Impact of early detection and steroid treatment on fetal ventricular heart rate and pacemaker implantation in anti-Ro/SSA positive congenital heart block.

INTRODUCTION: We investigated the effects of timing of detection and transplacental fluorinated steroid treatment on ventricular heart rate (HR) and age at pacemaker implantation in fetal third-degree atrioventricular block (AVB). MATERIAL AND METHODS: Twenty-five of 31 fetuses diagnosed with Ro/SSA autoantibody-positive AVB II-III at our tertiary fetal cardiology center (2000-2020) and AVB III as final feto-neonatal outcome were reviewed. RESULTS: AVB was detected approximately 5 weeks earlier in pregnancy if followed in a surveillance program compared to cases referred from primary care for bradycardia (20.6 [2.3] [mean (SD)] vs. 25.4 [3.2] weeks, p = 0.001). AVB detected before 24 weeks had higher HR than those detected later in gestation (63.3 [6.9] vs. 57.2 [6.9] bpm, p = 0.042), with a larger proportion having HR >60 bpm (80% vs. 33%, p = 0.041). The 17/25 cases that received treatment with fluorinated steroid were diagnosed earlier in gestation, with higher HR at diagnosis (61.7 [7.1] vs. 54.7 [6.3] bpm, p = 0.026), 1-2 weeks after diagnosis/treatment start, and before birth (65.4 [12.4] vs. 54.9 [5.7] bpm, p = 0.030) than untreated cases. Overall, 11 cases were commenced on betamimetics: three at diagnosis and eight at or after the examination made 1-2 weeks after diagnosis/treatment start, without any HR improvement. Two of 24 surviving babies were born preterm, and 4/24 received a neonatal pacemaker. Age at pacemaker implantation correlated significantly with HR before birth (Spearman R 0.57, p = 0.004), and fetuses with HR >60 bpm had a higher rate of pacemaker-free survival at three (90% vs. 40%, p = 0.018) and 12 months of age (80% vs. 13%, p = 0.002). The same trend was observed in pacemaker-free survival at 3 months of age in fluorinated steroid-treated compared to untreated cases (71% vs. 38%, ns). CONCLUSIONS: Our data confirm that AVB III detected earlier in gestation have a higher HR, and suggest that this higher HR can be successfully maintained to the end of gestation in cases treated with fluorinated steroids. Fetuses with HR >60 bpm before birth had a lower rate of pacemaker implantation at 3 and 12 months of age.

Fetal and Maternal Outcomes in a Cohort of Patients With Primary Sjogren's Syndrome: An Observational Study.

Background Pregnant women with primary Sjogren's syndrome (PSS) have a high incidence of maternal and fetal complications due to immunological variations caused by maternal antibodies (anti-Sjogren's-syndrome-related antigen A (SSA) and anti-anti-Sjogren's-syndrome-related antigen B (SSB) crossing the placenta from the 12th week of gestation, mediating the tissue damage. A multidisciplinary approach is required in the management of such patients. Data regarding the effects of PSS on pregnancy are deficient in the Indian context. Methods This was a retrospective observational study on the maternal and fetal outcomes of PSS on a cohort of pregnant women treated at our tertiary care center between 2011 and 2020. Patients who satisfied the criteria for PSS were included, and patients with other associated autoimmune disorders were excluded. Maternal age, number of miscarriages, prior obstetric history, and maternal and fetal complications were recorded and statistically analyzed. Results There were 16 pregnancies in 10 women with PSS (incidence: 1/1,000 pregnancies/year) in our study. The mean gestational age of the mother at presentation was 31 ± 9.0 weeks. Oligohydramnios in five (11.8), intrauterine fetal demise (IUFD) in two (11.8), and first-trimester medical termination of pregnancy (MTP) in four (23.5) were noted. The weight of neonates was 2.3 ± 0.8 kg, and the mean duration of neonatal intensive care (NICU) stay was seven days. Fetal echo revealed congenital heart block (CHB), with six (50.0%) complete and one (8.3%) incomplete (p = 0.004). One baby needed a permanent pacemaker. Conclusion Maternal and fetal complications are high in our set of mothers with PSS. Early detection, regular follow-up, and a multidisciplinary approach may improve the outcome.

Dual challenge inside the womb: a case report of concomitant fetal atrio-ventricular block associated with maternal anti-SSA antibodies and fetal tachyarrhythmia diagnosed as Wolff-Parkinson-White syndrome after birth.

