Congenital Complete Heart Block Research Articles

Epicardial pacing outcomes in infants with heart block: Lead and device complications from a multicenter experience

BackgroundInfants with complete heart block (CHB) require epicardial pacemaker (PM) insertion. Prior studies described epicardial pacing outcomes in infants and children, although they were limited by small or heterogeneous populations. ObjectiveThis study aimed to explore patient- and procedure-level associations with device complications in infants with CHB who received a permanent PM. MethodsThis was a multicenter, retrospective cohort study including infants receiving an epicardial PM between 2000 and 2021 for CHB. The primary outcome was time to device-related adverse event: lead failure requiring revision; pocket infection; exit block requiring increased pacing output; or lead-related coronary artery compression. Time-to-event analysis was performed by the Kaplan-Meier method with a multivariable Cox proportional hazards model. ResultsThere were 174 infants who received an epicardial PM (282 bipolar, 39 unipolar leads) for CHB. Median age and weight at PM were 93.5 days and 4.5 kg, respectively. Pacing indication was postoperative CHB in 63% and congenital CHB in 37%. The median follow-up was 2.1 years. The primary outcome occurred in 26 infants at a median time to event of 0.6 year. Age ≤90 days at PM implantation was the most significant risk factor for a device-related adverse event (hazard ratio, 7.02; P < .001), primarily driven by pocket infections. Lead failure occurred in 3% of leads with a 5- and 10-year freedom from failure of 93% and 83%, respectively. ConclusionDevice complications affect 15% of infants receiving a permanent PM for heart block. Age ≤90 days at PM implantation is especially associated with infectious complications. Epicardial lead durability appears similar to previously reported pediatric experiences.

Newborns with congenital complete heart block: Advice for perinatal care providers.

Congenital complete heart block (CCHB) is a rare, but a potentially life-threatening manifestation of autoimmune diseases in neonates. Bradycardia in CCHB can be misdiagnosed as foetal distress in utero and thus precipitating a Caesarean section. We report a case series of three neonates with bradycardia without any electrolyte abnormalities and structurally normal hearts with favourable outcomes.

Congenital complete heart block in a neonate-not always maternal lupus

Congenital complete heart block (CCHB) is a rare cardiac disorder in neonates and maternal lupus is the most common cause. More rarely, CCHB can be associated with congenitally corrected transposition of the great arteries. In this case, the neonate was born via emergency caesarean section due to bradycardia. Post-natal electrocardiogram and echocardiography confirmed the diagnosis. A pacemaker was inserted and the baby was kept under follow-up.

Ventricular dysfunction after permanent pacemaker implantation in children - what are the implications?

Abstract Introduction Children (&amp;lt;18 years of age) account for less than 1% of patients requiring permanent pacemaker implantation (PPI), but form a complex subset of population due to technical difficulties, higher prevalence of structural abnormalities and higher rates of reinterventions. Post-pacemaker ventricular dysfunction is seen in around 10-15% and is related to the electrical and mechanical ventricular dyssynchrony. However, similar data is lacking from patients who underwent permanent pacemaker implantation below 18 years of age. Methods We retrospectively analysed 235 patients who underwent PPI below 18 years of age, for development of ventricular systolic dysfunction, defined by ejection fraction below 50% of the systemic ventricle. Results Male: female ratio was 1.1:1, with median age of implantation being 7 years. 43.4% underwent epicardial PPI, and the commonest indication being congenital complete heart block (50.2%) followed by post-operative CHB (28.1%). 65.5% underwent VVI pacemaker implantation. 41.3% had associated congenital heart disease out of which ventricular septal defect was commonest (20.6%) followed by atrial septal defect (18.6%). Over a median follow-up of 8 years, 15 (6.38%) patients developed ventricular dysfunction, with systemic ventricle of RV morphology, younger age at implantation, maternal ANA positivity, presence of post-operative CHB, associated congenital heart disease and epicardial PPI being major predictors. Five (2.1%) patients required hospitalization for heart failure, while 2 underwent upgradation to CRT. Those with ventricular dysfunction had higher mortality than those who had normal ventricular dysfunction (40% vs 1.5%, p &amp;lt;0.01). There was no association of ventricular dysfunction with the duration of pacing (p = 0.25), the percentage of pacing (p = 0.25) or number of chambers paced (p = 0.38). Conclusion Ventricular dysfunction is seen in 6.38% of patients who underwent PPI below 18 years of age, over a median duration of 8 years. It was associated with higher mortality, while younger age at implantation, maternal ANA positivity, presence of post-operative CHB, associated congenital heart disease and epicardial PPI were major predictors for development of ventricular dysfunction.

