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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.

Congenital complete heart block with concomitant biventricular noncompaction cardiomyopathy has been reported once previously. Although not universal, when restrictive physiology is present, impaired diastolic filling may pose a distinct challenge to pacing during the neonatal period. We present the case of a neonate with congenital complete heart block and biventricular noncompaction that resulted in severe diastolic dysfunction and atrioventricular dyssynchrony. We intentionally used 2:1 ventricular pacing to provide atrioventricular synchrony with every paced beat, and this resulted in hemodynamic and clinical improvement. This unconventional pacing technique may be beneficial in other neonates who have complete heart block and diastolic dysfunction.

Objective: Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge.Method: Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) in utero or neonatal period (up to 27 days after birth).Result: Eighty-nine cases of CHB were observed in 85 women with 88 pregnancies that occurred between 1969 and 2017. CHB was mostly detected in utero (84 cases, 94.2%), while five cases were observed in the neonatal period. A permanent pacemaker was implanted in 51 of 73 children born alive (69.8), whereas global mortality rate was 25.8% (23 cases): 16 in utero, five perinatal, and two during childhood. By univariate analysis, factors associated with fetal death were pleural effusion (p = 0.005, OR > 100; CI 95% 2.88->100 and hydrops (p = 0.003, OR = 14.09; CI 95% 2.01–122). Fluorinated steroids (FS) were administered in 71.4% pregnancies, and its use was not associated with better survival. Some centers treated all cases with fluorinated steroids and some centers did not treat any case. CHB was initially incomplete in 24 fetuses, and of them five cases of II degree block reverted to a lower degree block after treatments. Recurrence rate in subsequent pregnancies was 17.6% (3 out of 17). A prophylactic treatment was introduced in 10 of these 16 subsequent (58.8%) pregnancies, mostly with FS or high dose intravenous immunoglobulins.Conclusion: This is the first report from the Italian Registry of neonatal lupus/CHB. The live birth rate was nearly 80%, with nearly two thirds of the children requiring the implantation of a pacemaker. The management of fetuses diagnosed with CHB was heterogeneous across Italian Centers. The registry at present is mainly rheumatological, but involvement of pediatric cardiologists and gynecologists is planned.

Aims To describe aetiologies and temporal trends in young patients with atrioventricular block (AVB). Methods and results We identified all patients in Denmark, receiving their first pacemaker because of AVB before the age of 50 years between 1996 and 2015. Medical records were reviewed and clinical information and diagnostic work-up results were obtained to evaluate the aetiology. We used Poisson regression testing for temporal trends. One thousand and twenty-seven patients were identified, median age at time of implantation was 38 (interquartile range 25–45) years, 584 (56.9%) were male. The aetiologies were complications to cardiac surgery [n = 157 (15.3%)], congenital AVB [n = 93 (9.0%)], cardioinhibitory reflex [n = 52 (5.0%)], congenital heart disease [n = 43 (4.2%)], complication to radiofrequency ablation [n = 35 (3.4%)], cardiomyopathy [n = 31 (3.0%)], endocarditis [n = 18 (1.7%)], muscular dystrophy [n = 14 (1.4%)], ischaemic heart disease [n = 14 (1.4%)], sarcoidosis [n = 11 (1.1%)], borreliosis [n = 9 (0.9%)], hereditary [n = 6 (0.6%)], side-effect to antiarrhythmics [n = 6 (0.6%)], planned His-ablation [n = 5 (0.5%)], complication to alcohol septal ablation [n = 5 (0.5%)], and other known aetiologies [n = 11 (1.1%)]. The aetiology remained unknown in 517 (50.3%) cases. While the number of patients with unknown aetiology increased during the study period (P Conclusion In a nationwide cohort, the aetiology of AVB was identified in only half the patients younger than 50 years referred for first-time pacemaker implantation. The number of patients with unknown aetiology increased during the study period. These findings indicate need for better insight into aetiologies of AVB and improved diagnostic work-up guidelines.

