical symptoms or any particular history, was transferred to our tertiary centre for profound fetal bradycardia on recent scans. At birth, the infant's ECG showed a third-degree atrioventricular block and echocardiography was normal. Cardiac neonatal lupus was confirmed with positive maternal anti-Ro antibodies. Under close monitoring, the infant tolerated the bradycardia well (median 72 beats per minute (bpm)) and was discharged on day 21 of life. There was no indication for pacemaker at discharge, but he would be on regular follow-up with a paediatric cardiologist. [ABSTRACT FROM AUTHOR]

Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes.

Sjögren’s syndrome (SjS) is a systemic autoimmune disease, most common in middle-aged women, characterized by chronic inflammation of exocrine glands. It can present by itself or alongside other autoimmune diseases. Main symptoms are dry eyes and mouth, but the disease can affect joints, lungs, kidneys, peripheral nervous system (PNS), and rarely central nervous system (CNS). SS-A antibodies in SjS can pass the fetoplacental barrier and cause congenital heart block. Patients with SjS are at higher risk of developing lymphoma.

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 (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3–4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died. Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known: • Congenital complete heart block is a very rare condition associated with high morbidity and mortality. • Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect. What is New: • Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death. [ABSTRACT FROM AUTHOR]

Congenital complete heart block (CCHB) affects 1 in 20,000 newborns. This study evaluates fetal and neonatal risk factors predictive of neonatal pacemaker placement in antibody-mediated complete heart block. The Children's Hospital Los Angeles institutional fetal, pacemaker, and medical record databases were queried for confirmed SSA/SSB cases of CCHB between January 2004 and July 2019. Cases excluded were those with a diagnosis beyond the neonatal period, diagnosis of a channelopathy, or if maternal antibody status was unknown. We recorded the gestational age (GA), birth weight (BW), fetal heart rates (FHRs) of the last echocardiogram before delivery, specific neonatal ECG and echocardiogram findings, age at pacemaker placement, and mortality. Of 43 neonates identified with CCHB, 27 had confirmed maternal antibody exposure. Variables associated with neonatal pacemaker implantation were FHRs < 50 bpm (p = 0.005), neonatal heart rates < 52 bpm (p = 0.015), and neonatal left ventricular fractional shortening (FS) percentages < 34% (p = 0.03). On multivariate analysis, FHR remained significant (p = 0.03) and demonstrated an increased risk of neonatal pacemaker placement by an odds ratio of 12.5 (95% CI 1.3–116, p = 0.05). The median GA at which the FHR was obtained was 34 weeks (IQR 26–35 weeks). Neonatal pacemaker placement was highly associated with a FHR < 50 bpm, neonatal HR < 52 bpm, and neonatal FS < 34%. FHRs at 34 weeks GA (IQR 26–35 weeks) correlated well with postnatal heart rates and were predictive of neonatal pacemaker placement. [ABSTRACT FROM AUTHOR]

Congenital complete heart block (CCHB) defines atrioventricular conduction abnormalities diagnosed in utero or within the first 27 days of life. Maternal autoimmune disease and congenital heart defects are most commonly responsible. Recent genetic discoveries have highlighted our understanding of the underlying mechanism. Hydroxychloroquine shows promise in preventing autoimmune CCHB. Patients may develop symptomatic bradycardia and cardiomyopathy. The presence of these and other specific findings warrants placement of a permanent pacemaker to relieve symptoms and prevent catastrophic events. The mechanisms, natural history, evaluation, and treatment of patients with or at risk for CCHB are reviewed. [ABSTRACT FROM AUTHOR]

