Congenital Complete Heart Block Research Articles

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.

253 Imaging of congenital AV block caused by maternal autoantibodies: a single-centre experience

Abstract Aims Autoimmune congenital heart block (CHB) is a severe manifestation of neonatal lupus syndrome. There is lack of consensus regarding treatment of pregnant women with anti-SSA/SSB autoantibodies. To evaluate the effectiveness of the combined protocol therapy (oral steroid, plasmapheresis, and IVIG) for the CHB treatment. Methods and results All cases of CHB from 2000 to 2020 were retrospectively enrolled. All the patients underwent foetal echocardiography for the evaluation of CHB, ventricular rate and main related foetal complications: cardiomegaly, pericardial and pleural effusion, foetal hydrops, dilated cardiomyopathy, and heart failure. Moreover, postnatal major adverse cardiovascular, such as death and pacemaker implantation, were recorded. For statistical analysis, the population was divided into two cohorts: a protocol group receiving in utero combined therapy and a control group receiving other therapies or not treated. Among 252 pregnancies with anti-SSA/SSB antibodies, 36 developed CHB. At birth, complete CHB treated with protocol therapies showed a significantly higher ventricular rate (P = 0.042), a significant reduction in intrauterine or postnatal mortality (P = 0.018), and a lower rate of pacemaker implantation (P = 0.049). Conclusions The combined treatment protocol has proven effective in improving foetal and neonatal short- and long-term survival.

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.

Mucormycosis: An Uncommon Cutaneous Infection at Permanent Pacemaker-Implanted Site in a Very Low-Birthweight Baby

AbstractPermanent pacemaker implantation in low birthweight (LBW) babies with congenital complete heart block is extremely challenging due to a paucity of appropriate pulse generator placement pocket sites. The development of infection following an implantation procedure can pose a life-threatening risk to the patients. With more patients in the younger group receiving these devices than ever before and the rate of infection increasing rapidly, a closer look at the burden of infection and its impact on outcome of these patients is warranted. We report mucormycosis infection at the abdominal pacemaker pocket site of an infant requiring pacemaker explantation and re-insertion into the intrapleural space.

Congenital Complete Heart Block in Fetal Echocardiography

Congenital Complete Heart Block (CHB) in a fetus is a rare diagnosis occurring 1 in 15,000 to 20,000 live births which can be diagnosed by echocardiography. It’s less talked disease. It is uncommonly reported even in countries where fetal echocardiography is performed in much higher numbers. For countries like Nepal, where small number of fetal echocardiography is performed, we present a case of lupus as the cause of congenital CHB.

Abstract 11780: Longitudinal Echocardiographic Parameters Before and After Pacemaker Placement in Congenital Complete Heart Block

Introduction: Congenital complete heart block (CCHB) is rare, 1:15,000 - 1:20,000 live births, with left ventricular (LV) dysfunction developing in 7-14% of subjects. We investigate longitudinal trajectories of echo parameters in CCHB and the effect of pacemaker implantation on those parameters. Methods: This is a retrospective cohort study of identifiable CCHB subjects at the Children’s Hospital of Philadelphia from 1970-2018, excluding those with structural, genetic, or acquired cardiac conditions. Subjects with echo data available before and after pacemaker implantation are included. LV end diastolic diameter (LVEDD) z-score and LV ejection fraction (EF) are modeled with least-squares regression models, with slopes compared before and after pacemaker placement. Results: Of 114 subjects with CCHB, 52 (40% male, 73% white) have echo data available before and after pacemaker placement. Median age of diagnosis is 0.6 years, age at pacemaker placement 3.4 years, and duration of pacing 10.8 years. Figure 1 displays modeled trajectories of LVEDD z-score and EF over 20 years. Mean LVEDD z-score at pacemaker placement is less than 2 and decreases significantly over time, especially 10 years post placement. Participants have mean LV ejection fraction of 54% at pacemaker placement, rising significantly prior to placement and in the first 10 years post-placement, after which significant decreases are observed. Older age at pacemaker placement is associated with a higher EF at placement but an earlier and steeper decline in EF beginning at 8 years post-placement. LVEDD and EF trajectories do not vary significantly by sex, race, maternal antibody status, age at diagnosis, or initial pacemaker mode. Conclusions: In a cohort of 52 subjects with CCHB, LVEDD z-score steadily decreases over time whereas EF rises for 10 years post-pacemaker placement and subsequently decreases. Pacemaker placement has no apparent immediate effect on trajectories of these echo parameters.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Executive Summary.