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.

Left Bundle Branch Pacing in a Child Who Survived Cardiac Arrest with Congenital Complete Heart Block and Recurrent Torsades de Pointes

A 2-year-old girl was referred to the Harapan Kita National Heart Center due to the return of spontaneous circulation after cardiac arrest, bradyarrhythmia and repeated seizures with loss of consciousness. She had been treated with an epilepsy drug for 1 year. Electrocardiography during one epilepsy episode showed complete heart block with junctional escape rhythm and QT interval prolongation, which then progressed to torsades de pointes. Echocardiography revealed left atrium and left ventricle dilatation with reduced ejection fraction. Left bundle branch permanent pacemaker was successfully implanted through left subclavian vein access. During the 3-week follow-up, there were no recurrent seizures and echocardiography revealed improvement of left ventricle ejection fraction.

PO-03-126 CONTEMPORARY OUTCOMES IN NEONATES WITH ISOLATED CONGENITAL COMPLETE HEART BLOCK– AN ANALYSIS OF THE PHIS DATABASE

Congenital Complete Heart Block (CCHB) is a rare diagnosis, with limited multicenter outcomes data. Prior reports have observed pacemaker (PM) placement in 69% of fetal diagnosed patients by age 1 and in 74-96% of patients by age 20. Rates of PM placement and mortality in a contemporary CCHB cohort have not been well described. We performed a multicenter retrospective cohort study utilizing the Pediatric Health Information Systems (PHIS) database to analyze time to PM and in-hospital mortality in neonates with isolated CCHB. All subjects in the PHIS database (1/1/2007-12/31/2021) with ICD-9 or ICD-10 diagnosis code for congenital heart block or complete AV block and with age at index hospitalization < 30 days were included. Subjects with lower degree AV block, Long QT Syndrome, or structural congenital heart disease, except patent ductus arteriosus or atrial septal defect, were excluded. Cumulative incidence functions of time to PM were modeled with death as a competing risk. Cause-specific hazard regression analyses of PM placement were performed. There were 691 unique CCHB subjects identified from 47 US hospitals. The cohort was 58% female, 60% white, and 50% privately insured. Mean age at hospitalization was 0.8 days (SD 2.7). Median length of stay of index hospitalization was 11 days (IQR 5-28), and median follow-up duration was 1.7 years (IQR 0.1-6.4). PM placement occurred in 397 (57%) subjects, of whom 308 (45%) received PM in the index hospitalization. Median age at PM was 6 days (IQR 2-58), with 164 (41%) receiving single chamber and 224 (56%) dual chamber PM. In-hospital mortality occurred in 68 (9.8%) subjects. With death as a competing risk, predicted cumulative incidence of PM at 1 year is 50% (95% CI 47-54%). In multivariate analysis, gestational age (GA) 35-36 weeks had HR 1.4 of PM (95% CI 1.2-3.2, p=0.007) compared to term infants. GA ≤ 30 weeks and GA 31-34 weeks had increased hazard of mortality with HR 49 (95% CI 17-147, p<0.005) and 5.9 (95% CI 2-19, p=0.003), respectively. GA ≤ 30 weeks was associated with reduced hazard of PM with HR 0.5 (95% CI 1.4-3.6, p=0.005). In this largest published cohort of neonates with CCHB, predicted PM placement occurred in 50% by 1 year and in-hospital mortality rate was 9.8%. Severe and moderate prematurity are associated with increased mortality, which may explain the reduced risk of PM, whereas late preterm infants have increased risk of PM.Tabled 1Table 1: Characteristics of CCHB SubjectsCharacteristicsDescriptionAll (n=691)Permanent Pacemaker (n=397)No Pacemaker (n=237)Death Without Pacemaker Placement (n=57)P-valueSexMale294 (43%)158 (40%)108 (46%)28 (49%)0.21Female397 (58%)239 (60%)129 (54%)29 (51%)RaceBlack105 (15%)55 (14%)34 (14%)16 (28%)<0.0001White417 (60%)256 (65%)140 (59%)21 (37%)Other125 (18%)67 (17%)49 (21%)9 (16%)Missing44 (6%)19 (5%)14 (6%)11 (19%)Census RegionMidwest217 (31%)131 (33%)73 (31%)13 (23%)0.055Northeast118 (17%)58 (15%)48 (20%)12 (21%)South230 (33%)125 (32%)79 (33%)26 (46%)West126 (18%)83 (21%)37 (16%)6 (11%)Age in days at index hospitalization, mean (SD)0.8 (2.7)0.5 (2.1)0.9 (3.0)2.0 (4.7)0.0008Gestational age, n (%)<= 30 weeks76 (11%)23 (6%)22 (9%)31 (54%)<0.000131-34 weeks115 (17%)76 (19%)32 (14%)7 (12%)35-36 weeks143 (21%)100 (25%)37 (16%)6 (11%)>= 37 weeks269 (39%)146 (37%)117 (49%)6 (11%)Missing88 (13%)52 (13%)29 (12%)7 (12%)Birth weight in grams, mean (SD)2509 (823)2547 (703)2650 (830)1650 (1033)0.949Hydrops Fetalis, n (%)36 (5%)19 (5%)7 (3%)10 (18%)<0.0001Steroid use in index hospitalization, n (%)212 (31%)137 (35%)40 (17%)35 (61%)<0.0001Isoproterenol use in index hospitalization, n (%)269 (39%)197 (50%)41 (17%)31 (54%)<0.0001Length of stay, index hospitalization, median (IQR)11 (5, 28)14 (8, 32)8 (4, 19)5 (1, 16)Mortality in index hospitalization, n (%)68 (9.8%)11 (3%)0 (0%)57 (100%) Open table in a new tab

Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis

Neonatal lupus (NL) is a clinical syndrome that develops in the fetus as a result of maternal autoimmune antibodies. Congenital complete heart block (CHB) is the most common manifestation, while extranodal cardiac manifestations of NL, such as endocardial fibroelastosis (EFE) and myocarditis, are rare but more serious. Less is known about this atrioventricular valve rupture due to valvulitis as a consequence of maternal autoantibodies. We have described a case of cardiac neonatal lupus with an antenatally detected CHB patient who developed mitral and tricuspid valve chordal rupture at 45 days of age. We compared the cardiac histopathology and the fetal cardiac echocardiographic findings of this case with another fetus that was aborted after being antenatally diagnosed with CHB but without valvar rupture. A narrative analysis after a systematic review of the literature regarding atrioventricular valve apparatus rupture due to autoimmune etiology along with maternal characteristics, presentation, treatment, and outcome have been discussed in this article. To describe published data on atrioventricular valve rupture in neonatal lupus, including clinical presentation, diagnostic evaluation, management, and outcomes. We conducted a PRISMA-compliant descriptive systematic examination of case reports that included accounts of lupus during pregnancy or in the newborn period that resulted in an atrioventricular valve rupture. We gathered information on the patient's demographics, the details of the valve rupture and other comorbidities, the maternal therapy, the clinical course, and the results. We also used a standardized method to evaluate the cases' quality. A total of 12 cases were investigated, with 11 cases drawn from 10 case reports or case series and 1 from our own experience. Tricuspid valve rupture (50%) is more common than mitral valve rupture (17%). Unlike mitral valve rupture, which occurs postnatally, the timing of tricuspid valve rupture is perinatal. A total of 33% of the patients had concomitant complete heart block, while 75% of the patients had endocardial fibroelastosis on an antenatal ultrasound. Antenatal changes pertaining to endocardial fibroelastosis can be seen as early as 19 weeks of gestation. Patients with both valve ruptures generally have a poor prognosis, especially if they occur at close intervals. Atrioventricular valve rupture in neonatal lupus is rare. A majority of patients with valve rupture had antenatally detected endocardial fibroelastosis in the valvar apparatus. Appropriate and expedited surgical repair of ruptured atrioventricular valves is feasible and has a low mortality risk. Rupture of both atrioventricular valves occurring at close intervals carries a high mortality risk.

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.

