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 ◽  
...  

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.

Author(s):  
Johnni Resdal Dideriksen ◽  
Morten K Christiansen ◽  
Jens B Johansen ◽  
Jens C Nielsen ◽  
Henning Bundgaard ◽  
...  

Abstract Aims Atrioventricular block (AVB) of unknown aetiology is rare in the young, and outcome in these patients is unknown. We aimed to assess long-term morbidity and mortality in young patients with AVB of unknown aetiology. Methods and results We identified all Danish patients younger than 50 years receiving a first pacemaker due to AVB between January 1996 and December 2015. By reviewing medical records, we included patients with AVB of unknown aetiology. A matched control cohort was established. Follow-up was performed using national registries. The primary outcome was a composite endpoint consisting of death, heart failure hospitalization, ventricular tachyarrhythmia, and cardiac arrest with successful resuscitation. We included 517 patients, and 5170 controls. Median age at first pacemaker implantation was 41.3 years [interquartile range (IQR) 32.7–46.2 years]. After a median follow-up of 9.8 years (IQR 5.7–14.5 years), the primary endpoint had occurred in 14.9% of patients and 3.2% of controls [hazard ratio (HR) 3.8; 95% confidence interval (CI) 2.9–5.1; P < 0.001]. Patients with persistent AVB at time of diagnosis had a higher risk of the primary endpoint (HR 10.6; 95% CI 5.7–20.0; P < 0.001), and risk was highest early in the follow-up period (HR 6.8; 95% CI 4.6–10.0; P < 0.001, during 0–5 years of follow-up). Conclusion Atrioventricular block of unknown aetiology presenting before the age of 50 years and treated with pacemaker implantation was associated with a three- to four-fold higher rate of the composite endpoint of death or hospitalization for heart failure, ventricular tachyarrhythmia, or cardiac arrest with successful resuscitation. Patients with persistent AVB were at higher risk. These findings warrant improved follow-up strategies for young patients with AVB of unknown aetiology.


2021 ◽  
pp. 1-3
Author(s):  
Mehmet Taşar ◽  
Nur Dikmen Yaman ◽  
Burcu Arıcı ◽  
Ömer Nuri Aksoy ◽  
Huseyin Dursin ◽  
...  

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.


2021 ◽  
pp. 1-6
Author(s):  
Thomas Huang ◽  
Edward O’Leary ◽  
Mark E. Alexander ◽  
Laura Bevilacqua ◽  
Francis Fynn-Thompson ◽  
...  

Abstract Introduction: Reflex-mediated syncope occurs in 15% of children and young adults. In rare instances, pacemakers are required to treat syncopal episodes associated with transient sinus pauses or atrioventricular block. This study describes a single centre experience in the use of permanent pacemakers to treat syncope in children and young adults. Materials and methods: Patients with significant pre-syncope or syncope and pacemaker implantation from 1978 to 2018 were reviewed. Data collected included the age of presentation, method of diagnosis, underlying rhythm disturbance, age at implant, type of pacemaker implanted, procedural complications and subsequent symptoms. Results: Fifty patients were identified. Median age at time of the first syncopal episode was 10.2 (range 0.3–20.4) years, with a median implant age of 14.9 (0.9–34.3) years. Significant sinus bradycardia/pauses were the predominant reason for pacemaker implant (54%), followed by high-grade atrioventricular block (30%). Four (8%) patients had both sinus pauses and atrioventricular block documented. The majority of patients had dual-chamber pacemakers implanted (58%), followed by ventricular pacemakers (38%). Median follow-up was 6.7 (0.4–33.0) years. Post-implant, 4 (8%) patients continued to have syncope, 7 (14%) had complete resolution of their symptoms, and the remaining reported a decrease in their pre-syncopal episodes and no further syncope. Twelve (24%) patients had complications, including two infections and eight lead malfunctions. Conclusions: Paediatric patients with reflex-mediated syncope can be treated with pacing. Complication rates are high (24%); as such, permanent pacemakers should be reserved only for those in whom asystole from sinus pauses or atrioventricular block has been well documented.


ESC CardioMed ◽  
2018 ◽  
pp. 1966-1968
Author(s):  
Drago Fabrizio ◽  
Battipaglia Irma

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.


2020 ◽  
Vol 31 (3) ◽  
pp. 398-404
Author(s):  
Samuli J Salmi ◽  
Tuomo Nieminen ◽  
Juha Hartikainen ◽  
Fausto Biancari ◽  
Joonas Lehto ◽  
...  

