Congenital Complete Atrioventricular Block Research Articles

Transvenous lead advancement in pediatric pacing to overcome growth-induced lead straightening and stretching.

The stretching of the lead caused by somatic growth may lead to complications (dislodgement, fracture, failure) of transvenous leads implanted in pediatric patients. Atrial loop and absorbable ligatures may prevent it. Periodical lead advancement with lead pushing from the pocket may be an option to growth-induced stretching. Our aim was to analyze retrospectively the outcome of periodical transvenous lead advancement in children with pacemaker (PM). A procedure of lead advancement was performed in patients with a single-chamber PM implanted for isolated congenital complete atrioventricular block or sinus node dysfunction with growth-induced lead straightening/stretching. The PM pocket was opened, the lead was released from subcutaneous adherences and was gently advanced to shape again a loop/semi-loop in the atrium without dislodging the tip. Lead data (threshold, sensing, impedance) were compared before and after the procedure. Data are described as median (25th-75th centiles). 14 patients with 13 VVIR and 1 AAIR PM implanted at 6.8 (5.9-8.0) years of age, 23 (19-26) kg, 118 (108-124) cm, underwent 30 advancement procedures, 1.5 (1.0-2.3) per patient, during follow-up [45 (35-63) months]. Delta between procedures was: 18 (14-25) months, 11 (7-13) cm, 6 (4-9) kg; 90% of leads were successfully advanced without complications. Three unsuccessful procedures occurred with longer times [30 (14-37) months]. Electrical lead parameters did not show significant differences pre-/post-procedures. the advancement of transvenous leads in children seems safe and effective. This procedure may be another possible choice to preserve transvenous lead position and function until growth has completed.

Prevalence of neonatal lupus in a small group of antibody-carrying mothers and frequency of complete atrioventricular block.

Neonatal lupus (NL) is extremely rare and is caused by the transplacental passage of maternal IgG autoantibodies against Ro, La, and/or RNP proteins into the fetal circulation, which can cause congenital complete atrioventricular block (CCAB), permanent skin lesions, and liver involvement. To know the prevalence of NL in patients with CCAB and the clinical course in long-term follow-up. From January 1992 to December 2017, patients with CCAB were included. The presence of anti-SSA/Ro and anti-SSB/La antinuclear antibodies in maternal serum confirmed NL. Eight patients were included with a follow-up of 10 ± 6 years; NL was concluded in 62.5%; two were male. One of them was diagnosed in utero, two at birth, and a pacemaker was implanted in them, one at 12 years of age and another at 15. The other two cases were diagnosed at 18 and 26 years of age, and permanent pacemakers were implanted 8 and 5 years later, respectively. In one case, a definitive pacemaker was not implanted in a newborn with only 1 year of follow-up. At delivery, 60% of the mothers were free of rheumatic disease, and altogether, they all had 19 children; none of them presented NL manifestations. CCAB is rare and frequently associated with a maternal autoimmune disease, practically all of them will require a definitive pacemaker at some point in their lives.

Experience of treating congenital complete atrioventricular block with epicardial pacemaker in infants and young children: a retrospective study