Cohort study of congenital complete heart block among preterm neonates: a single-center experience over a 15-year period.

Congenital complete heart block (CCHB) is a very rare condition, with high risk of mortality. Prematurity is associated with immaturity of the cardiovascular system. Morbidity related to CCHB and prematurity has never been described. We describe a tertiary perinatal center experience over a 15-year period on CCHB management and complications in preterm infants. This is a single-center observational cohort study. All neonates admitted to neonatal intensive care unit with a diagnosis of isolated CCHB between January 2006 and January 2021 were identified. All preterm neonates (< 37 weeks) were compared with a control cohort of term neonates (≥ 37 weeks). Antenatal data, complications of prematurity, medical, and surgical management of CCHB were recorded. Twenty-four neonates with isolated CCHB (16 preterm and 8 term) were born during the study period, including 5 very preterm (< 32 weeks) and 11 preterm (32 to 37 weeks). All very preterm were born via emergency caesarian section without antenatal steroid administration. They had multiple severe morbidities including chronic lung disease, necrotizing enterocolitis, grades 3–4 intraventricular hemorrhage, cystic periventricular leukomalacia, and longer periods of mechanical and non-invasive ventilatory support than preterm. Thirteen out of sixteen preterm infants had permanent pacemakers inserted, compared to 1/8 for term newborns. All babies born before 35-week gestation were either paced or died.Conclusion: Premature neonates with CCHB have high risk of mortality and morbidity especially if undiagnosed and born by unnecessary emergency caesarian section without antenatal steroids. Prematurity below 35 weeks may be associated with death or pacemaker insertion. This supports better antenatal screening to avoid induced prematurity. What is Known:• Congenital complete heart block is a very rare condition associated with high morbidity and mortality.• Antenatal risk factors for poor outcome include fetal hydrops, low ventricular rate (HR <55 beats per minute), and congenital heart defect.What is New:• Infants born <32 weeks with CCHB had no antenatal steroid administration, and sustained high burden of morbidity (chronic lung disease, intraventricular hemorrhage, and cystic periventricular leukomalacia). • Birth <35 weeks is strongly associated with requiring pacing prior to discharge or death. Supplementary informationThe online version contains supplementary material available at 10.1007/s00431-021-04293-8.

Single Centre Experience in Pediatric Pacing

Abstract Corresponding author hosnihosni5627@gmail.com ( Hosny Hosny Elsayed Arafa) Background Permanent Pacemakers have a growing use in the pediatric population due to congenital and surgically acquired rhythm disturbances but they present unique problems and implications. Their implantation, follow up, the diversity and complexity of pediatric patients and congenital heart disease make device management a highly individualized art in pediatric pacing. Certain challenges are posed in adult-like somatic growth and active lifestyle susceptibility to infection and generally anticipated long survival. Objective The current study aims to present our institute's experience in pediatric and adolescent pacemaker implantation and long term outcome. Patients and Methods The study included 100 patients who have cardiac devices (permanent pacemaker)that were implanted in AinShams University and visit our patient clinic for regular programming during the period from July 2018 to December 2019. All patients were subjected to history taking, clinical examination, ECG recording then Echocardiography and device programming. Results The study included 100 patients,49 females and 51 males with a mean age of 13.12 ± 5.04. Patients weighted from 8 to 85 kg, measured from 40 to 185 cm in height. According to the mode of pacing, 67 were in the VVIR group while 33 in the DDDR group. According to the indication of pacing, two cases were due to sinus node dysfunction,52 due to congenital complete heart block and 46 due to postoperative CHB. 59 patients underwent cardiac surgery. 53 were admitted to CCU by syncope or presyncope. As Regards the complications,2 cases had superficial infection after 2 weeks relived by antibiotic, 2 patients suffered from pocket hematoma after 2 weeks, 10 cases of lead fracture from 1 to 8 years after implantation due to external trauma, 3 cases of lead dislodgment from 1 to 6 months postprocedure,1 case developed LV dysfunction and upgraded to CRT,1 case had psychological trauma and 3 cases had insulation breaks. The device with the longest longevity duration is Medtronic and Tendril leads had the most common complications. Conclusion Implantation of a pacemaker in children is generally safe. It could be justified to implant the pacemaker to improve the hemodynamic situation in individual patients or to consider biventricular pacing in LV dysfunctiont

Transposition of The Great Arteries, Situs Inversus with Congenital Complete Heart Block: Initial Management and Referring to a Special Unit

A case of a term female newborn, 40-week gestational age (by ultrasound (US)) with prenatal diagnosis of situs inversus and levocardia is reported. Transposition of the great arteries (TGA) and irregular fetal bradycardia were prenatally detected. Immediately after birth, electrocardiogram (ECG) monitoring revealed a complete heart block with no sign of hemodynamic compromise. The patient was then transferred to the neonatal intensive care unit (NICU) for ventilatory support. Close observation and further investigation with echocardiogram were undertaken. The patient was transferred by air at the age of 4 days to the Queen Sirikit National Institute of Child Health for definitive cardiovascular treatments. This paper describes how to overcome the challenges of transporting a very young neonate with complex cardiac problems.