Reversible Complete Heart Block in a Pregnant Woman Responding to Steroids: A rare Case Report

Background: Complete heart block (CHB) is the total absence of conduction from atria to ventricles with an escape rhythm most commonly arising from the AVN or His bundle. CHB in pregnancy is very rare, and most reported cases are due to congenital variety, where the pre-existing CHB from birth is either detected incidentally or presented with symptoms during pregnancy. Pregnancy is associated with various physiological changes, such as a pro-inflammatory, hypercoagulable state with possible oedema of the heart's conduction system, which may rarely give rise to CHB. There are no clear guidelines to manage CHB complicating pregnancy. Case Presentation:: An asymptomatic 27-year-old lady with G2P1L1, 39 weeks of gestation with breech presentation, was referred for delivery given the low maternal heart rate of 40 bpm. Per abdominal examination revealed a term size uterus with active contractions and a fetal heart rate of 140bpm. An ECG revealed a CHB with an escape rate of 40 bpm. Other routine investigations were normal, with negative serological evaluation for hepatitis B, C, and HIV. ANA and Anti-Ro ⁄ SSA antibodies. She underwent an emergency LSCS under spinal anaesthesia with a backup temporary pacemaker (TPM). She received a short course of empirical steroid therapy for three days as there were no obvious secondary causes for CHB, which reverted to sinus rhythm after 48 hours. Discussion: CHB complicating pregnancy with or without symptoms may require definitive therapy in the form of permanent pacemaker implantation. However, the insertion of a TPM for managing pregnancy is controversial as opinion in the literature is divided. There are no reports of CHB occurring in pregnancy without congenital or other known acquired causes. In the reported case, the CHB reverted to sinus rhythm with a short course of IV steroid therapy without any subsequent need for a PPM implantation. Conclusion: Congenital CHB remains the most common cause of rarely seen CHB complicating pregnancy. Through the conduction system oedema and inflammation, physiological changes in gestation may rarely cause CHB during pregnancy. In the absence of congenital CHB and other demonstrable acquired causes, a short course of steroid therapy may reverse the CHB avoiding PPM implantation as shown in the reported case.

Complete Congenital Heart Block in a Newborn Associated with Maternal Systemic Lupus Erythematosus: A Case Report

Background: Complete Congenital Heart Block (CCHB) is an uncommon life threatening disease of the newborn which leads to significant neonatal morbidity and mortality. It is usually diagnosed early in life and highly associated with maternal autoimmune and connective tissue disorders. CCHB frequently presents with bradycardia which can be diagnosed by fetal electrocardiogram. Results: This is a case report of a male neonate born with a third degree heart block to a mother with high Ro/SSA titer. Conclusion: Neonates born to women with SLE have an increased risk of CHD, and an increased risk of having a CHD repair procedure later on. It is important to diagnose, treat or refer neonates with CCHB early for definite management with pacemaker insertion as it helps for a better prognosis and prevention of associated complications, but this is not often possible in low resource settings lie Ethiopia. Keywords: Congenital Heart Block, Systemic Lupus Erythematosus, Bradycardia, SS-A/Ro Antibodies

Complete Congenital Heart Block in the Offspring of an Asymptomatic Woman with Isolated High Titer Anti-Ro Antibody

Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissuedisease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heartblock in the child.We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetalbradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (&gt;320 U/ml).Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well.While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Neonatal Lupus Erythematosus with Congenital Complete Heart Block in an Asymptomatic Mother

Neonatal Lupus Erythematosus with Congenital Complete Heart Block in an Asymptomatic Mother

Incidence of morbidity and mortality in a cohort of congenital complete heart block patients followed over 40 years.