BackgroundThis article summarizes the treatment experience for congenital complete atrioventricular block (CCAVB) in newborns and infants, and discusses the necessity and feasibility of treating CCAVB with permanent pacemaker implantation in this population.MethodsIn this study, the clinical data and follow-up results of nine children admitted at our center with CCAVB from January 2005 to March 2023 were retrospectively analyzed. Among them, two children received early implantation of permanent pacemakers (within 1 year of age), two children received non-early implantation (1 year or older), and the remaining five children received no pacemaker implantation. CCAVB diagnosis was confirmed by clinical symptoms and clinical examinations, including electrocardiography and echocardiography before surgery. After surgery, the pacing and sensing functions of the pacemaker were observed using electrocardiography, echocardiography, and pacing threshold monitoring. A comprehensive assessment of the treatment efficacy was conducted, encompassing improvements in clinical symptoms, growth and development, as well as the absence of any additional potential complications. The children who did not receive pacemaker implantation were followed up.ResultsAmong the four children who successfully received pacemaker implantation, one child who received non-early implantation died. For the remaining three children, the threshold level, amplitude, impedance, and minute ventilation sensor function of the pacemaker were good during the follow-up period, with a heart rate at the pacing rate. The growth and development of the aforementioned patients who received pacemaker implantation demonstrated adherence to the percentile curve, and their motor and cognitive development remained unaffected. However, among the children who did not undergo pacemaker implantation, two experienced death, while three were lost to follow-up, thereby limiting the evaluation of their long-term outcomes.ConclusionsEarly implantation of an epicardial pacemaker at an early stage in newborns and infants diagnosed with CCAVB can significantly improve clinical symptoms without affecting their growth and development. These data are in line with current literature and suggest that early implantation of an epicardial pacemaker in newborns and infants diagnosed with CCAVB but further studies are needed.

Epicardial pacing lead implantation for congenital complete atrioventricular block immediately after birth: a case report

BackgroundThe incidence of congenital complete atrioventricular block is estimated to be 1 per 20,000 deliveries. In the fetal period, the fetal mortality rate is high, but the treatment strategy has not yet been established. In severe cases, early postnatal pacing therapy is necessary.Case presentationA 0-day-old Japanese baby girl was diagnosed with fetal congenital complete atrioventricular block during a prenatal physical examination. A joint conference was held preoperatively among multidisciplinary departments, and a cesarean section was performed at 37 weeks pregnancy, immediately followed by scheduled internal ventricular pacing lead implantation in an adjacent room. Percutaneous pacing was ineffective. The epicardial pacing lead was sutured at 17.5 minutes after birth, and perioperative management was successful with a heart rate and pulse rate of 150 beats per minute.ConclusionThe infant with a congenital complete atrioventricular block was rescued by an uneventful epicardial lead implantation.

Complete Heart Block In Pregnancy And Manual Removal Of The Placenta After Vaginal Delivery

Management of the risk for cardiovascular and obstetrical complications is difficult in pregnant women with complete heart block. Complete congenital or acquired atrioventricular block can detected for the first time during pregnancy and delivery but only few cases have been reported in the literature. The first case was reported in 1914 by Nanta [1]. The acquired variety is very rare during pregnancy as this type is mostly seen after 50 years of age[2].During pregnancy acquired heart block may be due to myocarditis, collagen vascular diseases ,following infective endocarditis of aortic valve with root abscess or as a complication of cardiac surgery. In acquired heart block, heart rate is usually 40 or less per minute with wide QRS in ECG. They are usually symptomatic with temporary loss of consciouness in the form of presyncope or syncope[3]. The prognosis is generally worse, but ultimately depends on the underlying cause [4]. Complete atrioventricular block detected for the first time during pregnancy is usually congenital. With an incidence of 1 in 20,000 live born infants [5],congenital complete atrioventricular block is a rare disease. The aetiology is not completely understood. However, complete atrioventricular block may be isolated or combined with congenital heart diseases in up to 53% of affected individuals[6] It may be caused by cardiac malformations or damaged by maternal antibodies .Family history of complete atrioventricular block is one of the risk factor for congenital atrioventricular block [7-8].

Emergency pacing via the umbilical vein of a neonate with congenital complete atrioventricular block: a report of two cases.

We report two cases of successful emergency pacing via the umbilical vein in neonates with congenital complete atrioventricular block. The first patient, a neonate with normal cardiac anatomy, underwent emergency temporary pacing via the umbilical vein under echocardiographic guidance. The patient underwent permanent pacemaker implantation on postnatal day 4. The second patient, a neonate with heterotaxy syndrome, underwent emergency temporary pacing through the umbilical vein under fluoroscopic guidance. The patient underwent permanent pacemaker implantation on postnatal day 17.

Immediate single-chamber pacemaker implantation in a 2-hour-old infant with complete congenital atrioventricular block.