Abstract OBJECTIVES We sought to study the indications, long-term occurrence, and predictors of permanent pacemaker implantation (PPI) after isolated surgical aortic valve replacement with bioprostheses. METHODS The CAREAVR study included 704 patients (385 females, 54.7%) without a preoperative PPI (mean ± standard deviation age 75 ± 7 years) undergoing isolated surgical aortic valve replacement at 4 Finnish hospitals between 2002 and 2014. Data were extracted from electronic patient records. RESULTS The follow-up was median 4.7 years (range 1 day to 12.3 years). Altogether 56 patients received PPI postoperatively, with the median 507 days from the operation (range 6 days to 10.0 years). The PPI indications were atrioventricular block (31 patients, 55%) and sick sinus syndrome (21 patients, 37.5%). For 4 patients, the PPI indication remained unknown. A competing risks regression analysis (Fine–Gray method), adjusted with age, sex, diabetes, coronary artery disease, preoperative atrial fibrillation (AF), left ventricular ejection fraction, New York Heart Association class, AF at discharge and urgency of operation, was used to assess risk factors for PPI. Only AF at discharge (subdistribution hazard ratio 4.34, 95% confidence interval 2.34–8.03) was a predictor for a PPI. CONCLUSIONS Though atrioventricular block is the major indication for PPI after surgical aortic valve replacement, >30% of PPIs are implanted due to sick sinus syndrome during both short-term follow-up and long-term follow-up. Postoperative AF versus sinus rhythm conveys >4-fold risk of PPI. Clinical trial registration clinicaltrials.gov Identifier: NCT02626871


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Lozano Jimenez ◽  
V Monivas Palomero ◽  
J Goirigolzarri Artaza ◽  
S Navarro Rico ◽  
A Borrego Hernandez ◽  
...  

Abstract Background Evolution of left and right ventricular (LV and RV) function after heart transplantation (HT) has not been well described. Our objective was to evaluate the normal evolution of echocardiographic parameters of both ventricles and to explore if there is a link between the decrease of strain values and acute rejection (AR) or coronary allograft vasculopathy (CAV) Methods We followed 29 HT recipients with serial echocardiograms performed between 2011 and 2018, with a median follow-up of 5 years. LV global longitudinal strain (LV GLS) was analyzed by speckle tracking in 12 LV segments in 4 and 2 chamber views, and RV free wall longitudinal strain (RV free Wall LS) was measured in 4 chamber view. Acute rejection was diagnosed by EMB following our HT protocol. We take into consideration only moderate or severe rejection episodes (grade ≥2R).The presence of CAV was studied by coronariography or IVUS one year post-HT. Results As shown in the table below, LVEF was preserved from the begining of the follow up while LV GLS reached the normality in the 6th month, and both remained in normal ranges untill the 5th year. Regarding RV function, TAPSE was impaired in the early post-HT period and increased progressively and reached normality 1 year after HT. RV lateral wall LS rose during follow-up as well, reaching normal values 6 months after HT. Nevertheless, we noticed an impairment in this parameter at 5 years (−20.1±2.7, p=0.001), although it remained within normal ranges compared to guidelines reference parameters. We did not find any correlation between any parameter evaluated and the presence of AR or CAV at five years of follow-up. LV and RV function parameters LVEF LV GLS TAPSE FAC RV free wall LS Basal (14 days) 63.0±7.9 −17.2±3.6 12.1±2.9* 43.7±9.8 −19.3±4.2 3 months 65.0±8.6 −17.7±2.8 14.8±3.4* 45.3±8.2 −22.0±4.6 6 months 65.8±9.6 −18.7±3.4 16.1±3.6 44.6±9.6 −24.6±4.9* 1 year 63.5±8.1 −18.1±2.2 17.1±4.1 44.0±8.1 −26.7±7.1* 2 years 63.8±6.8 −18.3±9.0 19.4±3.7 45.3±7.9 −27.6±6.3* 5 years 64.4±7.3 −18.1±3.3 17.9±3.9 46.6±12.1 −20.1±2.8 P (Anova) 0.85 0.85 <0.001 0.82 <0.001 Conclusion As we show in this series of HT recipients with uneventful postoperative course, all LV and RV function parameters showed normal values 1 year after HT and manteined them during long-term follow-up. The presence of AR or CAV did not have any influence in ventricular function.


2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Sukhdeep Bhogal ◽  
Pooja Sethi ◽  
Yasir Taha ◽  
Muralidhar Papireddy ◽  
Akhilesh Mahajan ◽  
...  

Deglutition syncope is a relatively rare cause of syncope that belongs to the category of neurally mediated reflex syncopal syndromes. The phenomenon is related to vagal reflex in context to deglutition causing atrioventricular block and acute reduction in cardiac output leading to dizziness or syncope. We present case series of two cases of deglutition syncope, of which first was managed medically and second with pacemaker implantation.


2012 ◽  
Vol 2012 ◽  
pp. 1-2
Author(s):  
Mariana Paiva ◽  
Vania Ribeiro ◽  
Raquel Garcia ◽  
Sandra Amorim ◽  
Manuel Campelo ◽  
...  

We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.


Sign in / Sign up

Export Citation Format

Share Document