Daily Remote Monitoring of Pediatric Patients with Cardiac Implantable Electronic Devices: Examples of Effective Applications for Children.

Introduction In recent years, significant progress has been made in the treatment of arrhythmias in children with the use of implantable pacemakers (PMs) and implantable cardioverter-defibrillators (ICDs). The remote monitoring (RM) system was introduced to enable faster arrhythmia assessment and to shorten the time to clinical intervention. Case Reports A 10-year-old Title: Daily Remote Monitoring of Pediatric Patients with Cardiac Implantable Electronic Devices: Examples of Effective Applications for Children. Short Title: Pediatric Remote Monitoring of Cardiac Implantable Electronic Devices Authors : Piotr Wieniawski 1*, Jakub Zabłocki 2*, Bożena Werner 1 1 Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw. Warsaw, Poland. 2 Student Scientific Association at the Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw. Warsaw Poland. *Equal Contribution Corresponding Author : Prof. Bożena Werner, MD, PhD, Department of Pediatric Cardiology and General Pediatrics, Medical University of Warsaw, Żwirki i Wigury 61, 02–091 Warszawa, Poland, phone: +48 22 317 95 88, e-mail: [email protected]

remote monitoring, pacemaker, implantable cardioverter-defibrillator, children Key Clinical Message Remote monitoring of implantable devices is crucial for managing pediatric arrhythmia patients. Symptoms can vary significantly, and children, particularly those with intellectual disabilities, may struggle to communicate their symptoms, complicating detection by caregivers. RM enables clinicians to rapidly assess arrhythmias without hospitalization, allowing for quicker, more effective interventions.

In recent years, significant progress has been made in the treatment of arrhythmias in children with the use of implantable pacemakers (PMs) and implantable cardioverter-defibrillators (ICDs). The remote monitoring (RM) system was introduced to enable faster arrhythmia assessment and to shorten the time to clinical intervention. A 10-year-old with long QT syndrome, atrial fibrillation (AF), left ventricular hypertrophy, and intellectual disability was admitted for ICD with RM implantation. The device detected 52 AF episodes, one of which progressed to ventricular fibrillation and was successfully terminated by defibrillation. Notably, the parents were unaware of the arrhythmia as the child showed no abnormal behavior, likely due to intellectual disability. A 6-year-old patient with tachy-brady syndrome (TBS), tetralogy of Fallot, and pulmonary valve atresia underwent PM implantation with RM. Since then, the device has recorded 25 atrial flutter (AFL) episodes. The patient was hospitalized five times after device-triggered alerts and rhythm evaluations. Each time, conversion to sinus rhythm was achieved through pharmacological and electrical cardioversion. The patient then had ablation of the AFL focus, with no further arrhythmia-related hospitalizations observed. In a 2-year-old with AFL and second-degree AV block, TBS was identified during hospitalization in Pediatric Cardiology, based on a 24-hour Holter ECG. Sinus pauses of up to 5 seconds were noted. A PM with RM was implanted. Since then, the device recorded 14 AFL episodes, three requiring hospitalization after physician evaluation. All ended with successful electrical cardioversion.

Daily remote monitoring (RM) of cardiac implantable electronic devices (CIEDs) has been a breakthrough in pediatric electrocardiology. This case series represents the single-center experience of the Department of Pediatric Cardiology with RM of CIEDs, including implantable cardioverter-defibrillators (ICDs) and pacemakers (PMs).