Congenital complete heart block (CCHB) is seen in 1:15,000-20,000 births and commonly requires pacemaker placement by young adulthood. There is limited understanding of cardiac morbidity and mortality. The purpose of this study was to determine the long-term incidence of cardiac morbidity and mortality in subjects with CCHB and identify associated risk factors. Retrospective cohort analysis of subjects with CCHB at Children's Hospital of Philadelphia between 1976 and 2018 was performed. The primary outcome was a composite of death, left ventricular systolic dysfunction, heart failure, cardiomyopathy, or cardiac resynchronization therapy (CRT). Cox proportional hazard models assessed independent risk factors for the primary outcome and its components (death, heart failure and/or cardiomyopathy, CRT). One-hundred fourteen subjects (58% female; median age at last visit 15.2 years) were included. Eighty-eight (77%) underwent pacemaker implantation (median age at placement 1.9 years; interquartile range [IQR] 0.1-8.0 years). Twenty-six subjects (23%) reached the primary outcome; 7 (6%) died and 14 (12%) were diagnosed with heart failure and/or cardiomyopathy. Median time from diagnosis to primary outcome was 3.1 years (IQR 0.0-10.8 years). There were no significant associations between age at diagnosis <1 year (hazard ratio [HR] 1.5; 95% confidence interval [CI] 0.6-3.9), fetal diagnosis (HR 2.3; 95% CI 0.96-5.6), or maternal antibody positivity (HR 2.4; 95% CI 0.9-6.6) and the primary outcome. Fetal diagnosis had a higher associated hazard of heart failure and/or cardiomyopathy (HR 4.5; 95% CI 1.3-15.0). In 114 subjects with CCHB, 23% reached the composite outcome of cardiac morbidity and mortality, with no significant association between age at diagnosis, fetal diagnosis, and maternal antibody status with composite cardiac morbidity and mortality.

Transvenous laser lead extraction in patients with congenital complete heart block.

Data on lead management in patients with congenital complete heart block (CCHB) with cardiac implantable electronic devices are lacking. The purpose of this study was to describe the natural history and outcomes in patients with CCHB with cardiac implantable electronic devices undergoing transvenous lead extraction (TLE). Data on all attempted TLE procedures in patients with CCHB at 2 institutions between 2011 and 2021 were collected from a retrospective registry. Overall, 16 patients (mean age at transvenous device implant 13.8 ± 4.7 years) were included. Before TLE, patients underwent an average of 2.25 ± 1.3 generator changes, 3 (19%) underwent cardiac resynchronization therapy upgrade, and 7 (44%) underwent a lead revision with subsequently abandoned leads. Mean patient age at TLE was 34.4 ± 9.4 years with a mean duration of lead implant of 19.2 ± 6.9 years. Lead malfunction (n = 11 [69%]) and infection (n = 5 [31%]) were the most common indications for TLE. A total of 38 leads were removed, with complete procedural success achieved in 14 of 16 (87.5%). Two (12.5%) major complications occurred, including right ventricular laceration and superior vena cava tear requiring sternotomies. All patients survived at 1-year follow-up. Patients with CCHB represent a unique cohort highlighted by several generator changes, lead revisions, and abandoned leads at a young age, along with a long duration of lead dwelling time and a high prevalence of lead malfunction requiring TLE. There may be a high risk of major complications during TLE, suggesting TLE should be performed only in experienced centers. Larger studies are needed to confirm these findings.

Conduction System Pacing in Pediatrics and Congenital Heart Disease, a Single Center Series of 24 Patients.

His-bundle pacing has demonstrated feasibility in numerous adult studies to reverse and prevent pacing-induced cardiomyopathy, however, is met with higher capture thresholds with deployment sheaths designed for adults with his-bundles in the typical location. To describe 24 pediatric and adult congenital patients post-physiologic pacing. Patients at the University of Minnesota Masonic Children's Hospital with congenital complete heart block or congenital heart disease and atrioventricular block presented for pacemaker placement between November 2019 and January 2021. Twenty-four patients had attempted his-bundle placement using either Medtronic's C315 or C308 sheaths and 3830 leads except for 3 patients who had Boston Scientific's His system with the Shape 3 sheath and 7842 leads. Twenty-four total patients underwent physiologic pacing (23 his-bundle, 13 female, 11 male) with median age of 14years (range 8-39years) with median weight of 51kg (range 21.2-81kg) with five right-sided implants performed. Twelve patients had congenital heart disease including atrioventricular canal defects, tetralogy of Fallot, and ventricular septal defect repairs (nine patients with ventricular septal defect repairs). Twelve patients had selective His-bundle pacing (six with congenital heart disease). Median threshold to capture was 0.5V at 0.4ms (range 0.4 to 1.1V at 0.4ms), impedance 570ohms (range 456-1140ohms), and sensing median of 9.7mV (range 1.5-13.8mV if present). The median follow-up time was 610days (range 240-760days). No complications occurred peri-procedurally or during follow-up. His-bundle pacing is feasible in pediatric and congenital heart disease patients.

