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1.
Article in English | MEDLINE | ID: mdl-38642206

ABSTRACT

BACKGROUND: Cryoablation of APs localized near the atrioventricular (AV) junction is a well-established ablation strategy in children, and it has proved to be very safe. However, recurrence rates remain considerable for specific accessory pathway (AP) localizations. The aim of this retrospective study was to evaluate the efficacy and safety of a trans-jugular approach for cryoablation of right anterior, anterior-lateral, and anterior-septal APs in children, as compared to the conventional femoral approach. METHODS: From June 2019 to November 2023, 24 consecutive patients (mean age 13.2 ± 4.6, 12 males (50% of total cohort)) with right anterior-lateral, anterior, and anterior-septal/para-Hisian APs underwent 3D non-fluoroscopic transcatheter cryoablation through the right jugular vein at our Institution. Ablation results were compared with 24 patients for whom a conventional trans-femoral approach was used. RESULTS: Acute procedural success rate was 100% (n = 24/24), with a non-statistically significant difference as compared to the control group (100% vs. 83%, p = 0.1). During follow-up (1.1 years, interquartile range 0.6-1.3), one patient (4%) had a recurrence in the trans-jugular group, as opposed to eight (38%) in the control group (p = 0.006). No permanent complications occurred. CONCLUSIONS: 3D cryoablation of right anterior-lateral, anterior, and anterior-septal/para-Hisian APs in children using a trans-jugular approach is extremely effective and safe, resulting in higher chronic success rate compared to the conventional femoral approach.

2.
J Cardiovasc Dev Dis ; 11(4)2024 Mar 27.
Article in English | MEDLINE | ID: mdl-38667717

ABSTRACT

Guidelines are important tools to guide the diagnosis and treatment of patients to improve the decision-making process of health professionals. They are periodically updated according to new evidence. Four new Guidelines in 2021, 2022 and 2023 referred to pediatric pacing and defibrillation. There are some relevant changes in permanent pacing. In patients with atrioventricular block, the heart rate limit in which pacemaker implantation is recommended was decreased to reduce too-early device implantation. However, it was underlined that the heart rate criterion is not absolute, as signs or symptoms of hemodynamically not tolerated bradycardia may even occur at higher rates. In sinus node dysfunction, symptomatic bradycardia is the most relevant recommendation for pacing. Physiological pacing is increasingly used and recommended when the amount of ventricular pacing is presumed to be high. New recommendations suggest that loop recorders may guide the management of inherited arrhythmia syndromes and may be useful for severe but not frequent palpitations. Regarding defibrillator implantation, the main changes are in primary prevention recommendations. In hypertrophic cardiomyopathy, pediatric risk calculators have been included in the Guidelines. In dilated cardiomyopathy, due to the rarity of sudden cardiac death in pediatric age, low ejection fraction criteria were demoted to class II. In long QT syndrome, new criteria included severely prolonged QTc with different limits according to genotype, and some specific mutations. In arrhythmogenic cardiomyopathy, hemodynamically tolerated ventricular tachycardia and arrhythmic syncope were downgraded to class II recommendation. In conclusion, these new Guidelines aim to assess all aspects of cardiac implantable electronic devices and improve treatment strategies.

3.
Pacing Clin Electrophysiol ; 47(5): 635-641, 2024 May.
Article in English | MEDLINE | ID: mdl-38552167

ABSTRACT

BACKGROUND: 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). METHODS: 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). RESULTS: 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. CONCLUSION: 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.