Immediate single-chamber pacemaker implantation in a 2-hour-old infant with complete congenital atrioventricular block.

Ventricular Functional Analysis in Congenital Complete Heart Block Using Speckle Tracking: Left Ventricular Epicardial Compared to Right Ventricular Septal Pacing.

Chronic right ventricular (RV) apical pacing in patients with congenital complete atrioventricular block (CCAVB) is associated with left ventricle (LV) dyssynchrony and dysfunction. Hence, alternative pacing sites are advocated. The aim of this study was to compare LV function using STE in selected patients with LV epicardial pacing (LVEp) vs. RV transvenous pacing (RVSp). This was a single-center, retrospective study in patients with CCAVB who underwent permanent pacemaker implant at age ≤ 18years. Age- and gender-matched patients with a normal heart anatomy and function served as the control group. LV function was comprehensively assessed by conventional 2D Echocardiography and speckle-tracking echocardiography (STE). We included 24 patients in the pacemaker group [27.6% male, mean age of 17.1 at last follow-up, follow-up duration of 8.7years, RVSp (n = 9; 62.5%)] compared to 48 matched healthy controls. Shortening fraction (SF) and ejection fraction (EF) were normal and similar between cases and controls. However, STE detected abnormal LV function in the pacemaker group compared to controls. The former demonstrated lower/abnormal, Peak Longitudinal Strain myocardial (PLS Myo) [- 12.0 ± 3.3 vs. - 18.1 ± 1.9, p < 0.001] and Peak Longitudinal Strain endocardial (PLS endo) [- 16.1 ± 4.1 vs. 1.7 ± 1.7, p < 0.001]. STE parameters of LV function were significantly more abnormal in LVEp vs. RVSp subgroup as demonstrated by lower values for PLS Myo (- 10.1 ± 3.2 vs. - 13.1 ± 2.9, p = 0.03) and PLS Endo (- 13.8 ± 4.4 vs. - 17.5 ± 3.3, p = 0.03). STE was more sensitive in detecting subtle differences in LV function relative to standard conventional 2D echocardiography (SF and EF) in selected patients with CCAVB and a permanent pacemaker. Furthermore, STE demonstrated that transvenous RV septal pacing was associated with better LV systolic function preservation than LV epicardial pacing for comparable post-implant intervals.

621 TRANSVENOUS LEAD ADVANCEMENT IN PAEDIATRIC PACING TO OVERCOME GROWTH STRETCHING

Abstract Background One of the main complications of transvenous leads implanted in paediatric patients is the stretching of the lead caused by the somatic growth. It may cause pacing and sensing defects and lead dislodgement or even fracture. Absorbable lead ligature and atrial loop may reduce this risk. However, the loop may induce traction or may unroll too early and therefore impair lead function. Lead extraction and replacement is another solution, although it has some procedural risks in young patients. Lead advancement through pushing it from the pocket may solve growth-induced traction and spare the electrode throughout childhood until post-puberty. Objective of the study is the retrospective analysis of the outcome of the transvenous lead advancement in children with a pacemaker (PM) in a single tertiary paediatric center. Methods consecutive patients with a VVIR PM implanted for isolated congenital complete atrioventricular block (no structural heart disease) in alternative right ventricular (RV) pacing sites, with lead stretching underwent a trial of lead advancement during general anaesthesia, cefuroxime antibiotic profilaxis, from 2014 to 2021. After venous angiography showed venous patency, the PM pocket was opened, the lead was released from subcutaneous adherences and with a stylet was gently advanced to create a semi-loop in the atrium without dislodging the tip. Lead data (threshold, sensing, impedance) were compared before and after the procedure. Data are expressed as median (25th-75th centiles). Results 8 patients underwent PM implantation at 6.8 (5.8-8.0) years of age, 20 (18-21) kg, 116 (106-120) cm, with the lead positioned at parahisian(3)/mid-septum (5 pts) sites. During a follow-up of 3 (1-5) years, advancement procedures were 1.5 (1.0-4.3) per patient. Between procedures, delta age was 16 (12-20) months, height 8 (7-11) cm and weight 5 (3-7) kg. All leads were successfully advanced without any procedural complications. Procedure time (skin to skin) was 96 (73-108) minutes, fluoroscopy was 0.5 (0.2-1.4) mGy, 16 (10-46) microGy/m2. Electrical lead parameters did not showed significant differences between consecutive control times. Conclusion the advancement of transvenous leads in children is a safe and effective procedure, with low fluoroscopy exposure, without significant acute and chronic complications. This procedure seems to preserve transvenous leads until growth has completed.

