Decrease of implantable cardioverter-defibrillator shock therapy in children: correlation with ICD programming and remote monitoring

Decrease of implantable cardioverter-defibrillator shock therapy in children: correlation with ICD programming and remote monitoring | medRxiv /* */ /* */ <!-- <!-- /*! * yepnope1.5.4 * (c) WTFPL, GPLv2 */ (function(a,b,c){function d(a){return"[object Function]"==o.call(a)}function e(a){return"string"==typeof a}function f(){}function g(a){return!a||"loaded"==a||"complete"==a||"uninitialized"==a}function h(){var a=p.shift();q=1,a?a.t?m(function(){("c"==a.t?B.injectCss:B.injectJs)(a.s,0,a.a,a.x,a.e,1)},0):(a(),h()):q=0}function i(a,c,d,e,f,i,j){function k(b){if(!o&&g(l.readyState)&&(u.r=o=1,!q&&h(),l.onload=l.onreadystatechange=null,b)){"img"!=a&&m(function(){t.removeChild(l)},50);for(var d in y[c])y[c].hasOwnProperty(d)&&y[c][d].onload()}}var j=j||B.errorTimeout,l=b.createElement(a),o=0,r=0,u={t:d,s:c,e:f,a:i,x:j};1===y[c]&&(r=1,y[c]=[]),"object"==a?l.data=c:(l.src=c,l.type=a),l.width=l.height="0",l.onerror=l.onload=l.onreadystatechange=function(){k.call(this,r)},p.splice(e,0,u),"img"!=a&&(r||2===y[c]?(t.insertBefore(l,s?null:n),m(k,j)):y[c].push(l))}function j(a,b,c,d,f){return q=0,b=b||"j",e(a)?i("c"==b?v:u,a,b,this.i++,c,d,f):(p.splice(this.i++,0,a),1==p.length&&h()),this}function k(){var a=B;return a.loader={load:j,i:0},a}var l=b.documentElement,m=a.setTimeout,n=b.getElementsByTagName("script")[0],o={}.toString,p=[],q=0,r="MozAppearance"in l.style,s=r&&!!b.createRange().compareNode,t=s?l:n.parentNode,l=a.opera&&"[object Opera]"==o.call(a.opera),l=!!b.attachEvent&&!l,u=r?"object":l?"script":"img",v=l?"script":u,w=Array.isArray||function(a){return"[object Array]"==o.call(a)},x=[],y={},z={timeout:function(a,b){return b.length&&(a.timeout=b[0]),a}},A,B;B=function(a){function b(a){var a=a.split("!"),b=x.length,c=a.pop(),d=a.length,c={url:c,origUrl:c,prefixes:a},e,f,g;for(f=0;f<d;f++)g=a[f].split("="),(e=z[g.shift()])&&(c=e(c,g));for(f=0;f<b;f++)c=x[f](c);return c}function g(a,e,f,g,h){var i=b(a),j=i.autoCallback;i.url.split(".").pop().split("?").shift(),i.bypass||(e&&(e=d(e)?e:e[a]||e[g]||e[a.split("/").pop().split("?")[0]]),i.instead?i.instead(a,e,f,g,h):(y[i.url]?i.noexec=!0:y[i.url]=1,f.load(i.url,i.forceCSS||!i.forceJS&&"css"==i.url.split(".").pop().split("?").shift()?"c":c,i.noexec,i.attrs,i.timeout),(d(e)||d(j))&&f.load(function(){k(),e&&e(i.origUrl,h,g),j&&j(i.origUrl,h,g),y[i.url]=2})))}function h(a,b){function c(a,c){if(a){if(e(a))c||(j=function(){var a=[].slice.call(arguments);k.apply(this,a),l()}),g(a,j,b,0,h);else if(Object(a)===a)for(n in m=function(){var b=0,c;for(c in a)a.hasOwnProperty(c)&&b++;return b}(),a)a.hasOwnProperty(n)&&(!c&&!--m&&(d(j)?j=function(){var a=[].slice.call(arguments);k.apply(this,a),l()}:j[n]=function(a){return function(){var b=[].slice.call(arguments);a&&a.apply(this,b),l()}}(k[n])),g(a[n],j,b,n,h))}else!c&&l()}var h=!!a.test,i=a.load||a.both,j=a.callback||f,k=j,l=a.complete||f,m,n;c(h?a.yep:a.nope,!!i),i&&c(i)}var i,j,l=this.yepnope.loader;if(e(a))g(a,0,l,0);else if(w(a))for(i=0;i (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0];var j=d.createElement(s);var dl=l!='dataLayer'?'&l='+l:'';j.src='//www.googletagmanager.com/gtm.js?id='+i+dl;j.type='text/javascript';j.async=true;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-P4HH5NV'); Skip to main content Home About Submit ALERTS / RSS Search for this keyword Advanced Search Decrease of implantable cardioverter-defibrillator shock therapy in children: correlation with ICD programming and remote monitoring View ORCID Profile Robin A. Bertels , View ORCID Profile Eric Boersma , Martijn D. Zeggelaar , Sophie A.F. van Dongen , View ORCID Profile Arend D.J. ten Harkel , Lieselot van Erven , Cynthia Smeding , View ORCID Profile Beatrijs Bartelds , View ORCID Profile Gert van den Berg , Ewout P. Boesaard , Rohit E. Bhagwandien , View ORCID Profile Sing-Chien Yap , View ORCID Profile Reinoud E. Knops , View ORCID Profile Nico A. Blom , View ORCID Profile Janneke A.E. Kammeraad doi: https://doi.org/10.1101/2025.04.08.25325494 Robin A. Bertels a Willem-Alexander Children’s Hospital – Leiden University Medical Center; Department of Pediatric Cardiology ; Albinusdreef 2, Leiden, the