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      Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits

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          Abstract

          Inherited ion channelopathies have come to the forefront as a significant cause of sudden cardiac death (SCD) in pediatric patients with structurally normal hearts. Implantable cardioverter-defibrillator (ICD) placement can be a life-saving primary preventative therapy, but because of actors inherent in the pediatric population, careful thought must be given to the specific indications for placement in each patient. The most common inherited ion channelopathies are long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. All have the potential to cause SCD. However, thanks to current research, more is now known about the range of phenotypes present within each disorder and also the benefits that medical therapy can provide. Risk stratification can allow clinicians to best predict which patients may most benefit from a primary preventative ICD while at the same time avoid placement in the larger group who may remain asymptomatic with the aid of medical therapy or even simply observation.

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          Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium.

          Kriengkrai Jirasirirojanakorn, K Bhuripanyo, Koonlawee Nademanee … (2011)
          The underlying electrophysiological mechanism that causes an abnormal ECG pattern and ventricular tachycardia/ventricular fibrillation (Vt/VF) in patients with the Brugada syndrome (BrS) remains unelucidated. However, several studies have indicated that the right ventricular outflow tract (RVOT) is likely to be the site of electrophysiological substrate. We hypothesized that in patients with BrS who have frequent recurrent VF episodes, the substrate site is the RVOT, either over the epicardium or endocardium; abnormal electrograms would be identified at this location, which would serve as the target site for catheter ablation. We studied 9 symptomatic patients with the BrS (all men; median age 38 years) who had recurrent VF episodes (median 4 episodes) per month, necessitating implantable cardioverter defibrillator discharge. Electroanatomic mapping of the right ventricle, both endocardially and epicardially, and epicardial mapping of the left ventricle were performed in all patients during sinus rhythm. All patients had typical type 1 Brugada ECG pattern and inducible Vt/VF; they were found to have unique abnormal low voltage (0.94±0.79 mV), prolonged duration (132±48 ms), and fractionated late potentials (96±47 ms beyond QRS complex) clustering exclusively in the anterior aspect of the RVOT epicardium. Ablation at these sites rendered Vt/VF noninducible (7 of 9 patients [78%]; 95% confidence interval, 0.40 to 0.97, P=0.015) and normalization of the Brugada ECG pattern in 89% (95% confidence interval, 0.52 to 0.99; P=0.008). Long-term outcomes (20±6 months) were excellent, with no recurrent Vt/VF in all patients off medication (except 1 patient on amiodarone). The underlying electrophysiological mechanism in patients with BrS is delayed depolarization over the anterior aspect of the RVOT epicardium. Catheter ablation over this abnormal area results in normalization of the Brugada ECG pattern and prevents Vt/VF, both during electrophysiological studies as well as spontaneous recurrent Vt/VF episodes in patients with BrS.
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            Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry.

            Silvia G Priori, Maurizio Gasparini, Carlo Napolitano … (2012)
            The PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) prospective registry was designed to assess the predictive accuracy of sustained ventricular tachycardia/ventricular fibrillation (VTs/VF) inducibility and to identify additional predictors of arrhythmic events in Brugada syndrome patients without history of VT/VF. Brugada syndrome is a genetic disease associated with increased risk of sudden cardiac death. Even though its value has been questioned, inducibility of VTs/VF is widely used to select candidates to receive a prophylactic implantable defibrillator, and its accuracy has never been addressed in prospective studies with homogeneous enrolling criteria. Patients with a spontaneous or drug-induced type I electrocardiogram (ECG) and without history of cardiac arrest were enrolled. The registry included 308 consecutive individuals (247 men, 80%; median age 44 years, range 18 to 72 years). Programmed electrical stimulation was performed at enrollment, and patients were followed-up every 6 months. During a median follow-up of 34 months, 14 arrhythmic events (4.5%) occurred (13 appropriate shocks of the implantable defibrillator, and 1 cardiac arrest). Programmed electrical stimulation performed with a uniform and pre-specified protocol induced ventricular tachyarrhythmias in 40% of patients: arrhythmia inducibility was not a predictor of events at follow-up (9 of 14 events occurred in noninducible patients). History of syncope and spontaneous type I ECG (hazard ratio [HR]: 4.20), ventricular refractory period <200 ms (HR: 3.91), and QRS fragmentation (HR: 4.94) were significant predictors of arrhythmias. Our data show that VT/VF inducibility is unable to identify high-risk patients, whereas the presence of a spontaneous type I ECG, history of syncope, ventricular effective refractory period <200 ms, and QRS fragmentation seem useful to identify candidates for prophylactic implantable cardioverter defibrillator. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
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              Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia.

              Nathalie Roux-Buisson, Seiji Takatsuki, Fabrice Extramiana … (2009)
              The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events, have not been fully ascertained. The outcome in 101 catecholaminergic polymorphic ventricular tachycardia patients, including 50 probands, was analyzed. During a mean follow-up of 7.9 years, cardiac events defined as syncope, aborted cardiac arrest, including appropriate discharges from implantable defibrillators, or sudden cardiac death occurred in 27 patients, including 2 mutation carriers with normal exercise tests. The estimated 8-year event rate was 32% in the total population and 27% and 58% in the patients with and without beta-blockers, respectively. Absence of beta-blockers (hazard ratio [HR], 5.48; 95% CI, 1.80 to 16.68) and younger age at diagnosis (HR, 0.54 per decade; 95% CI, 0.33 to 0.89) were independent predictors. Fatal or near-fatal events defined as aborted cardiac arrest or sudden cardiac death occurred in 13 patients, resulting in an estimated 8-year event rate of 13%. Absence of beta-blockers (HR, 5.54; 95% CI, 1.17 to 26.15) and history of aborted cardiac arrest (HR, 13.01; 95% CI, 2.48 to 68.21) were independent predictors. No difference was observed in cardiac and fatal or near-fatal event rates between probands and family members. Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia probands and affected family members during the long-term follow-up, even while taking beta-blockers, which was associated with a lower event rate. Further studies evaluating concomitant therapies are necessary to improve outcome in these patients.
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Innov Card Rhythm Manag
                Journal ID (iso-abbrev): J Innov Card Rhythm Manag
                Journal ID (publisher-id): JICRM
                Title: The Journal of Innovations in Cardiac Rhythm Management
                Publisher: MediaSphere Medical (United States )
                ISSN (Print): 2156-3977
                ISSN (Electronic): 2156-3993
                Publication date (Electronic): 15 September 2018
                Publication date Collection: September 2018
                Volume: 9
                Issue: 9
                Pages: 3297-3302
                Affiliations
                [1] 1Department of Pediatric Cardiology, Cleveland Clinic Children’s Hospital, Cleveland, OH, USA
                Author notes
                Address correspondence to: Peter F. Aziz, MD, FHRS, Department of Pediatric Cardiology, Cleveland Clinic Children’s Hospital, 9500 Euclid Avenue, M4-1, Cleveland, OH 44195, USA. Email: azizp@ 123456ccf.org .

                The authors report no conflicts of interest for the published content.

                Article
                Publisher ID: icrm.2018.090901
                DOI: 10.19102/icrm.2018.090901
                PMC ID: 7252732
                SO-VID: 5077e2c4-751c-4f4e-854f-ad06b43b2b3f
                Copyright © Copyright: © 2018 Innovations in Cardiac Rhythm Management
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 30 October 2017
                Date accepted : 4 December 2017
                Categories
                Subject: Research Review

                Keywords: icd,implantable cardioverter-defibrillator,ion channelopathy,pediatric
                Data availability:
                Keywords: icd, implantable cardioverter-defibrillator, ion channelopathy, pediatric

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