Risk Of Congenital Heart Block Research Articles

Management of systemic lupus erythematosus in pregnancy

Systemic lupus erythematosus is one of the most common autoimmune disorders affecting young women. Pregnant women with lupus are generally at higher risk for certain pregnancy complications than women without comorbidities. Even so, a pregnancy with lupus can be carried to term in optimal conditions if it is properly managed by a doctor. Monitoring is generally recommended six months after the onset of lupus symptoms, and ideally there should be no active lupus symptoms prior to conception. General screening tests should include the anti-phospholipid, anti-Ro and anti-La antibodies. Women who are positive for these antibodies have an increased risk of congenital heart block in the fetus. In addition, pregnant women with lupus have an increased risk of spontaneous abortion, intrauterine fetal growth restriction, pre-term birth, while neonatal lupus syndrome is a major fetal condition. The maternal risks are faced with disease flares, pre-eclampsia and other complications. Treatment options during pregnancy are limited to a few safe medications. For example, prednisone is unlikely to cause fetal malformations, but it increases the risk of diabetes and high blood pressure in the mother. Consequently, a careful multidisciplinary monitoring is essential for optimal results in pregnancy with lupus.

Fetal screening and prevention of autoimmune heart block: What about the French cardiofetalist practice?

Fetal autoimmune congenital heart block (CHB) is often diagnosed at an irreversible stage with a high related morbi-mortality. Cardiologic screening and preventive therapy during pregnancy are still not consensual. The objective of this study was to evaluate the prenatal follow up and management of pregnant women with anti-Ro/Sjögren's syndrome antigen A (SSA) or anti-La/Sjögren's syndrome antigen B (SSB) antibodies, among the French community of fetal cardiologists. Nationwide digital questionnaires were sent to the French community of fetal cardiologists. Fetal cardiologists were asked about their management, follow up, preventive care and curative therapy used in immune pregnancies with a CHB risk. Among the 45 fetal cardiologists surveyed, 38 (84%) accepted to respond. Regarding the follow-up of immune pregnancies, 97% of fetal cardiologists use to perform a dedicated systematic fetal echocardiography, twice a month, from 18 to 26 weeks of gestation. A more frequent and longer screening is usually performed for women who experienced a previous fetal immune CHB. 38% of fetal cardiologists would also propose a self-monitoring of fetal heart rate to complete their regular follow up. Regarding preventive therapy of fetal autoimmune CHB, the use of Hydroxychloroquine is recommended by 48% of fetal cardiologists. 43% would use bethametasone in case of a 1st and 2nd degree CHD and 24% in case of a 3rd CHD. 16% would also prescribe beta-mimetic drugs in case of 3rd atrio-ventricular block with fetal bradycardia. Treatments are usually introduced after a shared decision with internal medicine physicians and obstetricians. Most French fetal cardiologists perform a regular systematic follow-up in immune pregnancies with a CHB risk. This screening could be associated with a fetal heart rate self-monitoring at home. Preventive and curative therapies of CHD are still not consensual and rarely used, which is in line with their poor published efficacy.

Anti-SSA/Ro positivity and congenital heart block: obstetric and foetal outcome in a cohort of anti-SSA/Ro positive pregnant patients with and without autoimmune diseases.