Netherlands MD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Robin A. Bertels For correspondence: r.a.bertels{at}lumc.nl Eric Boersma b Erasmus Medical Center; Cardiovascular Institute; Thorax Center; Department of Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands PhD, MSc Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Eric Boersma Martijn D. Zeggelaar c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands Find this author on Google Scholar Find this author on PubMed Search for this author on this site Sophie A.F. van Dongen c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands Find this author on Google Scholar Find this author on PubMed Search for this author on this site Arend D.J. ten Harkel a Willem-Alexander Children’s Hospital – Leiden University Medical Center; Department of Pediatric Cardiology ; Albinusdreef 2, Leiden, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Arend D.J. ten Harkel Lieselot van Erven d Leiden University Medical Center; Department of Cardiology ; Albinusdreef 2, Leiden, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site Cynthia Smeding c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD Find this author on Google Scholar Find this author on PubMed Search for this author on this site Beatrijs Bartelds c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Beatrijs Bartelds Gert van den Berg c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Gert van den Berg Ewout P. Boesaard e Radboud University Medical Center – Amalia Children’s Hospital; Department of Pediatric Cardiology ; Geert Grooteplein Zuid 10, Nijmegen, the Netherlands MD Find this author on Google Scholar Find this author on PubMed Search for this author on this site Rohit E. Bhagwandien b Erasmus Medical Center; Cardiovascular Institute; Thorax Center; Department of Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD Find this author on Google Scholar Find this author on PubMed Search for this author on this site Sing-Chien Yap b Erasmus Medical Center; Cardiovascular Institute; Thorax Center; Department of Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Sing-Chien Yap Reinoud E. Knops f Amsterdam University Medical Centers; Department of Cardiology ; Meibergdreef 9, Amsterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Reinoud E. Knops Nico A. Blom a Willem-Alexander Children’s Hospital – Leiden University Medical Center; Department of Pediatric Cardiology ; Albinusdreef 2, Leiden, the Netherlands g Emma Children’s Hospital – Amsterdam University Medical Centers; Department of Pediatric Cardiology ; Meibergdreef 9, Amsterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Nico A. Blom Janneke A.E. Kammeraad c Erasmus Medical Center – Sophia Children’s Hospital; Cardiovascular Institute; Department of Pediatric Cardiology ; Dr. Molewaterplein 40, Rotterdam, the Netherlands MD, PhD Find this author on Google Scholar Find this author on PubMed Search for this author on this site ORCID record for Janneke A.E. Kammeraad Abstract Full Text Info/History Metrics Data/Code Preview PDF Abstract Background Implantable cardioverter defibrillator (ICD) therapy is effective in preventing sudden cardiac death in children. Unnecessary shocks should be avoided. ICD programming strategies and remote monitoring effective in preventing ICD shock therapy in adults have been applied in children Objectives To investigate the effect of ICD programming and remote monitoring on th incidence of ICD shock therapy in children. Methods Retrospective multi-center study, including children with transvenous or epicardial ICD implantation. During follow-up ICD-shocks, programming variables and use of remote monitoring were collected. Results One-hundred-sixteen children were included, median age 13.4 years (min-max 0.3-18), median follow-up 5.2 years (IQR 3.7-6.6). Fifty-three with an ICD implanted before 2010 and 63 after 2010. The total, appropriate and in appropriate annual shock rate decreased from 10.5% to 8% (difference in mean cumulative function (MCF) P=0.008), 7.8% to 5.8% (MCF P=0.036) and 4.3% to 2.6% (MCF P=0.28) respectively, without increase in cardiac related death. The VF zone was programmed higher (≥210 bpm) in patients before 2010 compared to after 2010 (76% vs 