Subject(s)
Pacemaker, Artificial , Humans , Male , Female , Retrospective Studies , Child , Child, Preschool , Electrodes, Implanted , Atrioventricular Block/therapy , Sick Sinus Syndrome/therapy
4.
Pacing Clin Electrophysiol ; 46(10): 1162-1169, 2023 10.
Article in English | MEDLINE | ID: mdl-37614072

ABSTRACT

BACKGROUND: Cardiac pacing from right ventricular (RV) sites may cause electromechanical ventricular dyssynchrony. Invasive and noninvasive mapping studies showed left ventricular (LV) activation sequence in adults. Aim of this study was to seek out the LV endocardial activation (LVEA) in pediatric patients who underwent RV pacing. METHODS: Single-center, prospective study conducted on pediatric patients who underwent left sided catheter ablation of accessory pathways with the Carto Univu mapping system. After successful ablation procedures, LVEA was recorded by the ablation catheter during sinus rhythm (SR) and during para-hisian (PHP), midseptum (MSP), and apical (RVAP) pacing. RESULTS: Seventeen patients, 13 males, aged 12 (10-15) years, registered LV activation maps and times (LVAT). SR showed significantly shorter LVAT than during pacing. LVAT of PHP was shorter than MSP, while there were not significant differences among PHP and MSP versus RVAP. In SR initial LV endocardial activation occurred in two midseptum sites, inferior-posterior and superior-anterior. During PHP, initial activation occurred at parahisian basal septum, rapidly followed by midseptum as in SR. During MSP and RVAP initial activation occurred at midseptum and apex, respectively. From all initial sites, the excitation spreads toward the base of the lateral LV free wall. A mild linear correlation was found between QRS duration and LVAT for MSP and for PHP. CONCLUSIONS: In pediatric patients LVEA maps during RV pacing showed that the shortest LVAT was obtained with PHP. The LV activation pattern seemed similar in sinus rhythm, PHP and MSP, from midseptum to LV lateral base.


Subject(s)
Cardiac Pacing, Artificial , Ventricular Function, Left , Male , Adult , Humans , Child , Prospective Studies , Ventricular Function, Left/physiology , Cardiac Pacing, Artificial/methods , Heart Ventricles/surgery , Arrhythmias, Cardiac
5.
Pediatr Cardiol ; 44(8): 1736-1740, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37597120

ABSTRACT

The autosomal recessive (AR) form of Long QT Syndrome (LQTS) is described both associated with deafness known as Jervell and Lange-Nielsen (JLN) syndrome, and without deafness (WD). The aim of the study is to report the characteristics of AR LQTS patients and the efficacy of the therapy. Data of all children with AR LQTS referred to the Bambino Gesù Children's Hospital IRCCS from September 2012 to September 2021were included. Three (30%) patients had compound heterozygosity and 7 (70%) had homozygous variants of the KCNQ1 gene, the latter showing deafness. Four patients (40%) presented aborted sudden cardiac death (aSCD): three with previous episodes of syncope (75%), the other without previous symptoms (16.6% of asymptomatic patients). An episode of aSCD occurred in 2/3 (66.7%) of WD and heterozygous patients, while in 2/7 (28%) JLN and homozygous patients and in 2/2 patients with QTC > 600 ms. All patients were treated with Nadolol. In 5 Mexiletine was added, shortening QTc and obtaining the disappearance of the T-wave alternance (TWA) in 3/3. Episodes of aSCD seem to be more frequent in LQTS patients with compound heterozygous variants and WD than in those with JLN and homozygous variants. Episodes of aSCD also appear more frequent in children with syncope or with QTc value > 600 ms, even on beta-blocker therapy, than in patients without syncope or with Qtc < 600 ms. However, our descriptive results should be confirmed by larger studies. Moreover, Mexiletine addition reduced QTc value and eliminated TWA.


Subject(s)
Deafness , Heart Arrest , Jervell-Lange Nielsen Syndrome , Long QT Syndrome , Child , Humans , KCNQ1 Potassium Channel/genetics , Mexiletine/therapeutic use , Long QT Syndrome/drug therapy , Long QT Syndrome/genetics , Long QT Syndrome/diagnosis , Jervell-Lange Nielsen Syndrome/drug therapy , Jervell-Lange Nielsen Syndrome/genetics , Jervell-Lange Nielsen Syndrome/diagnosis , Syncope/genetics
6.
Europace ; 25(9)2023 08 02.
Article in English | MEDLINE | ID: mdl-37536671