Interferon-α (IFN-α), belonging to the family of type I interferons, is a cytokine involved in the pathogenesis of many autoimmune diseases, including systemic lupus erythematosus (SLE) and primary Sjögren’s syndrome (pSS). As there are no standardised tests for the direct measurement of IFN-α, the expression of the myeloid cell surface receptor SIGLEC1 on CD14+ monocytes was examined as potential biomarker for a type I interferon signature. While several studies have analysed the SIGLEC1 expression in SLE patients, further research is required into other autoimmune diseases, including pSS and congenital heart block (CHB). It was therefore the aim of this study to analyse the expression of SIGLEC1 on CD14+ monocytes, and how it is influenced by certain immunomodulatory medication, in pSS patients and in females with a CHB pregnancy complication. It was found that patients with pSS had an increased expression of SIGLEC1 compared to healthy individuals, with levels comparable to those found in SLE patients. PSS patients with a systemic or extraglandular disease manifestation (n = 16) had a significantly higher expression of SIGLEC1 compared to patients where the disease was restricted to the glandular organs (n = 15; p = 0.0001, Mann-Whitney U test (MWU)). A total of 9 pregnant females were included in the study whose children developed CHB in utero, and were compared to 14 pregnant at-risk females with anti-Ro autoantibodies who bore unaffected children. We found a significantly higher expression of SIGLEC1 and significantly higher plasma concentrations of IFN-α in the affected group compared to the unaffected group (p = 0.0034 and p = 0.0135, respectively, MWU), while there was no significant difference for plasma concentrations of interferon-γ induced protein 10 (IP10; p = 0.14, MWU). The expression of SIGLEC1 was reduced in patients with SLE, pSS, and females with a CHB pregnancy complication upon introduction of glucocorticoids and hydroxychloroquine. In summary, this study highlights a potential clinical use of the biomarker SIGLEC1 as a biomarker indicative of a more severe disease manifestation in patients with pSS, for dose titration and monitoring treatment response to hydroxychloroquine and glucocorticoids, as well as to other medication still in development, and for risk evaluation in pregnant females with anti-Ro antibodies. Further studies are needed to fully understand the pathogenetic and clinical implications of an increased SIGLEC1 expression, reflecting an activated type I interferon system, for patients with autoimmune diseases., Das Zytokin Interferon-α (IFN-α) aus der Familie der Typ-I-Interferone ist am Pathomechanismus vieler Autoimmunerkrankungen beteiligt, wie z.B. dem systemischen Lupus Erythematodes (SLE) und dem primären Sjögren-Syndrom (pSS). Dennoch gibt es keine standardisierten Testverfahren zur direkten Messung von IFN-α, sodass die Expression des auf Myelozyten exprimierten Oberflächenmoleküls SIGLEC1 als potentieller Biomarker für eine Typ-I-Interferon-Signatur untersucht wurde. Während vorherige Studien die Expression von SIGLEC1 in Patienten mit SLE untersucht haben, besteht weiterhin Forschungsbedarf in anderen Autoimmunerkrankungen, wie beispielsweise dem pSS und dem kongenitalen Herzblock (CHB). Es war daher das Ziel dieser Studie, in Patienten mit pSS und Frauen mit einer CHB-Schwangerschaftskomplikation sowohl die Expression von SIGLEC1 auf CD14+-Monozyten, als auch deren Beeinflussung durch immunmodulierende Medikamente zu untersuchen. Die Studie ergab, dass Patienten mit pSS gegenüber gesunden Kontrollprobanden eine erhöhte Expression von SIGLEC1 aufwiesen, die vergleichbar war zu der Expression in Patienten mit SLE. PSS-Patienten mit einer systemischen bzw. extraglandulären Krankheitsmanifestation (n = 16) hatten eine signifikant höhere Expression als Patienten, bei denen das Krankheitsgeschehen auf das Drüsengewebe begrenzt war (n = 15; p = 0.0001, Mann-Whitney-U-Test (MWU)). Es wurden 9 schwangere Frauen in die Studie eingeschlossen, deren Kinder im Uterus einen Herzblock entwickelten und mit 14 schwangeren Frauen verglichen, die mit Anti-Ro-Antikörpern zwar ein Risikoprofil aufwiesen, jedoch gesunde Kinder gebaren. Hier fanden wir ebenfalls eine signifikant höhere SIGLEC1-Expression, wie auch signifikant höhere IFN-α-Konzentrationen bei den betroffenen Frauen (p = 0.0034 und p = 0.0135, MWU), während die Konzentrationen von Interferon-γ-induziertem Protein 10 (IP-10) sich zwischen den beiden Gruppen nicht signifikant unterschieden (p = 0.14, MWU). Die Expression von SIGLEC1 konnte bei Patienten mit SLE und pSS, sowie bei Frauen mit einer CHB-Schwangerschaftskomplikation durch Glukokortikoide und durch Hydroxychloroquin gesenkt werden. Zusammenfassend erweitert diese Studie den potentiellen klinischen Nutzen von SIGLEC1, sowohl als Biomarker, der auf einen schwereren Krankheitsverlauf bei Patienten mit pSS hindeuten kann, zur Dosisfindung und Therapiekontrolle einer Therapie mit Glukokortikoiden und Hydroxychloroquin, bzw. auch von Medikamenten, die sich derzeit noch in der Entwicklung befinden, sowie zur Risikoevaluation bei schwangeren Frauen mit anti-Ro-Antikörpern. Weitere Studien sind erforderlich, um die vollständige pathogenetische und klinische Bedeutung einer erhöhten SIGLEC1-Expression als Indikator eines aktivierten Typ-I-Interferonsystems für Patienten mit Autoimmunerkrankungen zu verstehen.