Neonatal lupus (NL) is an acquired disease caused by the transplacental passage of anti-SSA/Ro antibodies. The rate of congenital heart block (CHB), its most serious manifestation, ranges from 1 to 5%. The aim of this study was to retrospectively assess the prevalence of CHB in anti-SSA/Ro positive pregnant women with or without systemic autoimmune diseases from 2010 to 2020. Patients underwent monthly visit and a shared follow-up programme of weekly (16th-24th week) foetal heart rate assessment by obstetric ultrasound. 322 pregnancies in 258 anti-SSA/Ro patients were included; 314 were followed from the beginning of pregnancy because of the known presence of anti-SSA/Ro autoantibodies and 1 case of CHB occurred in an anti-SSA/Ro+ asymptomatic subject (0.3%). In the same period, 8 additional patients were referred to our clinics after in utero CHB diagnosis and subsequent discovery of anti-SSA/Ro without a disease diagnosis. Globally, 9 cases of congenital CHB (2.8%) occurred: 7 complete, 1 II-III degree and 1 rst degree CHB. Anti-SSB/La positivity was associated with a higher risk of CHB (7.8% vs. 1.2%; p=0.0071). No differences in maternal or foetal outcomes were found in comparison with a large cohort of unselected pregnancies except for caesarian section. Hydroxychloroquine (HCQ) was used in 58.3% pregnancies, with a different prevalence according with maternal diagnosis. Our data suggest that anti-SSA/Ro positive patents with a de ned systemic autoimmune disease undergoing a strict follow-up since positive pregnancy test display a low risk of pregnancy complications, including but not limited to NL.

Treatment of Systemic Lupus Erythematosus with Anti SSA and SSB Positive Pregnancies

Systemic lupus erythematosus (SLE) is autoimmune disease that results in inflammation in various organ systems, frequent recurrences in the clinical course characterized by the production of antibodies against core antigens. Pregnancy will trigger flares. Increased intravascular volume in pregnancy can worsen abnormal heart and kidney function. Increasing the glomerular filtration rate by 50% exacerbated previously stable proteinuria. Pregnancy-induced hypercoagulability increases the risk of thrombosis. Anti-Ro/SSA was found in 35% of SLE patients and anti-La / SSB was found in 15% of SLE patients. The presence of Ro and La antibodies puts the fetus at a risk of developing neonatal lupus erythematosus (NLE) 6-13% and can reach up to 25% if the mother has previously given birth to a child with NLE. Neonatal lupus erythematosus including congenital heart block or cutaneous lupus. Congenital heart block (CHB) is a very serious complication. The American College of Rheumatology (ACR) recommends serial fetal echocardiography from 16 weeks of gestation continuing through to 26 weeks. Pregnancies with grade I and II CHB are treated with oral dexamethasone 4 mg daily because fluorinated glucocorticoids such as dexamethasone and betamethasone can cross the placenta, however, this therapy cannot be given in third-degree heart block. Early diagnosis and therapy will reduce the risk of congenital heart block in neonates after birth.

Autoimmune Congenital Heart Block: A Review of Biomarkers and Management of Pregnancy.

Autoimmune Congenital Heart Block (CHB) is an immune-mediated disease due to transplacental passage of circulating anti-Ro/SSA and anti-La/SSB autoantibodies. It occurs in 2% of anti-Ro/SSA-exposed pregnancies, and recurrence rate is nine times higher in subsequent pregnancies. Aim of this review is to identify biomarkers of CHB and treatment strategies. The Ro-system is constituted by two polypeptides targeted by the anti-Ro52 and anti-Ro60 autoantibodies. The central portion of Ro52 (p200), more than the full amino-acid sequence of Ro-52, is recognized to be the fine specificity of anti-Ro associated to the highest risk of cardiac damage. If anti-p200 antibody should be tested, as biomarker of CHB, over standard commercial ELISAs is still debated. Recent studies indicate that type I-Interferon (IFN) can activate fibroblasts in fetal heart. In the mother the anti-Ro/La antibodies activate the type I IFN-signature, and maternal IFN-regulated genes correlate with a similar neonatal IFN-gene expression. Evaluation of maternal IFN-signature could be used as novel biomarker of CHB. The measurement of “mechanical” PR interval with weekly fetal echocardiogram (ECHO) from 16 to at least 24 weeks of gestation is strongly recommended for CHB prenatal diagnosis. However, ECHO screening presents some limitations due to difficult identification of first-degree block and possible occurrence of a complete block from a normal rhythm in few days. Maternal administration of Hydroxychloroquine from the tenth week of gestation, modulating toll-like receptor and autoantibody-dependent type I IFN activation on the fetus, has an important role in preventing CHB in pregnant women with high risk for recurrent CHB.