Data from 48 patients with PM or ICD and RM were evaluated. Three representative cases were selected. Clinical histories and RM data were obtained and analyzed through electronic medical records and the Home Monitoring™ Service Center, respectively. In each patient, in-person evaluations (IPE) were conducted quarterly, regardless of emergency hospitalizations due to RM alerts. An arrhythmic episode would be recognized by the device, and a RM alert (either “red” or “yellow” – programmed beforehand) would be sent to the clinician’s mobile phone, prompting contact with the parents. Patient No. 1 A 16-year-old male with an intellectual disability was referred to the Cardiology Department due to a prolonged QTc interval on the electrocardiogram (ECG). The boy had a history of two spontaneous syncopal episodes. ECGs revealed long-QT syndrome (LQTS) and atrial fibrillation (AF). An echocardiogram indicated left ventricular hypertrophy (LVH). The patient’s family history included an implantable cardioverter-defibrillator (ICD) in the mother (prolonged QT, hypertrophic cardiomyopathy, AF) and a brother with an intellectual disability who died at the age of 10 without a known cause (no autopsy performed). The patient and his mother underwent genetic testing using the TruSight Cardio Sequencing Panel, which revealed no abnormalities. AF was managed effectively with both pharmacological and electrical cardioversion. However, LQTS and LVH were clear indications for ICD implantation. The chosen device was the Rivacor 5 DR-T. Of a total of under two thousand recordings, 52 episodes were of atrial fibrillation (AF) and two episodes were of ventricular fibrillation (VF). Most AF episodes were self-limiting. Two shocks were delivered for each VF event, successfully converting it to sinus rhythm. An event worth noting was AF degenerating into VF, correctly recognized by the cardiac implantable electronic device (CIED). The device delivered two shocks of 40J, the second of which was effective. [Fig. 1A] The patient did not report either the presence of the arrhythmia or the shock delivery. The parents remained unaware of the event until the clinician informed them. Without remote monitoring (RM), this incident likely would not have been discovered until the in-person evaluation (IPE). Patient No. 2 A 6-year-old male, following corrective surgery for tetralogy of Fallot (ToF) with pulmonary valve atresia, was hospitalized after bradycardic tendencies were observed at night. Sick sinus syndrome (SSS) was diagnosed after 24-hour Holter monitoring, along with persistent atrial flutter (AFL). Notably, the patient remained asymptomatic. Antiarrhythmic treatment began with amiodarone, which was later changed to propranolol, while multiple sessions of electrocardioversion were performed, restoring sinus rhythm. However, the oral antiarrhythmic treatment further exacerbated the bradycardia. The only viable solution was the implantation of a pacemaker (PM). The preferred device was the Enitra 8 DR-T with epicardial electrodes. There were a total of 55 rhythm monitoring (RM) recordings, 25 of which indicated atrial tachycardia (AT), evaluated by the clinician for AFL. The events were primarily self-terminating. One of the recordings showed an atrial rate of 250 beats per minute (bpm), suggesting early AFL. No symptoms were reported. The arrhythmia was not self-limiting, prompting the device to switch to permanent ventricular stimulation mode to maintain effective ventricular contraction. [Fig. 1B] The clinician contacted the boy’s parents to enable final confirmation of AFL and rapid electrocardioversion. Patient No. 3 A 2-year-old male patient was diagnosed with episodes of AFL, primarily presenting as a 2:1 block with an atrial rhythm of 280 bpm. Occasionally, 4:1 or 5:1 block was observed. The patient was asymptomatic. Combined antiarrhythmic therapy (electrocardioversion and amiodarone) failed to restore sinus rhythm. During the diagnostic procedure, SSS was confirmed. A 24-hour Holter monitor revealed conduction pauses lasting up to 5 seconds. An Entira 8 DR-T PM with epicardial leads was implanted. RM presented 24 recordings, which included 14 episodes of AT, mostly self-limiting. However, three events necessitated hospitalization as the clinician suspected AFL; each of these emergency hospitalizations involved electrocardioversion followed by metoprolol therapy. A representative AFL recording was shown in Fig 1C. Figure 1. Recordings from Home Monitoring System. Patient no.1 experienced a VF episode, which was correctly recognized by the ICD and managed with successful shocks (Fig. 1A). Patient no.2 underwent an AFL episode with PM mode-switch to permanent ventricular stimulation (Fig. 1B). Similarly, Patient no.3 suffered from asymptomatic AFL (Fig. 1C). The cases presented demonstrate the effectiveness of remote monitoring (RM) in the early diagnosis and rapid intervention of life-threatening arrhythmias in pediatric patients with cardiac implantable electronic devices (CIEDs). The current HRS/EHRA/APHRS/LAHRS consensus briefly discusses RM in pediatrics, noting faster detection of arrhythmias and device malfunctions, even in asymptomatic patients [1]. As proven by the cases, children with arrhythmias, along with young age or intellectual disabilities, are prone to atypical clinical presentations. RM facilitates an early overview when an event occurs, prompting quicker and more successful intervention. Although research on RM in children is limited, the studies published to date support the significant role of RM for CIEDs in the pediatric population. A study by Malloy, L.E. et al. included 198 remotely monitored pediatric patients with CIEDs. The devices comprised 105 pacemakers (PMs) and 61 implantable cardioverter-defibrillators (ICDs). The indications for CIED implantation in that population varied greatly, with complete heart block accounting for most cases (n = 76). In that study, 39% of adverse events were asymptomatic, highlighting the silent nature of arrhythmias in children. Similarly, all three of our patients were either asymptomatic or unable to recognize symptoms. While we advise parents to monitor heart rates daily (using a pulse oximeter or – if possible – a stethoscope), in Patients 2 and 3, RM enabled a faster clinical response. Maldonado et al. evaluated the usefulness of RM in children with CIEDs. The authors conducted a long-term follow-up of 27 patients with a median age of 12.2 years. Twenty patients received PMs, while seven underwent ICD implantation. In the study, five patients presented “red” alerts, with three of those corresponding to ventricular fibrillation (VF). In two cases, appropriate shocks were delivered by the CIEDs, similar to Patient 1. In a study by Jouen R. et al., 13 of 34 patients had device-triggered alerts, including eight cases of ventricular tachycardia (VT). The authors emphasize that all hospitalization-requiring cases were treated within 24 hours thanks to RM’s early detection. In Patients 2 and 3, RM enabled avoiding anticoagulants and transesophageal echocardiography before electrical cardioversion, significantly reducing the length of hospitalization.