Autoimmune connective tissue-diseases are more frequent in women and deserve a multidisciplinary approach in which the dermatologist play a major role together with other physicians. Pregnancy in these patients has to be considered a high-risk situation, because of possible worsening of the mother's disease and increased morbility and mortality for the fetus; also, therapies have to be chosen carefully because some drugs cannot be used during pregnancy. For all these reasons, the decision to become pregnant needs to consider the type of disease, stage of disease, age and clinical condition, and requires a multidisciplinary approach. A correct counselling, a close monitoring, a specific approach based on the risks involved and the use of appropriate therapies are the keys to obtain optimal pregnancy outcomes.

Objectives In this study we aimed to investigate foetal and maternal pregnancy outcomes from a large multicentre cohort of women diagnosed with MCTD and anti-U1RNP antibodies. Methods This multicentre retrospective cohort study describes the outcomes of 203 pregnancies in 94 consecutive women ever pregnant who fulfilled the established criteria for MCTD with confirmed U1RNP positivity. Results The foetal outcomes in 203 pregnancies were as follows: 146 (71.9%) live births, 38 (18.7%) miscarriages (first trimester pregnancy loss of Conclusion In our multicentre cohort, women with MCTD had a live birth rate of 72%. While the true frequency of heart block associated with anti-U1RNP remains to be determined, this study might raise the consideration of echocardiographic surveillance in this setting. Pregnancy counselling should be considered in women with MCTD.

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.