MiR Profile of Chronic Right Ventricular Pacing: a Pilot Study in Children with Congenital Complete Atrioventricular Block

Chronic ventricular pacing can lead to pacing-induced cardiomyopathy (PICM). Clinical data alone is insufficient to predict who will develop PICM. Our study aimed to evaluate the circulating miR profile associated with chronic right ventricular pacing in children with congenital complete AV block (CCAVB) and to identify candidate miRs for longitudinal monitoring. Clinical data and blood were collected from chronically paced children (N = 9) and compared with non-paced controls (N = 13). miR microarrays from the buffy coat revealed 488 differentially regulated miRs between groups. Pathway analysis predicted both adaptive and maladaptive miR signaling associated with chronic pacing despite preserved ventricular function. Greater profibrotic signaling (miRs-92a, 130, 27, 29) and sodium and calcium channel dysregulation (let-7) were seen in those paced > 10 years with the most dyregulation seen in a patient with sudden death vs. those paced < 10 years. These miRs may help to identify early adverse remodeling in this population.Graphical abstract

To go left or right? Driving towards the best direction in paediatric pacing.

Permanent pacing in children with isolated congenital complete atrioventricular block may cause left ventricular dysfunction. To prevent it, alternative pacing sites have been proposed: left ventricular epicardial or selective right ventricular endocardial pacing. To compare the functional outcome (left ventricular systolic function and synchrony) in paediatric patients with congenital complete atrioventricular block and left ventricular apical epicardial or right ventricular transvenous mid-septal pacing. Retrospective study. Epicardial leads were implanted by standard surgical technique, transvenous leads by 3D electroanatomic mapping systems. 3D mapping acquired 3D right ventricular local pacing map and defined the narrowest paced QRS site. 3D mapping guided screw-in bipolar leads on that ventricular site. Electrocardiogram (ECG) (QRS duration) and echocardiographic data (synchrony: interventricular mechanical delay, septal to posterior wall motion delay, systolic dyssynchrony index; contractility: global longitudinal strain, ejection fraction) were recorded. Data are reported as median [interquartile ranges]. p < 0.05 was significant. There were 19 transvenous systems (age 8.8 [6-14] years; right ventricular mid-septum) and 17 epicardial systems (0.04 [0.001-0.6] years; left ventricular apex). Post-implantation QRS significantly widened either in endocardial or in epicardial patients. Most patients reached 4-year follow-up. One-year and 4-year ejection fraction and global longitudinal strain were mostly within normal limits and did not show significant differences between the two groups and between the same endocardial/epicardial group. Synchrony parameters were within normal limits in the two groups. Left ventricular apical epicardial pacing and 3D mapping-guided right ventricular mid-septal pacing preserved left ventricular contractility and synchrony in children and adolescents with congenital complete atrioventricular block at short-/mid-term follow-up, without relevant significant differences between the two groups.

