Congenital atrioventricular block: clinical presentation, clinical evaluation, and management

ESC CardioMed ◽  
2018 ◽  
pp. 1966-1968
Author(s):  
Drago Fabrizio ◽  
Battipaglia Irma
Keyword(s):  
Heart Disease ◽  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Cardiac Conduction ◽  
Ventricular Rate ◽  
Postnatal Life ◽  
Av Block ◽  
Cardiac Stimulation ◽  
Immune Mediated ◽  
Congenital Atrioventricular Block

Congenital atrioventricular block (CAVB) is a cardiac conduction disorder that is diagnosed in utero, at birth, or within the first month of life. When it is diagnosed between the first month and the 18th year of life, it is defined as childhood atrioventricular (AV) block. CAVB may occur in association with concomitant congenital heart disease, or be isolated, in a structurally normal heart (e.g. immune-mediated, inherited, or idiopathic CAVB). The majority of isolated CAVB is an immune-mediated AV block, due to transplacental passage of maternal autoantibodies, damaging the fetal cardiac conduction system. Only in a third of infants with immune-mediated CAVB is a well-defined autoimmune disease known in the mother. During fetal life, fetal echocardiography still represents the gold standard for the diagnosis of CAVB. Two major negative prognostic markers are low ventricular rate and the appearance of foetal hydrops. As regards prognosis, a risk for heart failure, syncope, and sudden death is present both during fetal and postnatal life. Dilated cardiomyopathy represents another complication in CAVB history, with different possible aetiologies (right ventricular permanent pacing, reactivation of autoimmune myocarditis). The indications for pacemaker implantation in patients with CAVB are similar to those for acquired heart disease, with some special technical considerations due to young age (epicardial versus endocardial systems, pacing site, etc.). As a future perspective, leadless cardiac stimulation in children with CAVB may represent a definitive solution and an answer to many questions.

Congenital atrioventricular block: clinical presentation, clinical evaluation, and management

ESC CardioMed ◽  
2018 ◽  
pp. 1966-1968
Author(s):  
Drago Fabrizio ◽  
Battipaglia Irma
Keyword(s):  
Heart Disease ◽  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Cardiac Conduction ◽  
Ventricular Rate ◽  
Postnatal Life ◽  
Av Block ◽  
Cardiac Stimulation ◽  
Immune Mediated ◽  
Congenital Atrioventricular Block

Congenital atrioventricular block (CAVB) is a cardiac conduction disorder that is diagnosed in utero, at birth, or within the first month of life. When it is diagnosed between the first month and the 18th year of life, it is defined as childhood atrioventricular (AV) block. CAVB may occur in association with concomitant congenital heart disease, or be isolated, in a structurally normal heart (e.g. immune-mediated, inherited, or idiopathic CAVB). The majority of isolated CAVB is an immune-mediated AV block, due to transplacental passage of maternal autoantibodies, damaging the fetal cardiac conduction system. Only in a third of infants with immune-mediated CAVB is a well-defined autoimmune disease known in the mother. During fetal life, fetal echocardiography still represents the gold standard for the diagnosis of CAVB. Two major negative prognostic markers are low ventricular rate and the appearance of fetal hydrops. As regards prognosis, a risk for heart failure, syncope, and sudden death is present both during fetal and postnatal life. Dilated cardiomyopathy represents another complication in CAVB history, with different possible aetiologies (right ventricular permanent pacing, reactivation of autoimmune myocarditis). The indications for pacemaker implantation in patients with CAVB are similar to those for acquired heart disease, with some special technical considerations due to young age (epicardial versus endocardial systems, pacing site, etc.). As a future perspective, leadless cardiac stimulation in children with CAVB may represent a definitive solution and an answer to many questions.

scholarly journals Outcome of Antibody‐Mediated Fetal Heart Disease With Standardized Anti‐Inflammatory Transplacental Treatment

2022 ◽  
Author(s):  
Wadi Mawad ◽  
Lisa Hornberger ◽  
Bettina Cuneo ◽  
Marie‐Josée Raboisson ◽  
Anita J. Moon‐Grady ◽  
...  
Keyword(s):  
Heart Disease ◽  
Immune Globulin ◽  
Atrioventricular Block ◽  
Fetal Heart ◽  
Survival Rates ◽  
Intravenous Immune Globulin ◽  
Ventricular Rate ◽  
Endocardial Fibroelastosis ◽  
Immune Mediated ◽  
The Impact