Management of pregnant patient with Sjögren syndrome. Case report and literature review

Sjögren’s syndrome, a chronic disease which affects particularly the female gender, represents one of the most common autoimmune diseases and does not affect women of childbearing age, but is especially prevalent during perimenopause. It may be primary when only exocrine glandular dysfunctin occurs (xerophthalmia, xerostomia) or it may be secondary, when is most often associated with rheumatoid arthritis or systemic lupus erythematosus and affects the whole body. Pregnancy cases in the context of this disease are relatively rare, precisely by their incidence in women for whom fertility is not a priority anymore, at the age of 40 or 50 years old, but observing the trend of the last decades, through the implications offered by the modern society, with the increasing age when the first baby is conceived, the number of cases and associated outcome increase. The pregnancy outcome in the context of this disease is insufficiently known comparing to the one appearing in women affected by other autoimmune disorders. The most fearfull complication is represented by the fetal risk of congenital heart block. We report the case of a 29-year-old patient diagnosed with primary Sjögren’s syndrome, consistent with AECG criteria (American-European Consensus Group, 2002) prior to pregnancy, which required a multidisciplinary approach and a close cooperation between the obstetrician, rheumatologist, ophthalmologist and internal medicine physicians. During the 36th week of pregnancy she delivered by caesarean section. Both the patient and the newborn postoperative evolutions were favorable, with no evidence of fetal heart complications. Taking into account the potential maternal and fetal complications, the management of pregnant women diagnosed with Sjögren’s syndrome represents a real challenge for the obstetrician and requires an interdisciplinary approach in order to decrease the morbidity and mortality, both of the mother, as well as the fetus.

Brief Report: Association of Natural Killer Cell Ligand Polymorphism HLA–C Asn80Lys With the Development of Anti‐SSA/Ro–Associated Congenital Heart Block

Fetal exposure to maternal anti-SSA/Ro antibodies is necessary but not sufficient for the development of autoimmune congenital heart block (CHB), suggesting that other factors, such as fetal genetic predisposition, are important. Given the previously described association between major histocompatibility complex alleles and CHB risk, we undertook the present study to test the hypothesis that a variant form of HLA-C Asn80Lys, which binds with high affinity to an inhibitory killer cell immunoglobulin-like receptor (KIR) and thus renders natural killer (NK) cells incapable of restricting inflammation, contributes to the development of CHB. Members of 192 pedigrees in the US and Europe (194 cases of CHB, 91 unaffected siblings, 152 fathers, 167 mothers) and 1,073 out-of-study controls were genotyped on the Immunochip single-nucleotide polymorphism microarray. Imputation was used to identify associations at HLA-C Asn80Lys (Asn, C1; Lys, C2) and KIR. Tests for association were performed using logistic regression. McNemar's test and the pedigree disequilibrium test (PDT) were used for matched analyses between affected and unaffected children. Compared with out-of-study controls of the same sex, the C2 allele was less frequent in the mothers (odds ratio [OR] 0.63, P = 0.0014) and more frequent in the fathers (OR 1.40, P = 0.0123), yielding a significant sex-by-C2 interaction (P = 0.0002). The C2 allele was more frequent in affected siblings than in unaffected siblings (OR 3.67, P = 0.0025), which was consistent with the PDT results (P = 0.016); these results were observed in both sexes and across the US and European cohorts. There was no difference in the frequency of the inhibitory KIR genotype (KIR AA) between affected and unaffected children (P = 0.55). These data establish C2 as a novel genetic risk factor associated with CHB. This observation supports a model in which fetuses with C2 ligand expression and maternal anti-SSA/Ro positivity may have impaired NK cell surveillance, resulting in unchecked cardiac inflammation and scarring.