ABSTRACT

AIMS: Patients with cardiomyopathies and channelopathies are usually younger and have a predominantly arrhythmia-related prognosis; they have nearly normal life expectancy thanks to the protection against sudden cardiac death provided by the implantable cardioverter defibrillator (ICD). The subcutaneous ICD (S-ICD) is an effective alternative to the transvenous ICD and has evolved over the years. This study aimed to evaluate the rate of inappropriate shocks (IS), appropriate therapies, and device-related complications in patients with cardiomyopathies and channelopathies who underwent modern S-ICD implantation. METHODS AND RESULTS: We enrolled consecutive patients with cardiomyopathies and channelopathies who had undergone implantation of a modern S-ICD from January 2016 to December 2020 and who were followed up until December 2022. A total of 1338 S-ICD implantations were performed within the observation period. Of these patients, 628 had cardiomyopathies or channelopathies. The rate of IS at 12 months was 4.6% [95% confidence interval (CI): 2.8-6.9] in patients with cardiomyopathies and 1.1% (95% CI: 0.1-3.8) in patients with channelopathies (P = 0.032). No significant differences were noted over a median follow-up of 43 months [hazard ratio (HR): 0.76; 95% CI: 0.45-1.31; P = 0.351]. The rate of appropriate shocks at 12 months was 2.3% (95% CI: 1.1-4.1) in patients with cardiomyopathies and 2.1% (95% CI: 0.6-5.3) in patients with channelopathies (P = 1.0). The rate of device-related complications was 0.9% (95% CI: 0.3-2.3) and 3.2% (95% CI: 1.2-6.8), respectively (P = 0.074). No significant differences were noted over the entire follow-up. The need for pacing was low, occurring in 0.8% of patients. CONCLUSION: Modern S-ICDs may be a valuable alternative to transvenous ICDs in patients with cardiomyopathies and channelopathies. Our findings suggest that modern S-ICD therapy carries a low rate of IS. CLINICAL TRIAL REGISTRATION: URL: http://clinicaltrials.gov/Identifier: NCT02275637.


Subject(s)
Cardiomyopathies , Channelopathies , Defibrillators, Implantable , Humans , Defibrillators, Implantable/adverse effects , Channelopathies/complications , Channelopathies/therapy , Treatment Outcome , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Cardiomyopathies/complications , Cardiomyopathies/therapy , Registries
7.
J Cardiovasc Med (Hagerstown) ; 24(9): 589-601, 2023 09 01.
Article in English | MEDLINE | ID: mdl-37409656

ABSTRACT

Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediatric age. PSVT may occur at any age, from fetus to adulthood, and its symptoms range from none to syncope or heart failure. VP too can range from no symptoms to sudden cardiac death. Therefore, these arrhythmias frequently need risk stratification, electrophysiologic study, drug or ablation treatment. In this review of the literature, recommendations are given for diagnosis and treatment of fetal and pediatric age (≤12 years) WPW, VP, PSVT, and criteria for sport participation.


Subject(s)
Accessory Atrioventricular Bundle , Tachycardia, Paroxysmal , Tachycardia, Ventricular , Wolff-Parkinson-White Syndrome , Humans , Child , Infant, Newborn , Infant , Child, Preschool , Wolff-Parkinson-White Syndrome/complications , Wolff-Parkinson-White Syndrome/diagnosis , Wolff-Parkinson-White Syndrome/surgery , Electrocardiography , Tachycardia, Paroxysmal/diagnosis , Tachycardia, Paroxysmal/surgery , Fetus
8.
Europace ; 25(4): 1482-1490, 2023 04 15.
Article in English | MEDLINE | ID: mdl-36794445