Symptomatic atrial bigeminy masquerading as congenital complete heart block.

Newborn male with symptomatic bradycardia initially diagnosed with complete atrioventricular block. Isoproterenol drip was initiated, and the patient was scheduled for pacemaker implantation. During the hospital course, repeat electrocardiogram and Holter monitor revealed evidence of near continuous blocked atrial bigeminy with occasional aberrantly conducted premature atrial contractions. Flecainide was started, resulting in normal sinus rhythm, and the pacemaker implantation was cancelled.

Third Trimester Fetal Heart Rates in Antibody-Mediated Complete Heart Block Predict Need for Neonatal Pacemaker Placement.

Congenital complete heart block (CCHB) affects 1 in 20,000 newborns. This study evaluates fetal and neonatal risk factors predictive of neonatal pacemaker placement in antibody-mediated complete heart block. The Children's Hospital Los Angeles institutional fetal, pacemaker, and medical record databases were queried for confirmed SSA/SSB cases of CCHB between January 2004 and July 2019. Cases excluded were those with a diagnosis beyond the neonatal period, diagnosis of a channelopathy, or if maternal antibody status was unknown. We recorded the gestational age (GA), birth weight (BW), fetal heart rates (FHRs) of the last echocardiogram before delivery, specific neonatal ECG and echocardiogram findings, age at pacemaker placement, and mortality. Of 43 neonates identified with CCHB, 27 had confirmed maternal antibody exposure. Variables associated with neonatal pacemaker implantation were FHRs < 50bpm (p = 0.005), neonatal heart rates < 52bpm (p = 0.015), and neonatal left ventricular fractional shortening (FS) percentages < 34% (p = 0.03). On multivariate analysis, FHR remained significant (p = 0.03) and demonstrated an increased risk of neonatal pacemaker placement by an odds ratio of 12.5 (95% CI 1.3-116, p = 0.05). The median GA at which the FHR was obtained was 34weeks (IQR 26-35weeks). Neonatal pacemaker placement was highly associated with a FHR < 50bpm, neonatal HR < 52bpm, and neonatal FS < 34%. FHRs at 34weeks GA (IQR 26-35weeks) correlated well with postnatal heart rates and were predictive of neonatal pacemaker placement.

Intrapleural Pocket for Pediatric Epicardial Pacemaker Through Right Axillary Incision

Congenital complete heart block and acquired complete heart block are serious conduction abnormalities that may necessitate permanent pacemaker placement. Small patient size and anatomy preclude transvenous pacemaker insertion and risk subcutaneous generator pocket complications. This report describes a case of placement of a pediatric bipolar epicardial pacemaker through a right axillary incision with an intrapleural polytetrafluoroethylene membrane pocket to facilitate future generator exchange.

B-PO05-031 AETIOLOGY OF ATRIOVENTRICULAR HEART BLOCK IN YOUNG AND MIDDLE AGED ADULTS FROM SOUTH AFRICA

There is a paucity of data regarding the aetiology of atrioventricular (AV) heart block in young and middle-aged patients particularly from the from low and middle income countries. To determine the aetiology of AV heart block in patients ≤ 55 years who were treated with a transvenous pacemaker from a middle income country. We performed a retrospective review of all patients who received a transvenous pacemaker for newly diagnosed AV block from 1 January 2013 to 31 December 2020 at Groote Schuur Hospital, Cape Town, South Africa. The cohort of patients ≤ 55 years were the focus of this study. During the study period, 1101 consecutive patients received a permanent transvenous pacemaker for AV block. 136 patients (12.4%) were ≤55 years. The mean (standard deviation) age of the study patients was 41 (11) years and 48.5% were female. Third degree AV block was diagnosed in 73.5% and 2:1 AV block in 8.1%. The aetiology of heart block could be determined in 97 (71.3%) patients. 43 (31.6%) patients were post-surgical, predominantly for isolated mitral valve (MVR) replacement (27.9%) and isolated aortic valve (AVR) replacement (20.9%). 16 had developed complete heart block from an acute myocardial infarction; inferior myocardial infarction in 9/16 and anterior myocardial infarction in 7/16 patients. Congenital complete heart block was diagnosed in 15 (11%) cases. Cardiac Magnetic Resonance (CMR) imaging was performed in 26 patients: cardiac sarcoidosis was diagnosed in 7/26 patients, prior myocarditis in 3/26, left ventricular non-compaction in 3/26, interatrial cardiac tumour in 1 patient, cardiac amyloid in patient with multiple myeloma in 1 patient and hypertrophic cardiomyopathy in 1 patient. No pathology could be identified 10/26 patients who had CMR. The aetiology of AV block remained unknown in 39 patients (28.7%). Prior valve surgery is the leading cause of AV block treated with transvenous pacemaker in patients ≤ 55 years at our institution. Cardiac magnetic resonance imaging identifies disease processes that require further management in a significant proportion of cases.