Environmental and lifestyle factors influencing risk of congenital heart block during pregnancy in anti-Ro/SSA-positive women

ObjectivesCongenital heart block (CHB) occurs in 1%–2% of anti-Ro/SSA antibody-positive pregnancies. A population-based recurrence rate of 12% indicates that factors other than maternal autoantibodies influence CHB development. Here we report...

Factors influencing fetal cardiac conduction in anti-Ro/SSA-positive pregnancies

Congenital heart block (CHB) develops in 1-2% of anti-Ro/SSA-positive pregnancies and has a recurrence rate of 12-20%, which indicates that factors other than maternal autoantibodies are crucial for CHB to occur. Here, we aimed to evaluate the influence of factors previously associated with CHB on the occurrence of milder forms of fetal cardiac conduction disturbances, shown to occur in up to 30% of anti-Ro/SSA-positive pregnancies, and on neonatal outcome in a large cohort of prospectively followed pregnancies. The association of maternal age, season of the year and history of atrioventricular block (AVB) with the development of fetal Doppler and neonatal ECG conduction disturbances was evaluated in 212 anti-Ro52/SSA-positive singleton pregnancies. Maternal age was significantly higher in AVB II-III pregnancies but was not correlated with fetal AV time intervals in fetuses without signs of AVB II-III. AV time intervals of fetuses surveilled during the winter were significantly longer than those of fetuses surveilled during the summer. Fetal AV time intervals in consecutive pregnancies from the same women were significantly correlated. A history of AVB II-III was associated with significantly longer AV time intervals, and AVB I-III was observed at birth in 38% of babies born after a sibling with abnormal fetal AV conduction. Our study shows that AV time intervals in anti-Ro/SSA antibody-exposed fetuses during the CHB risk period are influenced by the season of the year, and reveals that the recurrence of conduction disturbances in antibody-exposed fetuses is higher than previously reported when milder forms are taken into account.

Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with a high prevalence in females of childbearing age. Pregnancy in SLE nowadays has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, and preterm birth are well-known risks in such pregnancies. Anti-SS-A(Ro)/SS-B(La) antibodies put fetuses at risk for congenital heart block and neonatal lupus. Several risk factors for adverse pregnancy outcomes have been identified. Women with antiphospholipid antibodies or antiphospholipid syndrome and lupus nephritis represent a group with high risk for obstetric complications. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, and intensive surveillance during and after pregnancy are essential to improve pregnancy outcome. The aim of this review article is to update on the medical care of pregnancy in these women to ensure the best maternal and fetal prognosis.

Pregnancy and primary Sjögren’s syndrome: management and outcomes in a multicentre retrospective study of 54 pregnancies

Objectives: Primary Sjögren’s syndrome (pSS) is one of the most common autoimmune diseases, mainly affecting women during the fourth decade of life. During pregnancy, the presence of anti-Ro/SSa and anti-La/SSb antibodies increases the risk of congenital heart block (CHB). Foetal and pregnancy outcomes in pregnant women with pSS compared with the general population are difficult to evaluate because of confounding factors including age and body mass index (BMI).Method: The aim of this case–control study was to analyse the impact of pSS in pregnant women on foetal and pregnancy outcomes.Results: We enrolled 19 women with pSS (54 pregnancies) matched by age and BMI to 216 controls. Patients with pSS delivered significantly earlier (38 weeks + 3 days vs. 39 weeks + 2 days) and experienced more spontaneous abortions [< 22 weeks of gestation (WG)] than the controls [n = 16/54 (30.0%) vs. n = 1/216 (0.4%); p < 0.00001]. Preterm delivery (≤ 37+6 WG) was significantly higher in the pSS group than in the control group (29% vs. 12%, p = 0.04). pSS activity significantly affected the birthweight percentile, which was lower in pregnancies occurring after the diagnosis of pSS than in those occurring before (32.43 ± 21.57 vs. 60.46 ± 27.37; p = 0.008). No case of CHB was observed.Conclusions: pSS is responsible for an increased risk of spontaneous abortion. The duration of pregnancy is lower in patients with than without pSS, with more premature deliveries. Pregnancies that occur after the onset of the disease result in lower birthweight percentile children than when pSS is not clinically overt.