ABSTRACT

AIMS: In congenitally corrected transposition of the great arteries (CCTGA) the right ventricle (RV) is systemic. Atrioventricular block (AVB) and systolic dysfunction are frequently observed. Permanent pacing of the subpulmonary left ventricle (LV) may worsen RV dysfunction. The aim of this study was to seek out if LV conduction system pacing (LVCSP) guided by three-dimensional-electroanatomic mapping systems (3D-EAMs) can preserve RV systolic function in paediatric CCTGA patients with AVB. METHODS AND RESULTS: Retrospective analysis of CCTGA patients who underwent 3D-EAM-guided LVCSP. Three-dimensional-pacing map guided lead implantation towards septal sites with narrower paced QRS. Electrocardiograms (ECGs), echocardiograms, and lead parameters (threshold, sensing, and impedance) were compared at baseline (pre-implantation) and at 1-year follow-up. Right ventricle function was evaluated by 3D ejection fraction (EF), fractional area change (FAC), RV global longitudinal strain (GLS). Data are reported as median (25th-75th centiles). Seven CCTGA patients aged 15 (9-17) years, with complete/advanced AVB (4 with prior epicardial pacing), underwent 3D-guided LVCSP (5 DDD, 2 VVIR). Baseline echocardiographic parameters were impaired in most patients. No acute/chronic complications occurred. Ventricular pacing was >90%. At 1-year follow-up QRS duration showed no significant changes compared with baseline; however, QRS duration shortened in comparison with prior epicardial pacing. Lead parameters remained acceptable despite ventricular threshold increased. Systemic RV function was preserved: FAC and GLS improved significantly, and all patients showed normal RV EF (>45%). CONCLUSION: Three-dimensional-EAM-guided LVCSP preserved RV systolic function in paediatric patients with CCTGA and AVB after short-term follow-up.


Subject(s)
Atrioventricular Block , Transposition of Great Vessels , Humans , Child , Congenitally Corrected Transposition of the Great Arteries/complications , Transposition of Great Vessels/complications , Transposition of Great Vessels/diagnostic imaging , Transposition of Great Vessels/therapy , Retrospective Studies , Heart Conduction System , Heart Ventricles/diagnostic imaging , Cardiac Pacing, Artificial/methods
9.
Pacing Clin Electrophysiol ; 46(6): 543-547, 2023 06.
Article in English | MEDLINE | ID: mdl-36815543

ABSTRACT

The recently published "electrophysiologically guided low-voltage bridge (LVB) strategy" is effective in the ablation of atrioventricular nodal re-entry tachycardia (AVNRT) in children. This study aimed to evaluate its efficacy and safety in children <26 kgs. Fourteen children [64% males, median age 6.5 years (IQR 6-8 years), median weight 25.5 kg (IQR 24-26 kg)] with AVNRT were treated. In all patients, we detected a LVB associated to a typical slow pathway potential. The acute success rate was 100% with a mean of 5.5 cryoablation deliveries. All procedures were performed with near-zero fluoroscopic exposure (median time 0.15 min, IQR 0-0.7 min), in six patients fluoroscopy was 0 min. There were no complications or recurrences during the follow-up (median 20.91 months, IQR 11.7-26.7 months).


Subject(s)
Catheter Ablation , Cryosurgery , Tachycardia, Atrioventricular Nodal Reentry , Male , Humans , Child , Female , Cryosurgery/methods , Treatment Outcome , Action Potentials , Time Factors , Recurrence
10.
Cardiol Young ; 33(5): 760-765, 2023 May.
Article in English | MEDLINE | ID: mdl-35673795

ABSTRACT

BACKGROUND: 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. AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.


Subject(s)
Atrioventricular Block , Cardiac Pacing, Artificial , Adolescent , Humans , Child , Atrioventricular Block/therapy , Retrospective Studies , Treatment Outcome , Ventricular Function, Left
11.
Europace ; 25(2): 460-468, 2023 02 16.
Article in English | MEDLINE | ID: mdl-36107451