Summary Congenital heart block (CHB) is a potentially lethal condition characterized by a third-degree atrioventricular block (AVB). Despite anti-Ro52 antibodies being detected in nearly 90% of mothers of affected children, CHB occurs in only 1–2% of anti-Ro/Sjögren's-syndrome-related antigen A (SSA) autoantibody-positive pregnancies. Maternal antibodies have been suggested to bind molecules crucial to fetal cardiac function; however, it remains unknown whether a single antibody profile associates with CHB or whether several specificities and cross-reactive targets exist. Here, we aimed to define further the reactivity profile of CHB-associated antibodies towards Ro52p200 (amino acid 200-239). We first analysed reactivity of a monoclonal anti-Ro52 antibody shown to induce AVB in rats (7.8C7) and of sera from anti-Ro52p200 antibody-positive mothers of children with CHB towards a panel of modified Ro52p200 peptides, and subsequently evaluated their potential to induce AVB in rats upon transfer during gestation. We observed that CHB maternal sera displayed a homogeneous reactivity profile targeting preferentially the C-terminal part of Ro52p200, in contrast to 7.8C7 that specifically bound the p200 N-terminal end. In particular, amino acid D233 appeared crucial to maternal antibody reactivity towards p200. Despite low to absent reactivity towards rat p200 and different binding profiles towards mutated rat peptides indicating recognition of different epitopes within Ro52p200, immunoglobulin (Ig)G purified from two mothers of children with CHB could induce AVB in rats. Our findings support the hypothesis that several fine antibody specificities and cross-targets may exist and contribute to CHB development in anti-Ro52 antibody-positive pregnancies.

Background Children from mothers with systemic lupus erythematosus are frequently born with congenital heart block. This study aimed at evaluating long-term outcome because long-term data are scarce. Methods In the database of pediatric and congenital heart disease (University Hospitals Leuven), 19 children from systemic lupus erythematosus mothers and who were born with or developed atrioventricular block were identified. All records were reviewed for disease course and outcome. Results Median follow-up time was 7 years (interquartile ranges [IQR] 4.5–13 years). One child had no heart block at birth and developed only a first-degree block during follow-up. One had a second-degree heart block and developed a complete heart block. Seventeen patients (89%) were born with a complete heart block. Seventeen patients (89%) needed a definitive pacemaker. In all, epicardial leads were used at first implantation. Eighty-two percent received their pacemaker in the first year of life. The first battery had a median lifetime of 5 years (IQR 3.5–5 years), the second 6 years (IQR 4.5–6.3 years), and the third 5 years (IQR 5–6 years). Note that 47% of patients needed a lead replacement due to lead problems. Only one pericardial tamponade after pacemaker implantation. No device or lead infections occurred. The left ventricular systolic function at latest follow-up was normal for all. No patients died. Conclusion In children with heart block born from systemic lupus erythematosus mothers, an early need for pacemaker implantation was documented. The overall battery life was acceptable, but there was a high need for lead replacement. Complication rate was low. Late outcome was good.

Sjogren's syndrome is one of the most common autoimmune disorders and has a female predominance. Maternal circulating autoantibodies such as anti-Ro/SSA and anti-La/SSB antibodies can cause congenital heart block of fetus, and in severe case, emergency pacemaker implantation may be needed for neonate. Therefore, it is very important to understand maternal and fetal condition and pay attention to the status of the neonate during delivery. In this paper, we present a case of patient with Sjogren's syndrome who underwent cesarean section under spinal anesthesia.

Neonatal lupus (NL) is a syndrome diagnosed in neonatal infants, whose mothers frequently suffer from autoimmune rheumatic diseases, manifested by two major signs: skin lesion and cardiac lesion. The paper gives data from the history of a description of the syndrome, as well as current ideas on its pathogenesis, considers both typical and rarer clinical manifestations of the disease, and presents approaches to NL therapy and prevention.

Objective Congenital heart block (CHB) may develop in fetuses of women with anti-Ro/SSA autoantibodies, and carries substantial morbidity and mortality. The aim was to evaluate how information on CHB is imparted and identify areas of improvement. Methods A questionnaire was distributed to anti-Ro/SSA antibody-positive women who had either participated in a surveillance programme but whose expected child did not develop CHB ( n = 100, denoted Doppler-Assessed Pregnancies (DAP) group) or given birth to a child with CHB ( n = 88, denoted CHB-Affected Pregnancies (CAP) group). Results The response rate was 83% (157/188). Most women received the information on CHB when they were already pregnant (DAP group 60%, CAP group 83%). However, a majority of them would have wanted the information before pregnancy (DAP group 52%, CAP group 56%), and most stated that it would not have influenced their decision to have a child (DAP group 77%, CAP group 58%). The ability to both understand the information and to perceive the information as sufficient were significantly higher when someone trained in paediatric cardiology gave the information. Conclusions Our findings indicate that information on CHB should be given to women before pregnancy. The data further highlight the importance of having specific knowledge for giving relevant and understandable, yet sufficient information.