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.

Sjogren's Antibodies and Neonatal Lupus: A Scoping Review.

Sjogren's syndrome (SS) is an autoimmune disease characterized by inflammation of exocrine glands. The disorder predominantly affects middle-aged women. Autoantibodies, including anti-SS-A/Ro and anti-SS-B/La antibodies, are present in most cases of SS. These antibodies can cross the placenta and likely play a role in pregnancy complications as well as the development of neonatal lupus, resulting in congenital heart block (CHB). It is essential to monitor the fetus for CHB during pregnancy. In particular, screening with echocardiography and monitoring heart rate at home are recommended practices. Regarding medical management, hydroxychloroquine and glucocorticoids have shown promise in reducing cardiac manifestations, but further research is needed to elucidate their longer term efficacy and safety. This scoping review analyzes literature from 2001 to 2024, focusing on pregnancy outcomes among women with SS, clinical manifestations of neonatal lupus, the role of anti-SS-A/Ro and anti-SS-B/La antibodies in the development of neonatal lupus and CHB, and emphasizes the need for future research efforts to refine treatment protocols and enhance clinical care strategies for pregnant women with SS.

Congenital Complete Heart Block-To Stimulate (When?) or Not to Stimulate?

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.

Molecular Mechanisms of Fetal and Neonatal Lupus: A Narrative Review of an Autoimmune Disease Transferal across the Placenta.

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.

Isolated non-immune-mediated second-degree atrioventricular block in the fetus: natural history and predictive factors for spontaneous recovery.

OBJECTIVES: To determine the clinical course of fetal isolated non-immune-mediated second-degree atrioventricular block (AVB) and the factors associated with spontaneous recovery in these cases. METHODS: Fetuses with isolated non-immune-mediated second-degree AVB were recruited prospectively between 2014 and 2022. These fetuses were divided into two groups: those which recovered spontaneously and those which did not. Maternal and fetal characteristics and intrauterine and postnatal outcomes were compared between the two groups. RESULTS: The study cohort included 20 fetuses with isolated non-immune-mediated second-degree AVB, diagnosed at a median gestational age of 22.0 (range, 17.0-35.0) weeks. In 12 fetuses, 1:1 atrioventricular conduction was restored spontaneously in utero and there was no recurrence during the postnatal follow-up period. In the remaining eight fetuses, second-degree AVB was maintained and, in six of these, the pregnancy was terminated on parental request. Of the two liveborn children who had persistent second-degree AVB prenatally, one had progressed to complete AVB at the latest follow-up, at the age of 34 months, but was asymptomatic, without heart enlargement or dysfunction. The other child progressed to complete AVB after delivery and was diagnosed with type-2 long QT syndrome. This infant died aged 2 months. Fetuses in the group that recovered spontaneously had earlier gestational age at diagnosis (median, 20.0 (range, 17.0-26.0) vs 24.5 (range, 18.0-35.0) weeks; P = 0.004) and higher atrial rate at diagnosis (median, 147 (range, 130-160) vs 138 (range, 125-149) bpm; P = 0.006) in comparison with the group that did not recover spontaneously. The best cut-off values for prediction of failure to recover spontaneously were 22.5 weeks' gestational age at diagnosis and 144 bpm atrial rate at diagnosis, with sensitivities of 87.5% and 75.0%, respectively, and specificities of 92.0% and 87.5%, respectively. CONCLUSIONS: The outcome of 60% of fetuses with isolated non-immune-mediated second-degree AVB was favorable. Earlier gestational age and higher atrial rate at diagnosis were associated with spontaneous reversion to normal sinus rhythm. Prenatal genetic testing should be performed in cases with persistent AVB, to exclude heritable disorders including long QT syndrome. These findings provide important information for clinical management and prenatal counseling in these cases. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Home monitoring of fetal heart rhythm: Lived experiences of women with anti-SSA/Ro52 autoantibodies and their co-parents.

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.

Late-Onset Dilated Cardiomyopathy in Auto Immune-Mediated Complete Congenital Heart Block: A Case Report.