ABSTRACT

AIMS: Subcutaneous-implantable cardiac defibrillators (S-ICDs) are used increasingly to prevent sudden cardiac death in young patients. This study was set up to gain insight in the indications for S-ICD, possible complications, and their predictors and follow-up results. METHODS AND RESULTS: A multicentre, observational, retrospective, non-randomized, standard-of-care registry on S-ICD outcome in young patients with congenital heart diseases (CHDs), inherited arrhythmias (IAs), idiopathic ventricular fibrillation (IVF), and cardiomyopathies (CMPs). Anthropometry was registered as well as implantation technique, mid-term device-related complications, and incidence of appropriate/inappropriate shocks (IASs). Data are reported as median (interquartile range) or mean ± standard deviation. Eighty-one patients (47% CMPs, 20% CHD, 21% IVF, and 12% IA), aged 15 (14-17) years, with body mass index (BMI) 21.8 ± 3.8 kg/m2, underwent S-ICD implantation (primary prevention in 59%). This was performed with two-incision technique in 81% and with a subcutaneous pocket in 59%. Shock and conditional zones were programmed at 250 (200-250) and 210 (180-240) b.p.m., respectively. No intraoperative complications occurred. Follow up was 19 (6-35) months: no defibrillation failure occurred, 17% of patients received appropriate shocks, 13% of patients received IAS (supraventricular tachycardias 40%, T-wave oversensing 40%, and non-cardiac oversensing 20%). Reprogramming, proper drug therapy, and surgical revision avoided further IAS. Complications requiring surgical revision occurred in 9% of patients, with higher risks in patients with three-incision procedures [hazard ratio (HR) 4.3, 95% confidence interval (95% CI) 0.5-34, P = 0.038] and BMI < 20 (HR 5.1, 95% CI 1-24, P = 0.031). CONCLUSION: This multicentre European paediatric registry showed good S-ICD efficacy and safety in young patients. Newer implantation techniques and BMI > 20 showed better outcome.


Subject(s)
Defibrillators, Implantable , Heart Defects, Congenital , Humans , Child , Young Adult , Retrospective Studies , Treatment Outcome , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/therapy , Defibrillators, Implantable/adverse effects , Death, Sudden, Cardiac/epidemiology , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Registries , Heart Defects, Congenital/complications , Heart Defects, Congenital/diagnosis , Heart Defects, Congenital/epidemiology
12.
J Cardiovasc Dev Dis ; 9(2)2022 Jan 20.
Article in English | MEDLINE | ID: mdl-35200687

ABSTRACT

BACKGROUND: Pediatric patients with cardiomyopathies are at risk of malignant arrhythmias and sudden cardiac death (SCD). An ICD may prevent SCD. The aim of this study was to evaluate ICD implantation outcomes, and to compare transvenous and subcutaneous ICDs (S-ICDs) implanted in pediatric patients with cardiomyopathies. METHODS: The study is single center and retrospective, and includes pediatric patients with cardiomyopathies who required ICD implantation (2010-2021). Outcomes were recorded for appropriate/inappropriate ICD therapy and surgical complications. Transvenous ICD and S-ICD were compared. Data are presented as median values (25th-75th centiles). RESULTS: Forty-four patients with cardiomyopathies (hypertrophic 39%, arrhythmogenic 32%, dilated 27%, and restrictive 2%) underwent transvenous (52%) and S-ICD (48%) implantation at 14 (12-17) years of age, mostly for primary prevention (73%). The follow-up period was 29 (14-60) months. Appropriate ICD therapies were delivered in 25% of patients, without defibrillation failures. Lower age at implantation and secondary prevention were significant risk factors for malignant ventricular arrhythmias that required appropriate ICD therapies. ICD-related complications were surgical complications (18%) and inappropriate shocks (7%). No significant differences in outcomes were recorded, either when comparing transvenous and S-ICD or comparing the different cardiomyopathies. CONCLUSIONS: In pediatric patients with cardiomyopathy, ICD therapy is effective, with a low rate of inappropriate shocks. Neither ICD type (transvenous and S-ICDs) nor the cardiomyopathies subgroup revealed divergent outcomes.