Introduction: The use of cardiac implantable electronic devices (CIEDs) with transvenous leads (TVLs) in young patients continues to increase. Challenges persist, particularly related to TVLs complications. We present a unique case of redundant lead prolapse into the right ventricular outflow tract (RVOT) causing symptomatic pulmonary regurgitation (PR). Case Report: A 23-year-old female with congenital heart block underwent initial transvenous (TV) pacemaker implant at age 9 years, followed by new lead implants at age 20 due to lead fracture. The original TVLs were abandoned. She developed progressive exercise intolerance and exertional dyspnea. Catheterization showed normal pulmonary pressures; exercise testing indicated worsening PR and limited LV functional increase. Imaging confirmed significant lead prolapse of the original right ventricular (RV) lead causing PR. Extraction with cardiac resynchronization therapy-pacemaker (CRT-P) upgrade was recommended. A superior/inferior extraction approach was employed for extraction of all TVLs with CRT-P implant using the retained venous access post-extraction. Post-implant imaging revealed appropriately positioned leads, normalization of left ventricular (LV) function, and trace tricuspid regurgitation (TR) and trace-mild PR. Conclusion: This case highlights an unusual complication of excessive TVLs slack. Challenges remain with long-term TVLs management in young patients. Proactive lead management is recommended to ensure CIED benefit while reducing TVLs complications. When extraction is considered, a superior/inferior approach can afford tremendous versatility to maximize success.

ObjectiveCongenital heart block (CHB) may develop in fetuses of Ro/SSA autoantibody-positive women. Given the rarity of CHB, information on comorbidity and complications later in life is difficult to systematically collect for large groups of patients. We therefore used nation-wide healthcare registers to investigate comorbidity and outcomes in patients with CHB and their siblings.MethodsData from patients with CHB (n= 119) and their siblings (n= 128), all born to anti-Ro/SSA-positive mothers, and from matched healthy controls (n= 1,190) and their siblings (n= 1,071), were retrieved from the Swedish National Patient Register. Analyses were performed by Cox proportional hazard modelling.ResultsIndividuals with CHB had a significantly increased risk of cardiovascular comorbidity, with cardiomyopathy and/or heart failure observed in 20 (16.8%) patients versus 3 (0.3%) controls, yielding a HR of 70.0 (95% CI 20.8 to 235.4), and with a HR for cerebral infarction of 39.9 (95% CI 4.5 to 357.3). Patients with CHB also had a higher risk of infections. Pacemaker treatment was associated with a decreased risk of cerebral infarction but increased risks of cardiomyopathy/heart failure and infection. The risk of systemic connective tissue disorder was also increased in patients with CHB (HR 11.8, 95% CI 4.0 to 11.8), and both patients with CHB and their siblings had an increased risk to develop any of 15 common autoimmune conditions (HR 5.7, 95% CI 2.83 to 11.69 and 3.6, 95% CI 1.7 to 8.0, respectively).ConclusionsThe data indicate an increased risk of several cardiovascular, infectious and autoimmune diseases in patients with CHB, with the latter risk shared by their siblings.

Objective To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies. Methods A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy. Results In anti-Ro/SSA positive women, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely. For women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs. For women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. To prevent CHB, HCQ was recommended by 67% of respondents and most would start pre-pregnancy (62%). Respondents were asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. For first degree, respondents recommended starting dexamethasone (53%) or HCQ (43%). For second degree, respondents recommended starting dexamethasone (88%). For third degree, respondents recommended starting dexamethasone (55%) or IVIg (33%), although 27% would not start treatment. Conclusion Despite the absence of official guidelines, many physicians with a focus on pregnancy and rheumatic disease have developed similar patterns in the screening, prevention and treatment of CHB.