Background Transplacental fetal treatment of immune‐mediated fetal heart disease, including third‐degree atrioventricular block (AVB III) and endocardial fibroelastosis, is controversial. Methods and Results To study the impact of routine transplacental fetal treatment, we reviewed 130 consecutive cases, including 108 with AVB III and 22 with other diagnoses (first‐degree/second‐degree atrioventricular block [n=10]; isolated endocardial fibroelastosis [n=9]; atrial bradycardia [n=3]). Dexamethasone was started at a median of 22.4 gestational weeks. Additional treatment for AVB III included the use of a β‐agonist (n=47) and intravenous immune globulin (n=34). Fetal, neonatal, and 1‐year survival rates with AVB III were 95%, 93%, and 89%, respectively. Variables present at diagnosis that were associated with perinatal death included an atrial rate <90 beats per minute (odds ratio [OR], 258.4; 95% CI, 11.5–5798.9; P <0.001), endocardial fibroelastosis (OR, 28.9; 95% CI, 1.6–521.7; P <0.001), fetal hydrops (OR, 25.5; 95% CI, 4.4–145.3; P <0.001), ventricular dysfunction (OR, 7.6; 95% CI, 1.5–39.4; P =0.03), and a ventricular rate <45 beats per minute (OR, 12.9; 95% CI, 1.75–95.8; P =0.034). At a median follow‐up of 5.9 years, 85 of 100 neonatal survivors were paced, and 1 required a heart transplant for dilated cardiomyopathy. Cotreatment with intravenous immune globulin was used in 16 of 22 fetuses with diagnoses other than AVB III. Neonatal and 1‐year survival rates of this cohort were 100% and 95%, respectively. At a median age of 3.1 years, 5 of 21 children were paced, and all had normal ventricular function. Conclusions Our findings reveal a low risk of perinatal mortality and postnatal cardiomyopathy in fetuses that received transplacental dexamethasone±other treatment from the time of a new diagnosis of immune‐mediated heart disease.

Minimally invasive permanent pacemaker implantation immediately after birth: from delivery room to heart surgery

2021 ◽  
pp. 1-3
Author(s):  
Mehmet Taşar ◽  
Nur Dikmen Yaman ◽  
Burcu Arıcı ◽  
Ömer Nuri Aksoy ◽  
Huseyin Dursin ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Atrioventricular Block ◽  
Heart Surgery ◽  
Pacemaker Implantation ◽  
Skin Incision ◽  
Permanent Pacemaker ◽  
Invasive Technique ◽  
Permanent Pacemaker Implantation ◽  
Congenital Atrioventricular Block ◽  
The Right

Abstract Introduction: Congenital atrioventricular block is diagnosed in uterine life, at birth, or early in life. Atrioventricular blocks can be life threatening immediately at birth so urgent pacemaker implantation techniques are requested. Reasons can be cardiac or non-cardiac, but regardless of the reason, operations are challenging. We aimed to present technical procedure and operative results of pacemaker implantation in neonates. Materials and methods: Between June 2014 and February 2021, 10 neonates who had congenital atrioventricular block underwent surgical operation to implant permanent epicardial pacemaker by using minimally invasive technique. Six of the patients were female and four of them were male. Mean age was 4.3 days (0–11), while three of them were operated on the day of birth. Mean weight was 2533 g (1200–3300). Results: Operations were achieved through subxiphoidal minimally skin incision. Epicardial 25 mm length dual leads were implanted on right ventricular surface and generators were fixed on the right (seven patients) or left (three patients) diaphragmatic surface by incising pleura. There were no complication, morbidity, and mortality related to surgery. Conclusion: Few studies have characterised the surgical outcomes following epicardial permanent pacemaker implantation in neonates. The surgical approach is attractive and compelling among professionals so we aimed to present the techniques and results in patients who required permanent pacemaker implantation in the first month of life.

Anatomical predictors of high grade atrioventricular conduction disorders in patients with specific pacemaker implanted after TAVI

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
P Frey ◽  
A Brochier ◽  
N Nezzouhairi ◽  
D Irles
Keyword(s):  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Atrioventricular Conduction ◽  
High Grade ◽  
Av Block ◽  
Conduction Disorders ◽  
Transcatheter Aortic Valve ◽  
The Difference ◽  
Valve Implantation ◽  
Membranous Septum