Transvenous lead advancement in paediatric pacing to overcome growth stretching

Abstract Funding Acknowledgements Type of funding sources: None. Background/Introduction One of the main complications of transvenous leads implanted in paediatric patients is the stretching of the lead caused by the somatic growth. It may cause pacing and sensing defects and lead dislodgement or even fracture. Absorbable lead ligature and atrial loop may reduce this risk. However, the loop may induce traction or may unroll too early and therefore impair lead function. Lead extraction and replacement is another solution, although it has some procedural risks in young patients. Lead advancement through pushing it from the pocket may solve growth-induced traction and spare the electrode throughout childhood until post-puberty. Purpose of the study is the retrospective analysis of the outcome of the transvenous lead advancement in children with a pacemaker (PM) in a single tertiary paediatric center. Methods Consecutive patients with a VVIR PM implanted for isolated congenital complete atrioventricular block (no structural heart disease) in alternative right ventricular (RV) pacing sites, with lead stretching underwent a trial of lead advancement during general anaesthesia, cefuroxime antibiotic profilaxis, from 2014 to 2021. After venous angiography showed venous patency, the PM pocket was opened, the lead was released from subcutaneous adherences and with a stylet was gently advanced to create a semi-loop in the atrium without dislodging the tip. Lead data (threshold, sensing, impedance) were compared before and after the procedure. Data are expressed as median (25th-75th centiles) Results: 7 patients underwent PM implantation at 6.9 (5.5-8.0) years of age, 20 (18-21) kg, 116 (106-120) cm, with the lead positioned at parahisian(3)/mid-septum (4 pts) sites. During a follow-up of 3 (1-5) years, advancement procedures were 2 (1-4) per patient. Between procedures, delta age was 15 (12-19) months, height 7 (6-11) cm and weight 4 (2-6) kg. All leads were successfully advanced without any procedural complications. Procedure time (skin to skin) was 91 (69-105) minutes, fluoroscopy was 0.4 (0.2-1.2) mGy, 13 (9-35) microGy/m2. Electrical lead parameters did not showed significant differences between consecutive control times. In one parahisian pacing, chronic threshold increased after 3 years (2 advancement procedures) from 0.7V to 2.6 V/0.4 ms. Conclusion the advancement of transvenous leads in children is a safe and effective procedure, without significant procedural complications and during follow-up, and with low fluoroscopy exposure. This procedure may maintain a good function of transvenous leads until growth has completed.

Complete Congenital Atrioventricular Block Found by Ultrasound in Pregnancy

A 27-year-old woman at 7 months of pregnancy presented to the obstetric emergency department with a history of fetal bradycardia for 3 days. Her vital signs were within normal limits. Autoimmune disease screening revealed positive antinuclear antibodies and weakly positive anti–Sjögren's syndrome A antibodies. Color Doppler ultrasound of the fetal 4-chamber view showed low pulse rates, mitral regurgitation, and increased cardiothoracic ratio (Video E1 [available at http://www.annemergmed.com]), which might have certain clinical significance.

Pacing therapy of autoantibody-related congenital complete atrioventricular block in 3 neonates

3例免疫性先天性完全性房室阻滞（CCAVB）的新生儿，20~24周胎龄起病，胎心率35~55次/min，母亲均诊断为干燥综合征，经激素等治疗胎儿CCAVB无逆转。分别于生后24 h、生后即刻及44日龄（校正胎龄36+5周）经剑突下小切口植入心外膜永久起搏器，电极固定于近右室流出道中后间隔部位。随访3个月至2.5年起搏阈值稳定，心功能及生长发育正常。.

Outcomes in congenital and childhood complete atrioventricular block: A meta-analysis.