Complete congenital heart block (CHB), a rare and fatal bradyarrhythmia observed in children, carries significant mortality and morbidity. When congenital heart block occurs in isolation with a structurally normal heart, it prompts suspicion of an autoimmune etiology, wherein maternal antibodies are transmitted transplacentally, impacting the fetal conducting system. The manifestation of congenital complete atrioventricular block (CCAVB) can lead to complications such as dilated cardiomyopathies, arrhythmias, and fibroelastosis in certain cases. Notably, dilated cardiomyopathy is a significant prognostic factor in children diagnosed with congenital heart block. Pathological investigations have revealed the presence of antibodies, complements, and indicators of inflammation or fibrosis across the myocardium, emphasizing the shared molecular mechanisms between CCAVB and the development of dilated cardiomyopathy (DCM). This article presents the case of a one-year-old female child who presented with signs of dilated cardiomyopathy, later identified through retrospective evaluation as having autoimmune congenital heart block. The mother of the child was diagnosed with Sjogren's syndrome, characterized by positive anti-RO titers. Remarkably, the child remained asymptomatic for a year without the need for pacing intervention. The child's condition was successfully stabilized with appropriate treatment, and plans for pacemaker insertion will be considered once specific criteria are met. The onset of cardiomyopathy in a known case of CCAVB should serve as a crucial alert for prognostic considerations and the potential necessity for early-pacing intervention.

Autoimmune congenital heart block: a case report and review of the literature related to pathogenesis and pregnancy management.

Autoimmune congenital heart block (ACHB) is a passively acquired immune-mediated disease characterized by the presence of maternal antibodies against components of the Ro/SSA and La/SSB ribonucleoprotein complex that mainly affects the cardiac conducting system. ACHB occurs in 2% of women with positive anti-Ro/SSA and anti-La/SSB antibodies and causes a high risk of intrauterine fetal death, neonatal mortality, and long-term sequelae. In this review, we first describe a case of ACHB to provide preliminary knowledge. Then, we discuss the possible pathogenic mechanisms of ACHB; summarize the pregnancy management of patients with positive anti-Ro/SSA and anti-La/SSB antibodies and/or rheumatic diseases, the prevention of ACHB, and the treatment of ACHB fetuses; and propose routine screening of these antibodies for the general population. Careful follow-up, which consists of monitoring the fetal heart rate, is feasible and reassuring for pregnant women with positive anti-Ro/SSA and/or anti-La/SSB antibodies to lower the risk of ACHB in fetuses. Moreover, maternal administration of hydroxychloroquine may be useful in preventing ACHB in pregnant women with anti-Ro/SSA and/or anti-La/SSB antibodies.

Reverse complete heart block using transcutaneous pacing and repeated plasmapheresis in a neonate with lupus: a case report.

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.

Pregnancy and Inflammatory Rheumatological Diseases: A Single-Center Retrospective Cohort Study.

Background Rheumatic diseases pose risks to pregnant women, leading to complications like preterm birth, congenital heart block, and pregnancy loss. These diseases are expected to deteriorate during pregnancy and further in the postpartum period. The impact of these diseases on the pregnancy will add further burden on the patient, fetus, physician, and healthcare system. Advances in diagnosis and treatment have improved outcomes making them similar to that of healthy women, but close follow-up in a multidisciplinary clinic is essential. The objective of this study is to study the outcome of pregnancy in women with rheumatological disease and the behavior of the disease during pregnancy. Methods A retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC) in Riyadh, Saudi Arabia, to compare the outcomes of pregnancy across three rheumatological diseases: Sjogren syndrome (SS), systemic lupus erythematosus (SLE), and rheumatoid arthritis (RA) from 2016 to 2021. A total of 128 pregnancies in 107 women with rheumatological diseases were included in this study. Pregnancy measures and outcomes were investigated by assessing maternal health, fetal health, and pregnancy complications, specifically maternal disease activity, medications to control the disease, infection, preterm birth, birth weight, abortions/stillbirths, mode of delivery, bleeding, preeclampsia, congenital heart block, and neonatal lupus. Results There were 55 patients with RA (63 RA pregnancies), 44 with SLE (54 SLE pregnancies), and eight with primary SS (11 SS pregnancies). In most of the pregnancies (n= 108; 95.58%), the patients were in clinical remission before pregnancy. Lupus nephritis, which was in remission before pregnancy, has been reported in nine (16.67%) out of 54 SLE pregnancies. Vaginal delivery was the most common mode of delivery (n=87; 67.97%). On the other hand, there were 38 cesarean sections (29.69%). Rheumatological disease flares occurred in 10 pregnancies (7.87%). One hundred and twenty-two live births were delivered. Preterm infants were born in 25 pregnancies (20.16%), and 16 (13.22%) of the newborns needed neonatal intensive care unit (NICU) care. Interestingly, congenital heart block (CHB) was found in five (12.2%) neonates out of 41 anti-SS-related antigen A (anti-SSA) positive mothers; one of those five died from heart block. Eleven neonates were delivered with positive serology, and five were diagnosed with neonatal lupus. Conclusion The outcome of pregnancy in patients with rheumatological disease is favorable. A multidisciplinary team approach and close clinical follow-up are the cornerstone for such success. A small dose of prednisolone (5 mg or less) is safe and will not have a negative impact on maternal or fetal health. CHB is a concern for pregnant women with positive anti-SSA.