Autoimmune congenital heart block: complex and unusual situations.

Autoimmune congenital heart block (ACHB) is an immune-mediated cardiac disease included among the manifestations collectively referred to as neonatal lupus. The placental transference of maternal Ro/La autoantibodies may damage the conduction tissues during fetal development leading to blocking of signal conduction at the atrioventricular (AV) node in an otherwise structurally normal heart. Irreversible complete AV block is the main cardiac manifestation of ACHB, but some babies may develop endocardial fibroelastosis, valvular insufficiency, and/or frank cardiomyopathies with significantly reduced cardiac function requiring transplant. The severity of ACHB is illustrated by a global mortality rate of 20% and pacemaker rates of at least 64%, often within the first year of life. This review analyses the main complex and/or unusual clinical situations associated with ACHB, including unusual maternal immunological profiles, infrequent maternal autoimmune diseases, cardiac damage unrelated to AV block, fetal invasive management, late complications after birth, risk of congenital heart block (CHB) in ovodonation and in vitro fertilization techniques, the role of maternal features other than autoimmunity, the influence of the birth order or the risk of CHB in twins and triplets.

Anti-Ro/SSA autoantibody-positive women's experience of information given on the risk of congenital heart block.

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. 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). 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. 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.

Maternal predictive factors for fetal congenital heart block in pregnant mothers positive for anti-SS-A antibodies

Objective: To determine the maternal predictive factors for fetal congenital heart block (CHB) in pregnancy in mothers positive for anti-SS-A antibodies.Methods: The Research Team for Surveillance of Autoantibody-Exposed Fetuses and Treatment of Neonatal Lupus Erythematosus, the Research Program of the Japan Ministry of Health, Labor and Welfare, performed a national survey on pregnancy of mothers positive for anti-SS-A antibodies. We analyzed 635 pregnant mothers who tested positive for anti-SS-A antibodies before conception but had no previous history of fetal CHB. We performed univariate and multivariate analysis (models 1, 2, and 3 using different set of independent variables) investigated the relation between risk of fetal CHB and maternal clinical features.Results: Of the 635 pregnant mothers, fetal CHB was detected in 16. Univariate analysis showed that fetal CHB associated with use of corticosteroids before conception (OR 3.72, p = 0.04), and negatively with use of corticosteroids (equivalent doses of prednisolone (PSL), at ≥10 mg/day) after conception before 16-week gestation (OR 0.17, p = 0.03). In multivariate analysis, model 1 identified the use of corticosteroids before conception (OR 4.28, p = 0.04) and high titer of anti-SS-A antibodies (OR 3.58, p = 0.02) as independent and significant risk factors, and model 3 identified use of corticosteroids (equivalent doses of PSL, at ≥10 mg/day) after conception before 16-week gestation as independent protective factor against the development of fetal CHB (OR 0.16, p = 0.03). Other maternal clinical features did not influence the development of fetal CHB.Conclusion: The results identified high titers of anti-SS-A antibodies and use of corticosteroids before conception as independent risk factors, and use of corticosteroids (equivalent doses of PSL, at ≥10 mg/day) after conception before 16-week gestation as an independent protective factor for fetal CHB.

Ro/SSA autoantibody-positive pregnancy: reactions to serial fetal Doppler echocardiographic surveillance.

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. 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). 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. Fetal echocardiographic surveillance holds many and predominantly positive effects for Ro/SSA-positive women during pregnancy in addition to the medical advantages.