Introduction: Pacing‐induced cardiomyopathy (PICM) is a potential complication of chronic right ventricular (RV) pacing, but its characterization in adult patients is often complicated by pre‐existing cardiomyopathy. This study investigated the incidence of PICM in patients with congenital heart block (cHB) who have conduction disease from birth without confounding pre‐existing cardiac conditions. Methods and Results: This retrospective cohort analysis included 42 patients with cHB and baseline left ventricular ejection fraction (LVEF) ≥50%. Kaplan–Meier analysis was used to assess freedom from cardiomyopathy (defined as LVEF <50%) between paced and nonpaced patients. Patients were 26 ± 3 years old at first presentation, 64% were women and baseline LVEF was 60.0 ± 0.2%. Median follow‐up from birth was 35 (interquartile range [IQR]: 20–42) years with a median of 6.7 years (IQR: 3.6–9.2) at our institution. Thirty‐two patients received pacing at mean age 21 ± 3 years. Patients receiving a pacemaker (PM) were significantly more likely to develop a cardiomyopathy (p =.021) and no patient developed a cardiomyopathy in the absence of a PM. Four patients who developed a new cardiomyopathy were upgraded to biventricular pacing, leading to stabilization or improvement of LVEF. Conclusion: In a relatively young and healthy cHB cohort, RV pacing is associated with a higher risk of developing a cardiomyopathy. These data confirm the deleterious effects of RV pacing on myocardial function in patients without pre‐existing structural cardiac disease and has clinical implications to the management of patients with cHB. [ABSTRACT FROM AUTHOR]

Anti-Ro antibodies are detected frequently in the general population, but more so in patients with autoimmune conditions as Sjögren’s syndrome and systemic lupus erythematous (SLE). During pregnancy, anti-Ro antibodies can cross the placenta by hijacking physiological mechanisms and can have deleterious effects on the fetus. Administration of hydroxychloroquine (HCQ) to pregnant women with documented anti-Ro antibodies has been shown to prevent congenital heart block (CHB ). Serial fetal ultrasound scans and echocardiograms are controversial in pregnant women with anti-Ro antibodies. When complete CHB is diagnosed, this is irreversible and can lead to fetal heart failure, hydrops, and death. After delivery, babies with complete CHB require pacemaker. In the presence of maternal anti-Ro antibodies, there is a high risk of recurrence of CHB for future pregnancies, if there is a previously affected child. Adequate counselling and prophylactic treatment with HCQ should be encouraged. [ABSTRACT FROM AUTHOR]

A female newborn, born at 38 weeks from an uncomplicated pregnancy in a healthy 36-years-old mother, presented at birth with desquamative erythematous plaques with irregular borders, distributed bilaterally in the orbital, nasal and malar regions. Although there was no history of maternal autoimmune disease, neonatal lupus (NL) was suspected. Maternal and newborn screenings were positive for Sjögren syndrome type A antigen (anti-Ro/SSA) antibodies. No other alterations of NL were found in the newborn. Rheumatologic consultation on the mother showed no other alterations besides the antibodies. The newborn was discharged on day 3 of life without treatment and recommendations to avoid sun exposure. Outpatient follow-up was ensured in neonatology, dermatology and pediatric cardiology. The rash resolved during the first year of life, leaving slight local skin atrophy. NL is a rare transferred autoimmune disease with an incidence estimated as 1:20,000 live births. It occurs due to placental transfer of maternal autoantibodies. The major manifestations are cardiac and cutaneous, but hepatic, hematologic or neurologic findings may also be present. The rash usually affects the face and scalp and may be present at delivery but more often develops later, after exposure to ultraviolet light. It usually resolves within the first year of life without sequelae. NL is the leading cause of congenital heart block, but if it is not present at birth it rarely develops. Some mothers do not have a known autoimmune disease at the time of birth but may develop it later in life. Despite NL being a passively acquired autoimmune disease, the child is at increased risk of rheumatologic disease in childhood or adolescence.