Neonatal Outcomes in Pregnant Women with Systemic Lupus Erythematosus: A 13-Year Experience in Southern Thailand.

The purpose of the study was to determine the clinical features of NLE and to compare the neonatal outcomes between newborns born to pregnant women with SLE and healthy pregnant women. We conducted a retrospective cohort analysis between 2007 and 2019 in a tertiary referral hospital in Thailand. A total of 118 pregnant women with SLE with 132 neonates compared with 264 randomly selected healthy pregnant women. The median (interquartile range) gestational age and birth weight of 132 neonates born to women with SLE were 37 (35-38) weeks and 2687 g (2045-3160 g), respectively. The clinical features of NLE infants were hemolytic anemia (8%), thrombocytopenia (2.7%) and hyperbilirubinemia (5.3%). There was no neonate with a congenital complete heart block or skin lesion. Moreover, logistic regression analysis found that neonates born to women with SLE increased the risk of preterm birth [odd ratio (OR) 8.87, 95% confidence interval (95% CI) 4.32-18.21, p < 0.001], low birth weight (OR 10.35, 95% CI 5.08-21.08, p < 0.001), birth asphyxia (OR 2.91, 95% CI 1.26-6.73, p = 0.011) and NICU admission (OR 4.26, 95% CI 2.44-7.42, p < 0.001). SLE disease activity and corticosteroid and azathioprine usage were associated with preterm delivery in pregnant women with SLE. The major clinical features of NLE patients were hematologic and hepatobiliary abnormalities in our study. Pregnancies with SLE dramatically increased the risk of preterm delivery and neonatal complications. Neonatal lupus erythematosus (NLE) is the consequence of the transplacental passage of autoantibodies to newborns during pregnancy. The clinical features of NLE infants in our study were hemolytic anemia (8%), thrombocytopenia (2.7%) and hyperbilirubinemia (5.3%). There was no neonate with a congenital complete heart block or skin lesion. We also compared the neonatal outcomes between 118 pregnant women with SLE and 264 randomly selected healthy pregnant women. Our study found that the neonates born to women with SLE increased the risk of preterm birth, low birth weight, birth asphyxia and NICU admission. Moreover, SLE disease activity and corticosteroid and azathioprine usage were associated with preterm delivery in pregnant women with SLE.

Permanent His Bundle Pacing in Patients With Congenital Complete Heart Block

This study retrospectively assessed the safety and efficacy of permanent His bundle pacing (HBP) in patients with congenital complete heart block (CCHB).HBP has become an accepted form of pacing in adults. Its role in CCHB is not known.Seventeen patients with CCHB who underwent successful HBP were analyzed at 6 academic centers between 2016 and 2019. Nine patients had de novo implants, and 8 patients had previous right ventricular (RV) leads. Three RV paced patients had reduced left ventricular ejection fractions at the time of HBP. Implant/follow-up device parameters, New York Heart Association functional class, QRS duration, and left ventricular ejection fraction data were analyzed.Patients' mean age was 27.4 ± 11.3 years, 59% were women, and mean follow-up was 385 ± 279 days. The following parameters were found to be statistically significant between implant and follow-up, respectively: impedance, 602 ± 173 Ω versus 460 ± 80 Ω (p < 0.001); and New York Heart Association functional class, 1.7 ± 0.9 versus 1.1 ± 0.3 (p = 0.014). In patients with previous RV pacing, HBP resulted in a significant decrease in QRS duration: 167.1 ± 14.3 ms versus 118.3 ± 13.9 ms (p < 0.0001). In de novo implants, HBP resulted in increases in QRS duration compared with baseline: 111.1 ± 19.4 ms versus 91.0 ± 4.8 ms (p = 0.016). Other parameters exhibited no statistically significant differences. During follow-up, 2 patients required lead revision due to elevated pacing thresholds.HBP seems to be safe and effective, with improvement in clinical outcomes in patients with CCHB. Larger studies with longer follow-up periods are required to confirm our findings.