An Incidental Finding of Congenital Complete Heart Block Presenting in Active Labor: A Multidisciplinary Approach

Congenital complete heart block is a rare occurrence. In some cases, it remains asymptomatic until adulthood or in the case of women until pregnancy. It is usually secondary to placental transfer of maternal antibodies and is associated with high mortality and morbidity. We present a case of a parturient who presented in active labor with premature rupture of membranes and decreased fetal movements. We found that the patient had a complete heart block with mild effort intolerance on evaluation. Markers for metabolic and ischemic causes were negative, and we made a provisional diagnosis of congenital complete heart block. The patient underwent a lower section cesarian section under spinal anesthesia with temporary pacemaker backup. Postoperatively, the patient underwent permanent pacemaker implantation. This case report underlines the importance of standard American Society of Anesthesiologists (ASA) monitoring, including a 12-lead electrocardiogram (ECG), which could prove decisive and life-saving in dire circumstances.

Systemic Lupus Erythematosus Management in Pregnancy.

Systemic lupus erythematosus (SLE) affects reproductive aged women. Issues regarding family planning are an important part of SLE patient care. Women with SLE can flare during pregnancy, in particular those who have active disease at conception or prior history of renal disease. These flares can lead to increased adverse pregnancy outcomes including fetal loss, pre-eclampsia, preterm birth and small for gestational aged infants. In addition, women with antiphospholipid antibodies can have thrombosis during pregnancy or higher rates of fetal loss. Women who have anti-Ro/SSA and anti-La/SSB antibodies need special monitoring as their offspring are at risk for congenital complete heart block and neonatal lupus. Ideally, SLE patients should have their disease under good control on medications compatible with pregnancy prior to conception. All patients with SLE should remain on hydroxychloroquine unless contraindicated. We recommend the addition of 81mg/d of aspirin at the end of the first trimester to reduce the risk of pre-eclampsia. The immunosuppressive azathioprine, tacrolimus and cyclosporine are compatible with pregnancy and lactation, mycophenolate mofetil (MMF)/mycophenolic acid are not. Providers should use glucocorticoids at the lowest possible dose. Methotrexate, leflunomide and cyclophosphamide are contraindicated in pregnancy and lactation. SLE patients on the biologics rituximab, belimumab and abatacept can continue these medications until conception and resume during lactation.

Long term clinical outcomes in patients requiring cardiac pacing due to congenital complete heart block

BackgroundIsolated congenital complete heart block (CCHB) is rare cardiac condition associated with maternal Anti-Rho antibodies. It is managed with permanent pacemaker insertion. We sought to determine the long-term outcomes of individuals with CCHB who had undergone pacemaker insertion. MethodsA database search was performed at a UK tertiary cardiac referral centre. The online medical records of patients with CCHB were analysed for pacing requirements and complications, echocardiographic data, and clinical status. ResultsA total of 72 patients (female n = 46) were identified, 20% of whom were autoantibody positive. Mean age of initial implantation was 15.5 years (SD 12.7) with mean follow up of 21 years (SD 8.3). 88% of patients were NYHA class I at censure of data. Major adverse cardiac events (MACE) were observed in 2 patients with 0% mortality throughout the follow up period. 16 patients (22.2%) developed cardiomyopathy, of which 15 had CRT devices inserted.Five patients had device related infections and 7 required system extractions. Mean left ventricular ejection fraction on most recent echocardiogram was 53.7% (SD 8.40) with no significant change compared with their historic scan. Mild tricuspid regurgitation was the most frequently observed valvular pathology, identified in 28% of patients. Only 2 patients (2.7%) had severe valve incompetence but neither required surgical intervention. ConclusionLong-term outcomes for patients with congenital complete heart block who undergo pacemaker insertion are highly favourable. Despite high pacing requirements over an extended period, the incidence of MACE and pacing related complications is low. Cardiac function and valvular competence are largely preserved but dilated cardiomyopathy remains a late concern in a minority; ongoing clinical surveillance is paramount in this population.

Management of fetal congenital heart block in pregnancies with anti-Ro antibodies

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