Objective: To evaluate the association between ethnicity and neonatal lupus erythematosus (NLE), as well as specific NLE manifestations in a large multiethnic population.Methods: We conducted a cohort study of the children (≤ 1 yr of age) seen in the NLE clinic at The Hospital for Sick Children (SickKids), between January 2011 and April 2019. The cohort was divided into European, non-European, and mixed European-non-European groups according to parent-reported child's ethnicity (Canada Census categories). Outcomes were NLE and specific NLE manifestations (cardiac, cutaneous, cytopenias, transaminitis, and macrocephaly). The frequency of NLE and specific manifestations were compared between ethnic groups (Fisher exact test). We tested the association between ethnicity and (1) NLE risk, and (2) specific NLE manifestations with logistic regression models, including covariates for child's sex, maternal rheumatic disease status during pregnancy, and maternal use of antimalarials during pregnancy (multiple comparisons threshold P < 0.008).Results: We included 324 children born to 270 anti-Ro antibody-positive mothers. Median age at first visit was 1.8 (IQR 1.4-2.3) months, and median follow-up time was 12 (IQR 2-24) months. The majority was non-European (48%), with 34% European, and 18% mixed European-non-European. There was no significant association between non-European ethnicity (OR 1.18, 95% CI 0.71-1.94, P = 0.51), mixed European-non-European ethnicity (OR 1.13, 95% CI 0.59-2.16, P = 0.70), and NLE risk compared with European ethnicity. We also did not find an association between ethnicity and specific NLE manifestations in univariate or multivariable-adjusted models.Conclusion: In a large multiethnic cohort, there was no association between a child's ethnicity and NLE risk or specific NLE manifestations. [ABSTRACT FROM AUTHOR]

Most rheumatic diseases (RDs) have a predilection for women in the reproductive age group. Common drugs used in rheumatology practice have identified risks to the fetus; thus, adequate pregnancy counseling is of utmost importance. Contraception and adequate preparation for the same can enhance the experience of motherhood and decrease intra as well as peripartum complications. The knowledge, as well as practices of contraception in autoimmune diseases, are low and varied in various populations. The challenges faced in different RDs are unique and keen understanding can be fruitful toward better patient care. A multi-disciplinary team effort between the patient, obstetricians, and the rheumatologist is the key to better maternal and fetal outcomes. [ABSTRACT FROM AUTHOR]

Neonatal lupus erythematosus is an autoimmune disease acquired during fetal life as a result of transplacental passage of maternal anti-Sjögren's-syndrome-related antigen A (anti-SSA/Ro), anti-Sjögren's-syndrome-related antigen B (anti-SSB/La) or anti-U1 ribonucleoprotein (anti-U1-RNP) antinuclear autoantibodies. Clinical manifestations include skin lesions, congenital heart block, hepatobiliary involvement and cytopenias. Most of the disorders disappear spontaneously after clearance of maternal antibodies. Cardiac symptoms, however, are not self-resolving and often pacemaker implantation is required. Diagnosis is based on clinical presentation and the presence of typical antibodies in the mother's or infant's serum. Neonatal lupus erythematosus may develop in children born to anti-SSA/Ro or anti-SSB/La women with various systemic connective tissue diseases. However, in half of the cases, the mother is asymptomatic, which may delay the diagnosis and have negative impact on the child's prognosis. Testing for antinuclear antibodies should be considered in every pregnant woman since early treatment with hydroxychloroquine or intravenous immunoglobulin (IVIG) has proven to be effective in preventing congenital heart block. [ABSTRACT FROM AUTHOR]

Anti-Ro/SSA antibodies are associated with a risk of 1-2 % to develop complete atrioventricular block (AVB) in fetuses of positive mothers. Complete AVB is irreversible, but studies suggest that anti-inflammatory treatment during the transition period from a normal fetal heart rate (FHR) to an AVB might stop this progression and restore sinus rhythm. The most efficient method for diagnostic evaluation of this arrhythmia is the pulsed-Doppler fetal echocardiography. However, weekly or bi-weekly recommended fetal echocardiographic surveillance can rarely identify an AVB in time for treatment success, also because the transition from a normal rhythm to a third degree AVB is very fast. Daily FHR monitoring in a medical facility could increase the chances of identifying the AVB onset but is difficult to realize. For this reason, an alternative method of FHR monitoring, performed directly by mothers in their home context, has been recently proposed. We present a case report utilizing this approach and review the current evidence about this condition. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]