13.
J Cardiovasc Dev Dis ; 9(2)2022 Jan 31.
Article in English | MEDLINE | ID: mdl-35200700

ABSTRACT

Cardiomyopathy (CMP) is a rare disease in the pediatric population, with a high risk of morbidity and mortality. The genetic etiology of CMPs in children is extremely heterogenous. These two factors play a major role in the difficulties of establishing standard diagnostic and therapeutic protocols. Isolated CMP in children is a frequent finding, mainly caused by sarcomeric gene variants with a detection rate that can reach up to 50% of analyzed cohorts. Complex multisystemic forms of pediatric CMP are even more heterogenous. Few studies in literature take into consideration this topic as the main core since it represents a rarity (systemic CMP) within a rarity (pediatric population CMP). Identifying etiology in this cohort is essential for understanding prognosis, risk stratification, eligibility to heart transplantation and/or mechanical-assisted procedures, preventing multiorgan complications, and relatives' recurrence risk calculation. The previous points represent a cornerstone in patients' empowerment and personalized medical care approach. The aim of this work is to propose a new approach for an algorithm in the setting of the diagnostic framework of systemic pediatric CMP. On the other hand, during the literature review, we noticed a relatively common etiologic pattern in some forms of complex/multisystem CMP. In other words, certain syndromes such as Danon, Vici, Alström, Barth, and Myhre syndrome share a common pathway of directly or indirectly defective "autophagy" process, which appears to be a possible initiating/triggering factor for CMPs. This conjoint aspect could be important for possible prognostic/therapeutic implications in this category of patients. However, multicentric studies detailed functional and experimental models are needed prior to deriving conclusions.

14.
Int J Cardiol ; 350: 83-89, 2022 Mar 01.
Article in English | MEDLINE | ID: mdl-34998950

ABSTRACT

INTRODUCTION: The aim of this study was to report clinical and arrhythmic features in a pediatric population affected by arrhythmogenic cardiomyopathy (ACM). Moreover, we assessed the concordance between the 2010 International Task Force criteria (ITF) and the 2020 Padua criteria. METHODS: Inclusion criteria were "definite" or "borderline" ACM diagnosed according to the "Padua criteria" in patients <18 years old. History, electrocardiograms, ECG-holter monitorings, exercise testings, imaging investigations, electrophysiological studies, genetic testings and follow-up data were collected. RESULTS: We enrolled 21 patients (mean age 13.9 ± 2 years). Most of them presented for minor arrhythmias. Premature ventricular complexes burden was 7.9 ± 10%. Cardiac magnetic resonance (19/21, 90.5% patients) showed right ventricular (RV) dilatation, wall motion abnormalities and late gadolinium enhancement (LGE) of both ventricles as predominant features [in 9 patients (52.9%) LGE left ventricle]. Genetic results (19/21 patient) showed compound heterozygous variants in 3/19 patients (15.8%), digenic in 3/19 (15.8%) and single in 6/19 (31.6%). Cardiac defibrillator (ICD) was indicated in 15 patients (71.4%): 6 in class I, 7 in class IIa, 2 in class IIb. Appropriate shocks occurred in 2 patients (13.3%), follow-up 5.46 ± 3.17 years According to 2010 ITF criteria: among the 18 patients with a "definite" ACM diagnosis, one patient would have had a "borderline" diagnosis, three a "possible" diagnosis and one no diagnosis and among the three patients with "borderline" diagnosis two would have had a "possible" diagnosis. CONCLUSIONS: Pediatric ACM can be diagnosed in the majority of cases secondary to incidental finding of simple ventricular arrhythmias. PVC burden is low and exercise induced arrhythmias rarely occur. Few patients with ICD experience appropriate shocks. "Padua criteria" improve the diagnostic accuracy.


Subject(s)
Arrhythmogenic Right Ventricular Dysplasia , Contrast Media , Adolescent , Arrhythmogenic Right Ventricular Dysplasia/diagnostic imaging , Arrhythmogenic Right Ventricular Dysplasia/genetics , Child , Electrocardiography , Gadolinium , Heart Ventricles , Humans
16.
J Interv Card Electrophysiol ; 63(3): 621-628, 2022 Apr.
Article in English | MEDLINE | ID: mdl-34697756