Reactivity to the p305 Epitope of the α1G T-Type Calcium Channel and Autoimmune-Associated Congenital Heart Block.

BackgroundOnly 2% of mothers positive for anti-SSA/Ro (Ro) antibodies have children with congenital heart block (CHB). This study aimed to determine whether reactivity with p305, an epitope within the α1G T-type calcium channel, confers added risk over anti-Ro antibodies.Methods and ResultsUsing sera from anti-Ro-exposed pregnancies resulting in offspring with CHB, no disease but CHB-sibling, and no disease and no CHB-sibling, as well as disease (lupus without anti-Ro) and healthy controls, reactivities were determined for binding to Ro60, p305, and an epitope within Ro60, p133-Ro60, which shares structural properties with p305, including key amino acids and an α-helical structure. Candidate peptides were further evaluated in an in vitro model that assessed the binding of maternal antibodies to apoptotic cells. In anti-Ro-positive mothers, anti-p305 autoantibodies (>3 SD above healthy controls) were detected in 3/59 (5%) CHB pregnancies, 4/30 (13%) unaffected pregnancies with a CHB-sibling, and 0/42 (0%) of unaffected pregnancies with no CHB-sibling. For umbilical bloods (61 CHB, 41 healthy with CHB sibling), no association of anti-p305 with outcome was detected; however, overall levels of anti-p305 were elevated compared to mothers during pregnancy in all groups studied. For anti-p133-Ro60, reactivity paralleled that of anti-p305. In the screen employing apoptotic cells, p133-Ro60, but not p305, significantly attenuated the binding of immunoglobulin G isolated from a mother whose child had CHB (42.1% reduced to 13.9%, absence/presence of p133-Ro60, respectively, P<0.05).ConclusionsThese data suggest that anti-p305 is not a robust maternal marker for assessing increased risk of CHB during an anti-SSA/Ro pregnancy.

« Lupus néonatal » : revue de la littérature

Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.

Preventing congenital neonatal heart block in offspring of mothers with anti-ssa/ro and anti-SSB/La antibodies: review of literature and registered trials

Since offspring of women with anti-SSA/Ro - SSB/La antibodies are believed at risk for congenital heart block (CHB), and since it is unknown whether this risk can be reduced, to review current knowledge of what constitutes standard of care treatment.

Pregnancy outcome in anti ro positive women – outcomes of a targeted approach

Anti Ro antibodies are relatively common and may be detected in up to 1–2% of randomly tested pregnant women. 50% of these women are otherwise asymptomatic and the remaining may...

Lupus eritematoso neonatal

Neonatal lupus erythematosus (NLE) is an uncommon disease caused by transport of maternal autoantibodies against Ro, La and/or ribonucleoprotein (RNP) into the fetal circulation. These IgG antibodies cross the placenta and can potentially damage fetal tissue and cause the clinical manifestations of NLE. NLE is more common in girls, has no racial association and can affect multiple organs. Cutaneous manifestations are present in 50% of patients and are clinically similar to the lesions of subacute lupus erythematosus. Patients with NLE have a higher risk of congenital heart block, a potentially fatal complication. Other, less frequent manifestations are hepatic and hematologic. Approximately half of all patients have cutaneous manifestations and the other half cardiac manifestations. Approximately 10% of patients have both cutaneous and cardiac manifestations. Cutaneous, hematological and hepatic manifestations are transient, healing at 6 months of age, due to the clearance of maternal autoantibodies. Congenital heart block is a permanent manifestation that usually requires pacemaker implantation. Pregnant women with autoimmune diseases or anti-Ro or anti-La antibodies should be followed-up by ultrasound during pregnancy to detect any manifestations susceptible to treatment. Currently, there is no consensus on preventive treatment in high-risk patients or in those with a risk of recurrence. Equally, there is insufficient data to confirm the efficacy of currently available preventive treatments. What seems clear is that early detection of congenital heart block should be treated with fluorinated corticosteroids.