Background: Anti-p200 antibodies have been receiving growing interest in view of findings associating their presence to risk of fetal autoimmune congenital heart block (CHB). The study compares and evaluates the performance of two assays currently being used for their detection. Methods: One hundred and sixteen pregnant women positive for anti-SSA/Ro52 antibodies were considered as the study population. Fifty women negative for anti-SSA/Ro52 antibodies were considered as the control population. Anti-p200 antibodies were analyzed using two home-made ELISA assays: one with biotinylated antigen and the other with free antigen. Results: The specificity of the p200-free assay was significantly higher with respect to that of the p200-biotin assay (p=0.023). Both methods showed a high area under curve (AUC), thus, a good accuracy. There was a significant prevalence of anti-p200 antibodies when the p200-free assay was used to analyze the sera of the pregnant women with CHB fetuses (p=0.007). Cohen’s κ and Spearman’s ρ coefficients showed a good concordance (0.71) and a high correlation (0.93), respectively. Conclusions: The p200-free assay with respect to the biotin-based method was more specific in detecting p200 antibodies in women positive for anti-SSA/Ro52 antibodies. In addition, only the p200-free method significantly found p200 antibodies in patients with fetal CHB.

His bundle is a part of the cardiac conduction system of mammals, birds and crocodilians. His bundle is important for high cardiac output in endothermic vertebrates. His bundle is a connection between atria and ventricles in fibrous atrioventricular isolation and it serves as an electrical conduction pathway via bundle branches to ventricular apex, where ventricular contraction begins. Defect in cardiac conduction system leads to cardiac arrhytmia and can cause sudden cardiac death. Thesis describes development of His bundle in mammals and birds, its phylogenensis and atrioventricular conduction in other vertebrates. Thesis also describes disorders of atrioventricular canal development, fibrous anulus and His bundle such a preexcitation syndromes and congenital heart block. Thesis also depicts history of a discovery of the atrioventricular conduction tissue. Key words: cardiac conduction system, His bundle, atrioventricular canal, anulus fibrosus, ventricular preexcitation, perinatal AVRT, WPW syndrome, congenital heart block

Aim To analyze the clinical features, outcomes and prognosis of neonatal lupus erythematosus (NLE) in China. Method We reviewed 12 NLE cases at the Peking Union Medical College Hospital and compared the data with 111 cases reported in China between 1990 and 2014. The Chinese medical journal search engines used in this study were Wanfang.data and Science China. Results No gender dominance in NLE incidence was found. Cutaneous lesions were present in more than 96% of patients, while cardiac, hematological and hepatobiliary manifestations were seen in 12.61%, 45.53% and 17.89% of cases, respectively. Congenital heart block (CHB) tended to be more persistent, with two cases showing CHB for 1 year and three cases persisting for 7–10 years. In this study more than 90% of mothers were anti-Sjogren's syndrome A positive, and 65.04% were asymptomatic prior to the pregnancy. Conclusion These results indicate that clinicians, especially dermatologists, in China should improve their recognition of this disease to avoid misdiagnosis, and more attention should be paid to the follow-up of NLE patients and their asymptomatic mothers.

Objective The risk for congenital heart block (CHB) associated with maternal Ro/SSA autoantibodies is low, but the possibility of treating early stages of disease has seen the introduction of Doppler echocardiographic surveillance programs with serial examinations during the CHB susceptibility weeks of pregnancy. The aim of the present study was to understand how Ro/SSA autoantibody-positive women having undergone Doppler echocardiographic surveillance programs and giving birth to children without CHB experienced their pregnancy and frequent ultrasound examinations. Methods A validated questionnaire based on data from an interview-study was distributed to Ro/SSA-positive women supervised with Doppler examinations during their pregnancy ( n = 100). Results The response rate was 79%. The majority of the women (61%) reported that the increased number of ultrasound examinations influenced their pregnancy, but in a positive way, with qualified information and additional support from health care personnel in conjunction with the examinations. Further, the visits to the clinic provided opportunities to see the ultrasound picture of the expected infant. However, one-third of the women also reported stress in relation to the examinations. Conclusions Fetal echocardiographic surveillance holds many and predominantly positive effects for Ro/SSA-positive women during pregnancy in addition to the medical advantages.