RM represents a significant advancement in pediatric cardiology for the direct monitoring of patients with CIEDs, along with potential interventions and treatments. It offers numerous benefits for physicians, patients, and parents alike. Further research is necessary on the topic of RM for CIEDs in the pediatric population, particularly among Polish children with arrhythmias. Author Contributions Piotr Wieniawski: Conceptualization, Formal analysis, Investigation, Methodology, Project administration, Resources, Software, Supervision, Validation, Visualization, Writing - review & editingAcknowledgments. Jakub Zabłocki: Data curation, Formal analysis, Investigation, Methodology, Project administration, Resources, Software, Validation, Visualization, Writing - original draft Bozena Werner (Corresponding Author) Methodology, Project administration, Supervision Writing - review & editing, The authors have nothing to report. Consent The patients’ parents signed consent to participate in the study. Parents of the patient provided written consent for the publication. During the study and the writing of the manuscript, the principles of the Declaration of Helsinki were adhered to. Acknowledgments The authors have nothing to report. Conflicts of Interest The authors declare no conflicts of interest.

1. Ferrick AM, Raj SR, Deneke T, et al. 2023 HRS/EHRA/APHRS/LAHRS expert consensus statement on practical management of the remote device clinic. Heart Rhythm. 2023; 20(9): 92-144. 2. Malloy LE, Gingerich J, Olson MD, et al. Remote monitoring of cardiovascular implantable devices in the pediatric population improves detection of adverse events. Pediatr Cardiol. 2014; 35(2): 301-6. 3. Maldonado S, Lafuente MV, Benjamin M. Usefulness of remote monitoring of pediatric patients with cardiac implantable electronic devices. Argentine Journal of Cardiology. 2018; 86: 28-32. 4. Jouen R, Delsarte L, Placide, L, et al. Remote monitoring of cardiac implantable devices in children: characteristics and outcome in a retrospective single centre contemporary study. TelePaedia study. European Heart Journal. 2023;44. Information & Authors Information Version history Copyright This work is licensed under a Non Exclusive No Reuse License. Collection

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