Abstract Funding Acknowledgements Type of funding sources: None. OnBehalf STIM TAVI-MS study Background  The evolution of atrioventricular conduction disorders after transcatheter aortic valve implantation (TAVI) remains poorly understood. Recent studies have identified short membranous septum (MS) length, deep implantation depth (ID) or their relation as anatomic risk of high-grade AV block and permanent pacemaker implantation. Purpose : We sought to examine whether the atrioventricular (AV) membranous septum (MS) measured by computed tomography (CT) and the depth of valve implantation measured from the final aortic angiogram could predict high-grade atrioventricular block (HG AVB) after TAVI, based on specific pacemaker memory data. Methods STIM-TAVI-MS was a prospective, multicentre observational study that enrolled patients implanted with a specific dual chamber pacemaker after TAVI, with the SafeR algorithm activated, allowing continuous monitoring of atrioventricular conduction. The primary endpoint was the occurrence of late (after Day 7) high-grade atrioventricular block(s) during the year after TAVI. We measured on CT scans the coronal MS lenght, infra-annular MS lenght and the quantification of calcifications, on the final angiogram after TAVI we measured the implant depth (ID) to identifie ΔID-MS corresponding to the difference between implant depth and MS length. The variables were compared with the occurrence of late HG-AVB on PM memory data. Results : Among 82 patients, (mean age 84,5 years ± 4,8, self-expending valve n = 24, 29,3%), n = 47 (57.3%) had ≥1 late high-grade atrioventricular block. Mean coronal MS length was 8,1 ± 2,5 mm, mean infra annular MS was 3,4 ± 3.1 mm, mean calcification volume was 93.0 ± 88, 85,5± 104 and 141,5 ± 137,5 mm3 for the noncoronary, right coronary and left coronary cusp respectively. Mean implant depth was 7,3 ± 3,3mm, and mean ΔID-MS = -0,7 ± 4,1mm. There were no association between MS length (OR = 1,06; CI 0,91 to 1,24), ID (OR = 1,6; CI 0,85 to 2,9), nor ΔID-MS (OR = 0,67; CI 0,37 to 1,23) and late HG AVB. Calcification volume were not associated with late HG AVB. Conclusion In an high risk high grade AV block population after TAVI, anatomical analysis of MS length, degree of calcification, implant depth and ΔID-MS did not predict occurrence of late (&gt; day 7 after TAVI ) high grade AV Blocks. Abstract Figure. CT scan A : aortic plane, B : MS lenght

A premature low-birth-weight infant with congenital complete atrioventricular block and myocarditis successfully treated by staged pacemaker implantation

2016 ◽  
Vol 26 (5) ◽  
pp. 1029-1032
Author(s):  
Tao Fujioka ◽  
Masaki Nii ◽  
Yasuhiko Tanaka
Keyword(s):  
Birth Weight ◽  
Low Birth Weight ◽  
Atrioventricular Block ◽  
Pacemaker Implantation ◽  
Myocardial Damage ◽  
Ventricular Rate ◽  
Complete Atrioventricular Block ◽  
Low Birth Weight Infant ◽  
Congenital Complete Atrioventricular Block ◽  
Complete Atrioventricular

AbstractCongenital complete atrioventricular block is a known lethal condition. Although antenatal diagnosis and the technical advances of pacemaker treatment have reduced its mortality, treatment of premature babies with significant myocardial damage remains a challenge. In this paper, we report the case of a premature low-birth-weight infant with congenital complete atrioventricular block and extremely low ventricular rate, fetal hydrops, and myocarditis who was successfully treated with staged permanent pacemaker implantation.

Anatomy and physiology of the atrioventricular node

ESC CardioMed ◽  
2018 ◽  
pp. 1957-1958
Author(s):  
M. J. Pekka Raatikainen
Keyword(s):  
Atrioventricular Node ◽  
Cardiac Conduction ◽  
Ventricular Rate ◽  
Pacemaker Activity ◽  
Av Block ◽  
Anatomy And Physiology ◽  
Specialized Tissue ◽  
Crucial Part ◽  
Decremental Conduction ◽  
Sinoatrial Node Dysfunction

The atrioventricular node (AVN) and the surrounding area is a crucial part of the cardiac conduction system. It consists of specialized tissue located at the base of the atrial septum within the triangle of Koch. The inherent physiological function of the AVN is to delay cardiac impulse propagation between the atria and the ventricles, and to function as a backup pacemaker in the setting of sinoatrial node dysfunction or advanced atrioventricular (AV) block. AV nodal conduction and pacemaker activity are under strict control by the autonomic nervous system. Due to the unique property of decremental conduction, the AVN protects the heart from an excessive ventricular rate during rapid atrial arrhythmias. On the other hand, the AVN is also an important source of brady- and tachyarrhythmias, and a target for various pharmacological and non-pharmacological arrhythmia therapies.

scholarly journals Aetiologies and temporal trends of atrioventricular block in young patients: a 20-year nationwide study