90%; P=0.142), in patients with versus without shocks (79% vs 89%; P=0.243) and at time of appropriate versus in appropriate shocks (86% vs 79%; P=0.0016). Remote monitoring was associated with a decrease of total shocks (MCF P=0.013) and appropriate shocks (MCF P=0.052). Conclusions The incidence of ICD shocks has significantly decreased in children with implantation after 2010. A higher programmed VF-zone and application of remote monitoring are correlated to this decrease and therefore justify its use in strategies to prevent unnecessary shocks in children. Introduction Implantable cardioverter defibrillator (ICD) therapy is effective in preventing sudden cardiac death caused by life threatening arrhythmia in children. The ICD indications and programming strategies for children are extrapolated from adult trials, 1 , 2 there are no randomized trials conducted in children. 3 Children requiring ICD therapy for primary and secondary prevention have a variety of underlying diseases, including hypertrophic and dilated cardiomyopathy, primary electrical disease and/or aborted sudden cardiac death, and a broad spectrum of congenital heart disease. 3 – 5 Several single center and multi-center retrospective ICD studies and prospective ICD registries in children report appropriate shock rates ranging from 19 to 31% (depending on follow-up duration) and almost no deaths attributable to arrhythmia or device failure. 4 , 6 – 10 However, the complication rate is still significant: in appropriate ICD shocks, infections, lead related problems and ICD failure are most common. 11 – 15 The incidence of in appropriate shocks in children ranges from 20 to 27% during a mean follow up of 3.3 to 5.3 years, 4 , 6 , 9 , 10 and has negative effect on the psycho-social wellbeing of young patients and their parents. 16 , 17 More recent literature tends to report a decrease of in appropriate shock rate to around 10%. 11 , 13 , 14 , 18 , 19 The incidence of ICD shock therapy over time has not been investigated in children. It is of utmost importance to minimize unnecessary ICD shock therapy. Technical advances in ICD lead characteristics, advancements in programming strategies of the ICD 20 – 22 and the introduction of remote monitoring 23 – 25 have led to a reduction in the number of in appropriate shocks in adults. These principles are also introduced in the pediatric population. The advantage of early recognition of lead dysfunction and supraventricular or sinustachycardia by remote monitoring is expected to be beneficial particularly for the actively moving pediatric population for the prevention of inappropriate ICD shocks. However, only few studies have assessed the effect of ICD programming 11 , 13 and remote monitoring on the ICD shock rate in children. 26 – 30 As a consequence specific guidelines describing pediatric ICD programming are not available, but could be useful, as guideline concordant programming in adults has led to a further reduction of ICD shock therapy and mortality. 31 , 32 The current study investigates the incidence of ICD shock therapy in a Dutch cohort of pediatric ICD patients over time and the effect of ICD programming strategies and remote monitoring on the incidence of appropriate and in appropriate ICD shocks in children. Methods This retrospective multi-center study included all pediatric patients (0-17 years of age), who had a transvenous or epicardial ICD implantation between 1995 and 2021 in one of three University Medical Centers in the Netherlands, regardless of the underlying diagnosis. Patients with a subcutaneous ICD system were excluded. An official waiver of ethical approval was granted from the local ethical committees. Baseline characteristics Patient baseline characteristics at ICD implantation were obtained from digital patients files: year of implant, age, sex, weight, diagnosis, primary or secondary prevention and implantation route. ICD programming variables were collected directly after implantation, at one month follow up and at most recent follow up. VF/fast-VT/VT zones and detection time were categorized to be able to perform statistical analysis; VF zone categorized in < 210/min, 210-230/min, ≥ 230/min, (fast-)VT zone categorized in < 190/min, 190-210/min, ≥ 