Background SelectSecure™ pacing leads (Medtronic Inc) are increasingly being used in pediatric patients and adults with structural congenital heart disease. The 4Fr lead is ideal for patients who may require lifelong pacing and can be advantageous for patients with complex anatomy. Objective The purpose of this study was to compare the extraction of SelectSecure leads with conventional (stylette-driven) pacing leads in patients with structural congenital heart disease and congenital atrioventricular block. Methods The data on lead extractions from pediatric and adult congenital heart disease (ACHD) patients from August 2004 to July 2014 at Bristol Royal Hospital for Children and the Bristol Heart Institute were reviewed. Multivariable regression analysis was used to determine whether conventional pacing leads were associated with a more difficult extraction process. Results A total of 57 patients underwent pacemaker lead extractions (22 SelectSecure, 35 conventional). No deaths occurred. Mean age at the time of extraction was 17.6 ± 10.5 years, mean weight was 47 ± 18 kg, and mean lead age was 5.6 ± 2.6 years (range 1–11 years). Complex extraction (partial extraction/femoral extraction) was more common in patients with conventional pacing leads at univariate ( P P = .04) levels. Lead age was also a significant predictor of complex extraction ( P Conclusion SelectSecure leads can be successfully extracted using techniques that are used for conventional pacing leads. They are less likely to be partially extracted and are less likely to require extraction using a femoral approach compared with conventional pacing leads.

Autoimmune congenital heart block (CHB) is an immune-mediated acquired disease that is associated with the placental transference of maternal antibodies specific for Ro and La autoantigens. The disease develops in a fetal heart without anatomical abnormalities that could otherwise explain the block, and which is usually diagnosed in utero, but also at birth or within the neonatal period. Autoantibody-mediated damage of fetal conduction tissues causes inflammation and fibrosis and leads to blockage of signal conduction at the atrioventricular (AV) node. Irreversible complete AV block is the principal cardiac manifestation of CHB, although some babies might develop other severe cardiac complications, such as endocardial fibroelastosis or valvular insufficiency, even in the absence of cardiac block. In this Review, we discuss the epidemiology, classification and management of women whose pregnancies are affected by autoimmune CHB, with a particular focus on the autoantibodies associated with autoimmune CHB and how we should test for these antibodies and diagnose this disease. Without confirmed effective preventive or therapeutic strategies and further research on the aetiopathogenic mechanisms, autoimmune CHB will remain a severe life-threatening disorder.

While fertility is preserved in females with systemic lupus erythematosus (SLE), it is well established that pregnancy in these patients is associated with adverse maternal and fetal outcomes, including pregnancy loss, pre-eclampsia, preterm delivery and intrauterine growth retardation, as well as neonatal mortality. Mechanisms underlying these adverse outcomes are poorly understood, and better understanding of these would allow development of targeted and personalized treatment strategies. Established risk factors for adverse pregnancy outcomes include active disease within 6 months prior to conception and during pregnancy, active nephritis, maternal hypertension, antiphospholipid antibodies and hypocomplementemia. While intensive monitoring is recommended, the comparative effectiveness of appropriate management strategies is unclear. While current strategies are able to achieve live births in 85-90% of pregnancies, certain aspects such as prevention of preterm birth, treatment of congenital heart block due to neonatal lupus and recurrent pregnancy loss despite best management, remains challenging. Pregnancy is also associated with an increased risk of flare of lupus, particularly in patients with active disease at time of conception or within 6 months prior to conception. Pregnant patients with SLE should be followed in a high-risk obstetric clinic, and care should be closely coordinated between the obstetrician and rheumatologist.