EP Europace ◽  
2019 ◽  
Vol 21 (11) ◽  
pp. 1710-1716 ◽  
Author(s):  
Johnni Rudbeck-Resdal ◽  
Morten K Christiansen ◽  
Jens B Johansen ◽  
Jens C Nielsen ◽  
Henning Bundgaard ◽  
...  
Keyword(s):  
Heart Disease ◽  
Atrioventricular Block ◽  
Temporal Trends ◽  
Pacemaker Implantation ◽  
Young Patients ◽  
Unknown Aetiology ◽  
Number Of Patients ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Abstract Aims To describe aetiologies and temporal trends in young patients with atrioventricular block (AVB). Methods and results We identified all patients in Denmark, receiving their first pacemaker because of AVB before the age of 50 years between 1996 and 2015. Medical records were reviewed and clinical information and diagnostic work-up results were obtained to evaluate the aetiology. We used Poisson regression testing for temporal trends. One thousand and twenty-seven patients were identified, median age at time of implantation was 38 (interquartile range 25–45) years, 584 (56.9%) were male. The aetiologies were complications to cardiac surgery [n = 157 (15.3%)], congenital AVB [n = 93 (9.0%)], cardioinhibitory reflex [n = 52 (5.0%)], congenital heart disease [n = 43 (4.2%)], complication to radiofrequency ablation [n = 35 (3.4%)], cardiomyopathy [n = 31 (3.0%)], endocarditis [n = 18 (1.7%)], muscular dystrophy [n = 14 (1.4%)], ischaemic heart disease [n = 14 (1.4%)], sarcoidosis [n = 11 (1.1%)], borreliosis [n = 9 (0.9%)], hereditary [n = 6 (0.6%)], side-effect to antiarrhythmics [n = 6 (0.6%)], planned His-ablation [n = 5 (0.5%)], complication to alcohol septal ablation [n = 5 (0.5%)], and other known aetiologies [n = 11 (1.1%)]. The aetiology remained unknown in 517 (50.3%) cases. While the number of patients with unknown aetiology increased during the study period (P < 0.001), we observed no significant change in the number of patients with identified aetiology (P = 0.35). Conclusion In a nationwide cohort, the aetiology of AVB was identified in only half the patients younger than 50 years referred for first-time pacemaker implantation. The number of patients with unknown aetiology increased during the study period. These findings indicate need for better insight into aetiologies of AVB and improved diagnostic work-up guidelines.

scholarly journals Maternal steroid therapy for fetuses with second-degree immune-mediated congenital atrioventricular block: a systematic review and meta-analysis

2018 ◽  
Vol 97 (7) ◽  
pp. 787-794 ◽  
Author(s):  
Andrea Ciardulli ◽  
Francesco D'Antonio ◽  
Elena R. Magro-Malosso ◽  
Lamberto Manzoli ◽  
Paul Anisman ◽  
...  
Keyword(s):  
Systematic Review ◽  
Atrioventricular Block ◽  
Steroid Therapy ◽  
Meta Analysis ◽  
Immune Mediated ◽  
Congenital Atrioventricular Block ◽  
Second Degree

scholarly journals Congenital complete atrioventricular block from literature to clinical approach – a case series and literature review

2020 ◽  
Author(s):  
Liliana Gozar ◽  
Claudiu Marginean ◽  
Amalia Fagarasan ◽  
Iolanda Muntean ◽  
Andreea Cerghit-Paler ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Atrioventricular Block ◽  
Speckle Tracking ◽  
Pacemaker Implantation ◽  
Case Series ◽  
Clinical Approach ◽  
Intrauterine Death ◽  
Congenital Atrioventricular Block ◽  
Therapeutic Behavior

Aim: Congenital atrioventricular block (CAVB) is an immunological condition, secondary to the transfer of maternal Ig G antibodies from seropositive mothers. Although the presence of these antibodies is high among pregnant women, the preva-lence of this fetal pathology is low. The aim of this paper is to analyze a series of cases with intrauterine diagnosis of CAVB and to present their follow-up protocol. Material and method: In the period between 2013-2020, five fetuses were diagnosed and followed up in the Pediatric Cardiology Clinic. In each of the cases, assessment of the hemodynamic status was done by calculation of the fetal cardiovascular profile score (CVPS). In the last cases the follow-up protocol was supplemented with longitudinal speckle tracking evaluation of the ventricular function. Results: In the present series, intrauterine death occurred in one case; in another case resumption of atrioventricular conduction was observed. Epicardial pacemaker implantation was required in three of the patients. Conclusion: Completing the evaluation of ventricular function with the longitudinal speckle tracking method in fetuses and newborn patients with congenital atrioventricular block may play an important role in establish-ing therapeutic behavior.

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