210/min, detection times categorized < 3 sec, 3-6 sec, ≥ 6 sec. For comparison purposes, the detection time recorded as number of beats were converted to time in seconds. Supraventricular tachycardia (SVT) discriminators, programmed anti-tachycardia pacing (ATP) therapy and starting date of remote monitoring (at the implantation or during follow-up) were registered. Follow-up During follow-up, all ICD shock episodes were collected, together with the ICD programming variables at the time of and after a shock episode. An ICD shock episode was defined as one or more ICD shocks until redetection of sinus rhythm by the device. To improve readability, one ICD therapy episode will be referred to as one ICD shock. In appropriate shocks were defined as ICD therapy for other tachycardia (SVT or sinus tachycardia) or for an abnormal sensing episode. Adverse events such as the need for device or lead revision and sudden cardiac arrest (SCA) requiring cardiopulmonary resuscitation (CPR), were registered separately. Follow-up continued until patients were 18 years of age or had at least a 5-year follow-up after they reached 18 years of age. Endpoints The primary end-points were differences in (F)VT/VF zones, detection time, SVT discriminator programming and the use of remote monitoring in 1) patients with an ICD implantation up to and including 2010 ( before 2010) versus after 2010; 2) patients with ICD shocks versus without ICD shocks; 3) in relation to the proportion of appropriate versus in appropriate shocks. The cut-off year 2010 was chosen to divide the patient cohort in two groups, as it marks the period around when data on the Dutch pediatric ICD patient cohort were published, 4 and when adult ICD studies concerning specific programming strategies led to changes in ICD management for children. 21 , 25 After 2010 remote monitoring got widely implemented. Statistical analysis Continuous data are presented as mean ± standard deviation or median (interquartile range), depending on the normality of the distribution, which was evaluated by the Shapiro-Wilk test. Differences between the before and after 2010 cohorts were analyzed using an independent t-test (for data with normal distribution) or Mann-Whitney U test. Categorical data are presented as an absolute count and percentage, and differences between the cohorts were evaluated by Chi-square tests or Fisher’s exact tests (in case of an expected value <5). The number of shocks, appropriate shocks and in appropriate shocks are presented as a (non-parametric) mean cumulative function (MCF) as this method facilitates multiple event (shocks) modelling. 33 Differences in MCF between the before and after 2010 cohorts, and between patients with versus without remote monitoring (at any time prior to the shock and the two cohorts taken together) were analyzed by a chi-square test according to the method of Nelson. 34 Differences between the cohorts in the time to the first occurrence of a shock (total, appropriate, in appropriate) were analyzed using the Cox proportional hazard regression model. Univariable models were ran, and models with multivariable adjustment for age and sex, and additionally for remote monitoring (as a time-dependent covariate), primary versus secondary prevention, and transvenous versus epicardial implantation. Results are presented as hazard ratios (HRs) with corresponding 95% confidence interval (CI). Statistical analysis was performed using SAS Analytics Software version 9.4. A p-value of < 0.05 was considered significant. Results A total of 163 children received a transvenous or epicardial ICD implantation, 88 patients before 2010 and 75 patients after 2010. Sufficient retrospective data could be retrieved of 116 patients to be able to include them in this study, 53 patients before 2010 and 63 patients after 2010. The baseline characteristics are presented in table 1 . The median age at implantation was 13.4 years of age, with no difference in age before and after 2010. The male to female ratio was 62 – 38 %. The ICD indications did not change over the years, with an almost 50-50 distribution of primary versus secondary prevention. View this table: View inline View popup Download powerpoint Table 1: Baseline characteristics Incidence of