Congenital Complete Heart Block in Young Women

Objectives: To present a rare case of Congenital Complete Heart Block (CCHB) in the setting of post-cesarean delivery of an asymptomatic young patient. Methods: A 30-year-old female patient complained of sudden weakness after C-section delivery with spinal anesthesia. She presented a slow heart rate and Complete Heart Block (CHB) on electrocardiogram (ECG). After one week of observation, the ECG still presented a CHB condition. A permanent pacemaker (PPM) with DDDR mode was then installed for this patient. Result: The etiology of CHB, especially at a young age, is unclear, hence challenging. A patient with a CCHB is difficult to diagnose, especially without any previously related symptoms. This abnormality is usually detected during routine screening not related to cardiovascular disease. The patient in this case study presented an ECG of persistent CHB from the time this patient was admitted until one week after observation. The echocardiography showed normal results. Other modalities to confirm diagnosis and evaluate the prognosis of a CCHB should be done. Conclusion: Establishing the etiology of CHB in young patients is challenging. The implantation of PPM is needed because the condition is permanent, regardless the etiology. However, implanting a permanent pacemaker is not always an easy decision, especially in young patients.

Is left ventricular superior to right ventricular pacing in children with congenital or postoperative complete heart block?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was 'Is left ventricular superior to right ventricular pacing in children with congenital or postoperative complete heart block?' Altogether, 19 papers were found using the reported search, of which 9 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Two large multicentric showed that site of pacing was the major determinant of left ventricular (LV) function with LV pacing being superior to RV pacing, though the number of patients paced via LV was lesser in comparison to right ventricular (RV). There were 2 prospective, 2 retrospective and 1 cross-sectional studies with fewer patients that demonstrated superiority of LV over RV pacing in preserving LV function. Only 1 small-scale retrospective study showed similar results of LV and RV pacing on LV function. One cross-sectional study showed superiority of LV apical pacing on exercise tolerance. As per the existing literature, LV apex seems to be the most optimal site for epicardial pacing while RV free wall pacing has the highest risk of causing LV dysfunction over the long term. We conclude that LV pacing appears to be superior to RV pacing in terms of long-term effect on cardiac function and ventricular synchrony.

Successful perinatal management and pacemaker stimulation during the first hour of life in a 1.6 kg newborn with autoimmune congenital complete heart block diagnosed prenatally

Successful perinatal management and pacemaker stimulation during the first hour of life in a 1.6 kg newborn with autoimmune congenital complete heart block diagnosed prenatally

Epicardial Versus Endocardial Pacemakers in the Pediatric Population: A Comparative Inquiry

Background: Most children in need of cardiac pacemakers remain dependent on the function of the permanent from childhood to adulthood. We sought to evaluate and compare the function between epicardial and endocardial pacemakers in pediatric groups with different conditions. Methods: Between 2012 and 2018, this single-canter study evaluated 44 pediatric patients with indications for epicardial or endocardial pacemakers. Results: The 2 groups, at a median age of 5 (0.1–16) years, were compared concerning the characteristics of the leads used (n = 80: bipolar, unipolar, steroid-eluting, and non–steroid-eluting), survival data, and complications. The reason for pacemaker implantation was congenital complete heart block in 11 (25%) cases and postoperative heart block in 33 (75%) cases. The commonest congenital heart disease accompanied by postoperative block was the ventricular septal defect. In the endocardial lead group, the mean ventricular pacing threshold immediately after the implantation and during the follow-up was less than that in the epicardial lead group (0.75 vs. 0.81 V; P = 0.01 and 0.8 vs. 2.4 V; P = 0.001). During the follow-up, the mean battery longevity was better in the endocardial group (last visit: 6.7 endocardial vs. 3.3 years epicardial). Lead failure was commoner in the epicardial pacemaker, and chronic high-pacing threshold pattern was seen in 14 patients in this group. After 3 years, freedom from lead failure was 94% and 63% in the endocardial and epicardial leads. Conclusions: Pacemakers with endocardial bipolar steroid-eluting leads showed better lead characteristics regarding survival and battery longevity than epicardial pacemakers without these lead characteristics. An appropriate pacemaker type should be selected based on the patient’s condition.