ABSTRACT

PURPOSE: Different authors have described three-dimensional (3D) voltage mapping of the Koch's triangle (KT) in order to find low-voltage bridges (LVB) as targets for a successful transcatheter ablation (TCA) of the slow pathway (SP) in children. Recently, the Advisor High Density (HD) Grid™ mapping catheter was introduced as new multipolar catheter for HD mapping. The aim of the study was to describe our preliminary experience with the use of HD Grid™ catheter in LVB and electrophysiologically guided cryoablation of SP in children. METHODS: Twenty-one children (mean age 13±3 years) with atrioventricular nodal re-entrant tachycardia (AVNRT) underwent cryoablation of SP guided by voltage HD mapping of the KT using HD Grid™ catheter. In order to better highlight the differences with conventional mapping, point collection was performed in each patient with this new multipolar catheter and with a quadripolar catheter. RESULTS: The conventional mapping collected 871±262 points and used 211±80 points in 887±275 s, whereas HD mapping collected 7468±2947 points, using 604±165 points in 513±181 s (p<0.001). Moreover, the LVB area mapped with HD Grid™ was about one-half smaller and clearly delineated. Cryoablation acute success rate was 100%. Overall median fluoroscopy exposure was 0.08 (0.01-5.42) µGy/m2, with a median fluoroscopy time of 0.1 (0.0-0.6) min. During the follow-up (4.8 ± 3.7 months), there were no recurrences. No complications occurred. CONCLUSIONS: Our preliminary experience shows that HD mapping is faster and offers higher spatial resolution and definition. Procedural time can be reduced maintaining the TCA safe, with reduced fluoroscopy use and success.


Subject(s)
Catheter Ablation , Cryosurgery , Tachycardia, Atrioventricular Nodal Reentry , Adolescent , Child , Cryosurgery/methods , Humans , Preliminary Data , Tachycardia, Atrioventricular Nodal Reentry/diagnostic imaging , Tachycardia, Atrioventricular Nodal Reentry/surgery , Treatment Outcome
17.
Cardiol Young ; 32(8): 1229-1234, 2022 Aug.
Article in English | MEDLINE | ID: mdl-34586049

ABSTRACT

INTRODUCTION: The aim of the study was to revise our more recent experience about epicardial posterior-septal accessory pathways radiofrequency transcatheter ablation in children and young patients using a transvenous approach through the coronary sinus, to understand if new mapping and ablation technologies can increase success rate and safety. METHODS AND RESULTS: Twenty children (mean age 13 ± 3 years) with epicardial posterior-septal accessory pathways (14 in coronary sinus and 6 in the middle cardiac vein) underwent radiofrequency transcatheter ablation with CARTO-3® system with help of the CARTO-Univu® module. Acute success rate was 73%. No patient was lost to follow-up (mean time 11.4 ± 9 months). The recurrence rate was 19%. Two patients underwent a successful redo-procedure; the overall long-term success rate was 65%. Navistar® catheter presented the highest acute success rate in the coronary sinus. Navistar SmartTouch® was the only catheter that did not present recurrences after the acute success, and it was successfully used in two patients previously unsuccessfully treated with a Navistar ThermoCool®. Acute success rate was 79% without image integration with angio-CT, while it was 63% after the introduction of CARTO-Merge®. CONCLUSION: Epicardial posterior-septal accessory pathways can be definitively eliminated by transvenous radiofrequency transcatheter ablation in more than half of the cases in children. Acute success rate does not seem to depend on catheters used, but contact-force catheter seems to be useful in cases with recurrences. Image integration with cardiac-CT does not increase success rate, but it is useful to detect coronary sinus alterations to better guide ablation strategy.


Subject(s)
Accessory Atrioventricular Bundle , Catheter Ablation , Coronary Sinus , Adolescent , Catheter Ablation/methods , Child , Humans , Technology
20.
JACC Clin Electrophysiol ; 7(11): 1437-1472, 2021 11.
Article in English | MEDLINE | ID: mdl-34794667

ABSTRACT

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.


Subject(s)
Cardiology , Defibrillators, Implantable , Adult , American Heart Association , Child , Electronics , Humans , Latin America , United States
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