ICD shock therapy Fifty-five out of 116 patients (47%) received a total of 216 shocks, with a median of 2 shocks per patient (range 1–29 shocks) during a median follow-up of 5.2 years (IQR 3.7–6.6 years, Table 2 ). The number of patients with an ICD shock decreased from 30 (57%) before 2010 to 25 (40%) after 2010. The difference in MCF of ICD shocks per patient was significant (P = 0.008, Figure 1 ). With a mean follow-up of 5.4 years and 5.0 years respectively, the annual ICD shock rate decreased from 10.5% before to 8.0% after 2010. View this table: View inline View popup Download powerpoint Table 2: Number of patients with or without shocks and types of shock Download figure Open in new tab Figure 1 Mean cumulative number of shocks per patient with ICD implant up to and including 2010 (blue line) or after 2010 (red dotted line) A similar significant decrease was observed in the MCF of appropriate shocks per patient (P = 0.036, Figure 1 ). The number of patients with appropriate shocks decreased from 22 (42%) to 18 (29%), and the annual appropriate shock rate from 7.8% before 2010 to 5.8% after 2010. The proportion of patients that received an in appropriate shock decreased from 23% before to 13% after 2010, the annual in appropriate shock rate decreased from 4.3% to 2.6%. The difference in MCF of in appropriate shocks per patient was not significant (P = 0.28 Figure 1 ). ICD programming Comparing the VF detection zone programming at the time of shock between appropriate and in appropriate shocks showed significant higher VF zone programming (≥ 210/min) in appropriate shocks (86% vs 79%; P = 0.0016, Table 3 ). Comparing the programmed VF detection zones in patients implanted before and after 2010 did not show a significant difference, but a trend towards higher programmed VF zones (≥ 210 BPM) at end of study follow up in 90% of patients with ICD implantation after 2010 versus 76% of patients implanted before 2010 (P = 0.142 Table 1 Supplementary material). Comparing patients with and without shocks also showed a trend towards higher programmed VF zones (≥ 210 BPM) at end of study follow up in the patients without shocks (79% vs 89%; P=0.243, table 3 ). The detection time programming in the VF zone was not different in the group with versus without shocks and in appropriate versus in appropriate shocks ( Table 3 ). The (fast-)VT zone was used for ICD therapy in 34% (39/116) of patients, 38% before and 30% after 2010 ( Table 1 Supplementary material), with no change in detection rate before versus after 2010. View this table: View inline View popup Download powerpoint Table 3: ICD programming in patients with shocks versus without shocks at end of study follow-up; and at time of appropriate shocks versus in appropriate shocks Remote monitoring Remote monitoring was applied during follow-up in 18/53 patients (34%) before 2010 and increased to 52/63 patients (83%) after 2010 (P < 0.001). The MCF of total shocks in patients with and without remote monitoring showed a hazard ratio of 0.59 (95% CI 0.41 – 0.85, P = 0.013, Figure 2 ) in patients with remote monitoring applied. For the MCF of appropriate ICD shocks the hazard ratio was 0.61 (95% CI 0.40 – 0.94) in patients with remote monitoring applied (P = 0.052). For in appropriate shocks the MCF did not show a significant difference with and without remote monitoring. Download figure Open in new tab Download figure Open in new tab Figure 2 Mean cumulative number of shocks per patient with remote monitoring applied (red dotted line) or with remote monitoring not applied (blue line) In explorative multivariate analysis, ‘implantation after 2010’ and ‘older age’ appeared to be negatively correlated with total ( Table 4 ) and appropriate ICD shocks. Surprisingly, in multivariate analysis of in appropriate shocks secondary prevention was positively correlated with in appropriate ICD shocks, hazard ratio 2.96 (95% CI 1.39 – 6.32). View this table: View inline View popup Download powerpoint Table 4: Hazard ratio in multivariate analysis of total ICD shocks. Complications of ICD treatment During a mean follow-up of 5.2 years, 39 patients (34%) underwent an ICD or lead revision. Reasons for ICD revision were end-of-life of the battery in 19 patients, lead