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

Background 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.

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.

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.

Influence of season of birth and maternal age in the development of congenital heart block in anti-Ro-SSA/La-SSB positive pregnancies

Congenital heart block (CHB) is a conduction abnormality that affects hearts of foetuses and/or newborn to mothers with autoantibodies reactive with the intracellular soluble ribonucleoproteins 48-kD La, 52-kD Ro and 60-kD Ro. CHB carries substantial mortality and morbidity, with more than 60% of affected children requiring lifelong pacemakers. Several hypotheses have been proposed to explain the pathogenesis of CHB. These can be grouped under three main hypotheses: Apoptosis, Serotoninergic and Ca channel hypothesis. Here, we discuss these hypotheses and provide recent scientific thinking that will most likely dominate the future of this field of research.

Congenital heart block is the most severe manifestation of neonatal lupus syndrome. It is a passively acquired disease where transplacental passage of maternal autoantibodies is associated with irreversible damage of the foetal cardiac conduction system. It is well established that the condition, in the absence of structural abnormalities, is strongly associated with maternal autoantibodies to the Ro/La antigens. More specifically the disease has been closely linked to antibodies to the Ro52 component of the antigen complex. Congenital heart block constitutes a unique model where specific autoantibodies target and mediate organ-specific disease. A wide panel of maternal antibodies has been discussed in literature in association with the disease and are described in this review.

The increasing data suggest an association between chronic right ventricular (RV) and left ventricular (LV) dysfunction. We sought to determine the effect of temporary interruption of long-term RV pacing on LV function and mechanical dyssynchrony in children and young adults with complete heart block.Twelve patients aged 20.0 + or - 7.4 years with congenital heart block (group I) and six patients aged 22.7 + or - 11.0 years with surgically acquired heart block (group II) with RV pacing were studied. The pacing rate was reduced to less than patient's intrinsic heart rate and maintained for 5 minutes. The LV ejection fraction (EF), three-dimensional systolic dyssynchrony index (SDI), two-dimensional global longitudinal strain and strain rate, and Doppler-derived isovolumic acceleration before and after interruption of RV pacing were compared.The LVEF and GLS increased while QRS duration decreased after the pacing interruption in both the groups (all P0.05). While SDI decreased in both groups I (6.8 + or - 2.3%- 3.8 + or - 0.8%, P = 0.001) and II (9.2 + or - 4.1%-5.0 + or - 1.6%, P = 0.032), it remained higher in group II than in group I (P = 0.046) after the pacing interruption. The prevalence of LV dyssynchrony (SDI4.7%) decreased in group I (83%-25%, P = 0.006) but not in group II (67%-50%, P = 0.50). The %increase in LVEF correlated positively with %reduction of LV SDI (r = 0.80, P = 0.001).Temporary interruption of chronic RV pacing acutely improves LV dyssynchrony and systolic function in children and young adults, the magnitude of which is greater in patients with congenital than those with surgically acquired heart block.

When a woman is known to have systemic lupus erythematosis, planning and preparation can improve the outcome of pregnancy and reduce the severity of some of the potential problems. All treatment options have risks but these risks have to be balanced against the risks of no treatment or, ‘too late’ treatment. In pregnancy, close fetal surveillance is important. Once there are definite signs of fetal harm this cannot always be corrected and late management decisions can only institute rescue. Urgent premature delivery is a frequent phenomenon. Proactive medical care of the mother, together with accurate information about the state of the baby, may reduce the frequency of sudden or early delivery thereby assisting neonatal management. Currently, some risks such as neonatal lupus with congenital heart block, retain a high mortality. The construction of an agreed detailed prospective management plan, understood by all those involved, can lead to increased satisfaction with the care process and a better outcome.

Persistent fetal bradycardia noted in the antenatal period can occur secondary to maternal conditions, fetal cardiac structural defects, or from congenital heart block. Fetal bradycardia can be mistaken for maternal pulse and should be confirmed with ultrasound whenever possible. Prompt evaluation of the fetus with bradycardia can lead to early interventions designed to prevent cardiac damage and/or hydrops.

Congenital heart block (CHB) has been linked with Sjogren's Syndrome. This paper reports a case of previously undiagnosed maternal Primary Sjogren's Syndrome (1 degrees SS) that was only discovered following the birth of the patient's first child with CHB. The possible pathophysiological mechanisms underlying CHB associated with 1 degrees SS are discussed.

In this work we demonstrated that IgG present in the sera of patients with congenital heart block (CHB) and their mothers could bind and activate beta adrenoceptors and muscarinic cholinergic receptors of neonatal heart. These antibodies were able to inhibit in a noncompetitive manner the binding of [3H]QNB and [3H]CGP to muscarinic cholinergic and beta adrenoceptors of purified neonatal rat myocardial membranes, respectively. Moreover, IgG from children with CHB and their mothers could modify biological effects mediated by these neurotransmitter receptors, i.e., heart contractility and cAMP production. Neither binding nor biological effects were obtained using adult instead of neonatal rat atria. Both reactivities (adrenergic and cholinergic) were independent of Ro/SS-A and La/SS-B antibodies and were absent in the sera of normal women of childbearing age and of normal children. It could be concluded that antibodies against cardiac neurotransmitter receptors may be another serum factor (or factors) to be considered in the pathophysiology of the development of CHB.