Congenital Heart Block and Its Association With Anti-Ro and Anti-La Antibodies in Pregnancy: A Case Report of a Rare Entity and a Review of the Current Evidence.

Systemic lupus erythematosus (SLE) is a heterogeneous chronic, multisystem, inflammatory autoimmune disorder with variable clinical features, with its manifestations being attributed to the presence of multiple autoantibodies and their subsequent autoimmune reactions. Multiple organs may be involved, with the kidneys, the joints, and the skin being the most common, increasing maternal and fetal morbidity and mortality. Our current article describes the case of a 32-year-old primigravida who was referred to our department after the detection of fetal bradycardia and the strong suspicion of an underlying cardiac abnormality. After a detailed fetal and maternal assessment, the diagnosis of SLE-associated fetal congenital heart block was established, and the appropriate management and treatment were provided, factors that led to the uncomplicated delivery and prompt successful management of an otherwise severely affected fetus. Our work, also, includes a detailed review of the accumulated evidence regarding the association between autoantibodies and congenital heart block, the available screening modalities of the condition, and its potential therapeutic interventions.

Percutaneous extraction of a protruding atrial pacing lead in an 8-year-old child.

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. Learning objective: 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.

Congenital Heart Block.

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.

Case report: Challenges and implications of conduction system pacing in pediatrics: Case series.

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.

Autoimmune Congenital Complete Heart Block: How Late Can It Occur?

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.

Identifying Congenital Heart Block in Primary Care.

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.

Contribution of S100A4-expressing fibroblasts to anti-SSA/Ro-associated atrioventricular nodal calcification and soluble S100A4 as a biomarker of clinical severity.

Background: Fibrosis and dystrophic calcification disrupting conduction tissue architecture are histopathological lesions characterizing cardiac manifestations of neonatal lupus (cardiac-NL) associated with maternal anti-SSA/Ro antibodies. Objectives: Increased appreciation of heterogeneity in fibroblasts encourages re-examination of existing models with the consideration of multiple fibroblast subtypes (and their unique functional differences) in mind. This study addressed fibroblast heterogeneity by examining expression of α-Smooth Muscle Actin (myofibroblasts) and of S100 Calcium-Binding Protein A4 (S100A4). Methods: Using a previously established model of rheumatic scarring/fibrosis in vitro, supported by the evaluation of cord blood from cardiac-NL neonates and their healthy (anti-SSA/Ro-exposed) counterparts, and autopsy tissue from fetuses dying with cardiac-NL, the current study was initiated to more clearly define and distinguish the S100A4-positive fibroblast in the fetal cardiac environment. Results: S100A4 immunostaining was observed in 4 cardiac-NL hearts with positional identity in the conduction system at regions of dystrophic calcification but not fibrotic zones, the latter containing only myofibroblasts. In vitro, fibroblasts cultured with supernatants of macrophages transfected with hY3 (noncoding ssRNA) differentiated into myofibroblasts or S100A4+ fibroblasts. Myofibroblasts expressed collagen while S100A4+ fibroblasts expressed pro-angiogenic cytokines and proteases that degrade collagen. Cord blood levels of S100A4 in anti-SSA/Ro-exposed neonates tracked disease severity and, in discordant twins, distinguished affected from unaffected. Conclusions: These findings position the S100A4+ fibroblast alongside the canonical myofibroblast in the pathogenesis of cardiac-NL. Neonatal S100A4 levels support a novel biomarker of poor prognosis.

Ventricular Functional Analysis in Congenital Complete Heart Block Using Speckle Tracking: Left Ventricular Epicardial Compared to Right Ventricular Septal Pacing.

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.