dysfunction in 17 patients, and infection and other indications in 8 patients. Some patients had a combination of reasons for revision. Three patients had an aborted sudden cardiac arrest requiring CPR despite ICD therapy, all with implantation after 2010. Two patients had a VT under the detection limit of 222/min (VF zone) and 231/min (fast-VT zone, VF-zone > 250/min) respectively, which was not hemodynamically tolerated; in a third patient with a VF zone > 240/min VF was detected, but only the fourth shock was effective. Twelve patients underwent a heart transplantation during follow-up, 5 patients before 2010 and 7 patients after 2010. In the group of patients before 2010, three patients died during follow-up of the study, compared to one patient in the group after 2010. The cause of death was cardiac, but unrelated to ICD therapy in 3 patients and unknown in another patient. Discussion Main findings This study demonstrates a significant decrease in total amount of ICD shocks over the last decade and a protective correlation of remote monitoring on the incidence of ICD shocks in one of the largest cohorts of pediatric patients. Contrary to expectations, this decrease and protective effect was clearly present for appropriate shocks, but could not be demonstrated for in appropriate shocks. In addition, the VF detection rate was significantly more often programmed in higher ranges (≥210/min) at time of appropriate shocks when compared to in appropriate shocks. Also a trend towards higher VF detection zone (≥210/min) after 2010 and in patients without shocks was demonstrated. Annual shock rate The annual appropriate shock rate in this study was 7.8% and 5.8%, before 2010 and after 2010 respectively. The annual appropriate shock rate before 2010, matches the previously reported annual appropriate shock rate of 8.4% in the Netherlands, which included partly the same patients. 4 The annual appropriate shock rate after 2010 is in range with other pediatric ICD series published over the last 10 years ( Table 2 Supplementary Material), describing annual appropriate shock rates ranging between 2.9 and 13.9%. Also the annual in appropriate shock rate of 2.6% after 2010 is in line with the annual in appropriate shock rate in the review varying between 1.1 to 6.8% in recent studies. These recently described appropriate and in appropriate shock rates in children approximate the numbers described for adults with inherited arrhythmia syndromes. In a meta-analysis of patients with a mean age of 39 years and inherited arrhythmia syndromes, the annual rate of in appropriate shocks was 4.7%. 35 Influence of ICD programming This study describes a decrease of ICD shocks over the last decade, with a trend towards higher detection rates (≥210 bpm) in VF zones after 2010. In addition, this study shows a significant higher programmed VF zone in appropriate shocks in comparison to in appropriate shocks. Both findings confirm the advantage of high VF zone programming. In a study from 2015 including pediatric and adult congenital heart disease patients with an ICD, with a comparable annual appropriate ICD shock rate of 5.7%, had a mean ventricular fibrillation detection rate of 222 +/− 15 beats/min. 8 It showed no difference in patients with or without in appropriate shocks, by comparing ICD programming data at the time of in appropriate shock to programming data of the patients without in appropriate shock at most recent follow up. A more recent study in 51 pediatric patients from 2023 did show a difference in programmed VF zone in patients with in appropriate shocks (VF zone 188/min) vs patients without in appropriate shocks (VF zone 222/min). 13 The inconsistency of these findings might partly be attributed to the potential incomparability of data measured at the time of the in appropriate shock and at random follow up time in patients without in appropriate shocks. This was the reason not to perform these analyses in the current study. However, the results of previous studies, as well as the results described in this study, justify the current practice to program VF detection rates up to 230-250/min in children, in accordance to adult guideline on ICD programming. 