Electrical instability of the heart has numerous congenital origins. This review approaches the problems from a morphological standpoint, utilizing selected examples from my own studies of the human cardiac conduction system performed during the last three decades. Subjects discussed include multifocal Purkinje cell tumors, benign congenital polycystic tumors of the AV node, several types of congenital heart block, postnatal morphogenesis of the AV node and His bundle (including considerations of persistent fetal dispersion and crib death), the Wolff-Parkinson-White syndrome, left superior vena cava, focal fibromuscular dysplasia of small coronary arteries, hereditary neuromuscular or musculoskeletal diseases, familial atrial fibrillation, long QT syndrome, and apoptosis of the heart. In many congenital disorders of cardiac electrical activity, both arrhythmias and conduction disturbances may occur. How the abnormal anatomy may relate to cardiac electrical instability is discussed, including certain clinical matters to be considered.

Summary: We reviewed the obstetrical performance and outcome of 15 pregnancies in patients with systemic lupus erythematosus (SLE) (study group) and compared them with 45 age and parity-matched normal pregnancies (control group). Eleven women (73.8%) were in remission phase and 4 (26.7%) had active disease at the time of conception. The time interval between disease diagnosis and the index pregnancy was 4.2 ± 2.5 years. Two patients with renal involvement had lupus flare-up during the antenatal period. There was no case of lupus flare-up in the postpartum period. Gestational age at delivery was significantly lower in SLE patients (35.9 ± 2.5 weeks) compared to the control group (37.4 ± 2.2 weeks). The incidence of intrauterine growth retardation was significantly higher in the SLE patients (40%). There was no case of neonatal lupus or congenital heart block.

Objective To analyze the electrocardiograms (EKGs) of infants born to mothers with anti-SSA/Ro antibodies in order to evaluate the QT interval (the time from the beginning of the QRS complex to the end of the T wave). Methods Sera from mothers and children were analyzed for anti-Ro and anti-SSB/La antibodies by enzyme-linked immunosorbent assay (ELISA) and by Western blot analysis. Fine specificity of anti-Ro antibodies was evaluated by solid-phase ELISA against recombinant 52- and 60-kd proteins and by Western blot. A retrospective chart review was conducted for EKG analysis. Twenty-eight EKG tracings (21 from anti-Ro–positive and 7 from anti-Ro–negative infants born to mothers with autoimmune diseases) were analyzed by a single investigator who was blinded to the infant's antibody status. The QT interval was measured and corrected for heart rate according to Bazett's formula. Results The mean QT interval was significantly longer in anti-Ro–positive than in anti-Ro–negative infants, also after correction for heart rate (QTc) (P = 0.001). Nine of 21 anti-Ro–positive infants and 0 of 7 anti-Ro–negative infants had QTc values above the upper normal limit (440 msec). A 24-hour EKG recording was performed on 5 patients and confirmed the QT prolongation. These infants were subsequently treated with a beta-blocker in order to prevent arrhythmias. Conclusion Infants born to mothers who carry anti-Ro autoantibodies may show QT interval prolongation and should be monitored with EKG during the first months of life.

A baby with congenital heart block and hydrops fetalis diagnosed prenatally was delivered at 36 weeks of gestation. Heart failure was controlled with temporary cardiac pacing which was complicated by perforation of the myocardium. She made an uneventful recovery after implantation of a permanent pacemaker. Sjögren's syndrome A antibodies were detected in her serum. Coincidentally, she also had pulmonary valvular stenosis as a structural abnormality.

This clinical review details our 15 year experience with permanent cardiac pacemakers in 81 infants and chiJdren. Pacing was found inappropriate in one infant. The other 80 patients were paced because of congenital heart block (24), post-operative block (50), or sick sinus syndrome (6). Maintenance of long-term pacing requires all too frequent re-operation for battery depletion (37%), lead related problems (32%) or wound dehiscence (31 %). Problems related to pacemaker size and the presence of a high myocardial threshold are particularly important in (he pediatric patient. In spite of these problems, children requiring cardiac pacemakers can be extremely well, their prognosis depending almost entirely on the presence of underlying heart disease.

Infection remains a significant problem in patients undergoing permanent cardiac pacemaker implantation, and removal of all components is usually required. The transvenous system carries minimal morbidity at implantation, but the development of infection is more life-threatening than in the epicardial system. Although the evolution of the tined porous endocardial lead reduced the incidence of wire displacement, the development of a serious infection is still a problem and may require major surgery for removal. We experienced this problem in a 15-month-old child who developed recurrent sepsis. Attempts at removal of the retained tined porous electrode through the neck incision proved dangerous and unsuccessful. Removal was carried out using cardiopulmonary bypass and infection was promptly controlled.

Summary A female patient aged 14 years with known congenital heart block presented for appendicectomy. The successful management under general anaesthesia without use of a pacemaker is described. Alternative methods including local anaesthesia are discussed.