The long-term outcomes of patients with congenital and childhood complete atrioventricular block (CCAVB/CAVB) after pacemaker implantation are unclear. We performed a meta-analysis of all the studies of CCAVB. A systematic search of PubMed and CENTRAL databases from January 1, 1967 to January 31, 2020 was performed. The quality of studies included was critically appraised using the Newcastle-Ottawa scale, and outcome data were analyzed using the restricted maximum likelihood function. Twenty-nine studies were eligible for analysis, with a total of 1553 patients. The all-cause-mortality was 5.7% (95% confidence interval [CI]: 2.5%-9.9%), while pacing-induced cardiomyopathy (PICM) was seen in 3.8% (95% CI: 1.2-7.2). Diagnosis at birth (effect size [ES] [95%CI]: -2.23 [-0.36 to -0.10]; p < .001), presence of congenital heart disease (ES [95%CI]: -0.67 [0.41-0.93]; p < .001), younger age at pacemaker implantation (ES [95%CI]: -0.01 [-0.02 to -0.001]; p = .02), and duration of pacing (ES [95%CI]: -0.03 [-0.05 to -0.003]; p = .03), were associated with an higher mortality on binominal logistic regression. None of the parameters were significant on multivariate analysis. Pooled proportional mortality in patients with CCAVB and CAVB is 5.7% with an infrequent incidence of PICM (3.8%) in the paced patients with AVB suggesting that pacing in these patients is an effective management strategy with a low incidence of long-term side effects. Registry and randomized data can throw additional light regarding the natural history and appropriate management strategy in these patients.

The first successful double switch operation for congenitally corrected transposition of the great arteries in Slovenia

Congenitally corrected transposition of the great arteries is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial discordance and can be potentially associated with several other concomitant anomalies, such as ventricular septal defect or congenital complete atrioventricular block. Different surgical options are used for treating the condition. Anatomic repair yields the best long-term outcomes; however, it is technically the most challenging. We present a case of a 3-year old female patient who was electively admitted for complete repair after pulmonary artery banding and pacemaker implantation soon after birth. Subsequently, an anatomic repair (double switch) was performed with an uneventful postoperative course and excellent mid-term outcomes.

Local device infection successfully treated without pacemaker removal in a neonate: a case report

Local device infection is a serious complication, especially in neonates. Complete device removal is the gold standard treatment for cardiac device infection; however, in selected cases alternative strategies could be adopted. We describe a case of a 14-day-old neonate, weighing 2.5kg, who had undergone epicardial double chamber pacemaker implantation for a congenital complete atrioventricular block. The generator pocket was created in the epigastric area below the rectus abdominis. At six days after implantation, pocket infection was found; blood cultures and the transoesophageal echocardiogram were normal. Due to the low weight of the neonate, and the limited possibility of finding a new comfortable site for housing the generator far from the infected area, we opted for a conservative strategy. We successfully applied a combination of antibiotic therapy, a vacuum-assisted wound closure system (KCI, Germany) for 40 days, and then skin transfer flap from the right flank without device removal. At one-year follow-up there were no local or systemic signs of infection.

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.

Mortality in patients with isolated congenital complete atrioventricular block in Sweden: a register-based, nationwide cohort study

Abstract Purpose The congenital heart block is a rare but potentially serious heart conduction disease and associated with high mortality during the fetal or neonatal periods. Earlier reports are based on relatively small number of patients and there are limited data about long-term outcomes. Methods Data were collected from the Swedish Patient and Cause of Death register to identify all patients who were born and diagnosed with isolated congenital complete atrioventricular block (CCAVB) between 1970 and 2017. Each patient with isolated CCAVB was matched with 10 control individuals, by birth year and sex from the Total Population Register. Results Totally 506 patients with CCAVB and 5,050 controls included in the study (55.3% males). The incidence rate of mortality in patients with isolated CCAVB was 166 per 10.000 person-years. The overall risk of mortality in patients with isolated CCAVB was 45.4 times (95% confidence interval (CI) 32.7–62.9) higher compared to matched controls. In a subgroup investigation, the highest risk of mortality was found in patients with isolated CCAVB from birth until the age of 17 (hazard ratio 80.8, 95% CI 51.6–126.4), compared to matched controls without CCAVB. Conclusion In this nationwide cohort study, patients with isolated CCAVB was associated with more than 40-fold risk for overall mortality compared to age and sex matched controls. The greatest risk was found during childhood. Regularly follow-up in tertiary centers and the aware of high risk of complications may be the key to prevention. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): The Swedish Government, the Swedish Research Council

VP23.18: Congenital complete atrioventricular block: two cases with different outcomes

VP23.18: Congenital complete atrioventricular block: two cases with different outcomes