32 These recommendations in adults are, amongst other studies, based on large trials like the MADIT-RIT and RISSY-ICD trials, which have clearly shown the beneficial effects of high-rate programming with VF-zones higher than 200-230/min on in appropriate shocks and mortality. 20 , 21 A longer detection time is also known to decrease the shock rate in adult patients. 22 In the ADVANCE III trial in adult patients the appropriate and in appropriate shock rate decreased 48% by increasing the detection time from 18 out of 24 intervals, to 30 out of 40 intervals. 36 Though this effect has not been confirmed in pediatric series, some studies do report the use of longer detection times, like a median detection time of 9.6 seconds in the UK National hypertrophic cardiomyopathy (HCM) cohort study. 11 The current study was unable to demonstrate an effect of the detection time on the total, as well as the appropriate or in appropriate shock rate. Monomorphic ventricular tachycardia has been found to be a risk factor for appropriate shocks in pediatric patients. 13 Therefore, therapy in VT-zone (ATP and shock) is often programmed in pediatric patients with prior documented VT episodes. 11 On the other hand, studies in adults and children have shown that the use of shock therapy in the VT-zone increases appropriate and in appropriate shocks, and even increases all-cause mortality. 8 , 21 In our present series we found that therapy in the (fast-)VT-zone was programmed in 34% of the patients, without a difference in detection rate between the patients with implantation before or after 2010. As the current study did not collect data on whether shock therapy was applied in the (fast-)VT or VF zone, the relation between (fast-)VT zone programming and ICD shock rate could not examined. Influence of remote monitoring Remote monitoring was significantly associated with a reduction in total amount of ICD shocks. Although remote monitoring was expected to be particularly advantageous for the prevention of in appropriate ICD shocks, its efficacy was only confirmed for a decrease in any shock and appropriate shocks. In the adult literature, there is clear evidence that both any ICD shock and in appropriate shocks can be reduced by 72% and 52% respectively with remote monitoring. 23 This finding was confirmed by a meta-analysis in 2015. 37 The evidence in literature for the additional value of remote monitoring in the prevention of ICD shocks in pediatric patients with an ICD is scarce. 38 A study from 2014 with 198 patients with a cardiac implantable electronic device (CIED) of which 61 ICD, showed that remote monitoring allows for early identification of arrhythmias (like SVT, atrial fibrillation/flutter or VT) and device malfunction. 28 But there is no previously published evidence that remote monitoring correlates with a reduction of the ICD shock rate in a pediatric population ( Table 2 Supplementary Material). In univariate analysis remote monitoring was associated with ICD shock reduction over the last decade. Multivariate analysis showed that only ‘implantation after 2010’ and ‘older age’ were negatively correlated with the amount of total and appropriate ICD shocks. This suggests that other factors also must have contributed to ICD shock reduction. A combination of technical advances in ICD lead preservation and ICD programming management probably played an important role, as for example the trend to higher detection rate programming described in this study. In addition, advances and new insights to disease treatment will have contributed to a decrease of the incidence of arrhythmia or ICD implantation in general. 39 , 40 These factors were not investigated in this study. Other risk factors for shocks In the current study a younger age was correlated with a higher proportion of total shocks and appropriate shocks in multivariate analysis. We did not find a correlation between age and in appropriate shocks. Evidence on the effect of age on the number of shocks in the literature is conflicting ( Table 2 Supplementary Material), some do report a correlation with appropriate shocks and younger age, 4 while others do not find any association. 10 The same accounts for the correlation between in appropriate shocks and age. 7 , 8 In general, an ICD indication at younger age is reserved for the most serious disease phenotypes, with consequently the highest arrhythmia burden and higher shock rates. Secondary prevention has been identified as a predictor of appropriate shocks in adult patients, 41 but was described with inconsistent results in pediatric series ( Table 2 Supplementary Material). Unexpectedly, multifactorial analysis in the current series, showed a correlation between secondary prevention and increased risk for in appropriate shocks. Since this has not been reported in other series, this result should be interpreted with caution. Implications for clinical practice Based on the results of the present study and the review of the literature, we conclude that higher VF detection zone programming and the application of remote monitoring are beneficial for children, and can reduce the number of appropriate and in appropriate shocks. The current study supports the concept to program pediatric ICDs with high VF zones above 210-230/min, with the application of remote monitoring. Exceptions to standard high rate VF/(fast-)VT zone programming are patients with ventricular dysfunction and/or patients who do not tolerate slow VT hemodynamically. In the current study ICD programming data were not related to ventricular ejection fraction or diastolic dysfunction. However, two patients required CPR despite ICD implantation because of hemodynamically non-tolerated slow VT under the programmed VF/(fast-)VT detection rates. Additional studies are necessary to investigate specific risk factors and ICD programming strategies for pediatric patients with ventricular dysfunction. Limitations This study describes a relatively large cohort of pediatric patients over a long follow-up period. The statistically significant but clinically small difference in follow-up duration before and after 2010, is not thought to explain the difference in number of shocks before and after 2010. Due to its retrospective design, available data were often insufficient for reliable data acquisition, particularly in the era < 2010, resulting in inability to investigate all the predetermined end-points (detection time and SVT discriminators). Data collection was focused on the (in) appropriateness of ICD shocks, but the exact tachycardia rate and zone for which the specific ICD shock therapy was applied, was not registered. Therefore the influence of (fast-)VT zone programming could not be analyzed, although it might have influenced the shock rates in the study population described. We were also unable to obtain reliable data on the incidence of ATP therapy, anti-arrhythmic drug treatment and left sympathetic cardiac denervation, which could also have affected the shock rate. Finally general conclusions are drawn for a heterogenous group of underlying cardiac diagnosis, but numbers are too low for subgroup analyses. Future large, international (multicenter) prospective studies are needed to overcome the above described limitations. Conclusion The incidence of total amount of ICD shocks and appropriate shocks in children with a transvenous or epicardial ICD, has significantly decreased in patients with implantation after 2010 compared to patients with implantation before 2010, without an increase of cardiac related death. Factors that are correlated with this decrease in number of shocks are a higher programmed VF-zone and applied remote monitoring. These findings encourage ICD programming of high VF zone detection rates (in children without severe ventricular dysfunction) and the application of remote monitoring in all children requiring ICD therapy. Data Availability The authors confirm that the data supporting the findings of this study are available upon request. Disclosures Authors have no conflicts to disclose. This investigator initiated study was supported by a research grant from the Dutch Hartekind Foundation and from Medtronic. Acknowledgements Marco N. Kruit, Arthur E. van der Mark and Michiel Zumbrink for their enormous efforts to provide the availability of data with respect to ICD programming. Mattie J. Lenzen and Danny Ng for technical support with the database. Arend W. van Deutekom for help with preliminary statistical analyses. 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