Advancing Wearable Biosensors for Congenital Heart Disease: Patient and Clinician Perspectives: A Science Advisory From the American Heart Association.

Wearable biosensors (wearables) enable continual, noninvasive physiologic and behavioral monitoring at home for those with pediatric or congenital heart disease. Wearables allow patients to access their personal data and monitor their health. Despite substantial technologic advances in recent years, issues with hardware design, data analysis, and integration into the clinical workflow prevent wearables from reaching their potential in high-risk congenital heart disease populations. This science advisory reviews the use of wearables in patients with congenital heart disease, how to improve these technologies for clinicians and patients, and ethical and regulatory considerations. Challenges related to the use of wearables are common to every clinical setting, but specific topics for consideration in congenital heart disease are highlighted.

Incorporation of the CardioMEMS™ System During an Exercise Physiology Test in a Pediatric Congenital Heart Disease Patient Contributing to Medical Decision-Making.

Exercise testing among the pediatric congenital heart disease population continues to transform and expand the way patients are evaluated and managed. We describe a case where a stress echocardiogram was performed while successfully collecting data from a previously implanted CardioMEMS™ HF system which helped guide decision-making.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

Institutional experience of healthy pediatric patients presenting with atrial fibrillation who had an electrophysiology study.

INTRODUCTION: Atrial fibrillation (AF) is a very common tachyarrhythmia with increasing prevalence with age, but uncommon in the pediatric population. Understanding that AF increases comorbidities make the need for investigation and potential elimination of alternate etiologies in pediatric AF patients critical. The objective of this study was to review our institutional data and compare our findings with previously documented adult AF risk factors to pediatric patients while also identifying which patients had alternate electrophysiology diagnoses amenable to transcatheter ablation. METHODS: A retrospective chart review was performed identifying AF patients who were less than 21 years old, had no significant congenital cardiovascular anomalies, a documented episode of AF on electrocardiogram and underwent invasive electrophysiology study (EPS). RESULTS: Nineteen patients were identified over a 9-year period of time finding a male predominance (74%), the average age of 14.95 ± 4.17 years, the average weight of 78.5 ± 31.4 kg, and average body mass index of 26.8 ± 6.87 kg/m2 . Preprocedural left atrial volumes made on echocardiograms demonstrated a mean of 33.96 ± 16.35 mL/m2 (Z-scores -0.81 ± 1.50), indicating no dilation. Five of nineteen patients (26%) had additional electrophysiologic diagnoses during EPS, including atrioventricular reentrant tachycardia (n = 2, 10%) and atrioventricular nodal reentrant tachycardia (n = 3, 16%). Four patients underwent successful ablation with no documented or clinical AF recurrence. CONCLUSIONS: Adult risk factors of male predominance and obesity were seen in pediatric AF patients, while left atrial enlargement was not. Twenty-one percent of the pediatric AF patients who had additional electrophysiologic substrates and successful ablations resulted in no further clinical episodes of AF. This suggests that pediatric patients presenting with AF might benefit from an EPS as part of a complete evaluation.

Reveal LINQ Versus Reveal XT Implantable Loop Recorders: Intra- and Post-Procedural Comparison.

OBJECTIVES: To compare the procedure, recovery, hospitalization times, and costs along with patient/parent satisfaction after newer-generation cardiac implantable loop recorder (Reveal LINQ; Medtronic Inc, Minneapolis, Minnesota) and previous-generation implantable loop recorder (Reveal XT; Medtronic Inc). STUDY DESIGN: A prospective study of patients undergoing LINQ implantations between April 2014 and October 2015 was performed. Retrospective chart review of patients undergoing XT implantations was performed for comparison. RESULTS: Thirty-one patients received LINQ and 15 patients received XT. Indications included syncope/palpitations (28/46, 61%), history of arrhythmias (9/46, 20%), arrhythmia burden in congenital heart disease (5/46, 10%), and monitoring in channelopathies (4/46, 9%). The LINQ group underwent more conscious sedation procedures than the XT group (8/31 vs 0/15, P = .04) with shorter procedural time (9 vs 34 minutes, P <.001), room occupation time (38 vs 81 minutes, P <.001), recovery time (21 vs 67 minutes, P <.001), and total hospital time (214 vs 264 minutes, P = .046). The LINQ group also had shorter return to activity time (2 vs 5 days, P = 1). Three device erosions in the LINQ group required reintervention. The LINQ group had fewer body image issues than the XT group (1/26 vs 5/14, P = .01) with both groups scoring 5/5 overall patient/parent satisfaction score at follow-up. Both groups had comparable total direct hospital costs (US $5905 vs $5438, P = .8). CONCLUSIONS: LINQ offers better procedural and recovery time compared with XT. LINQ implantations under conscious sedation reduce total hospitalization time.

Electrophysiologic substrate in congenital Long QT syndrome: noninvasive mapping with electrocardiographic imaging (ECGI).

BACKGROUND: Congenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope and sudden death. Although its genetic basis has become well-understood, the mechanisms whereby mutations translate to arrhythmia susceptibility in the in situ human heart have not been fully defined. We used noninvasive ECG imaging to map the cardiac electrophysiological substrate and examine whether LQTS patients display regional heterogeneities in repolarization, a substrate that promotes arrhythmogenesis. METHODS AND RESULTS: Twenty-five subjects (9 LQT1, 9 LQT2, 5 LQT3, and 2 LQT5) with genotype and phenotype positive LQTS underwent ECG imaging. Seven normal subjects provided control. Epicardial maps of activation, recovery times, activation-recovery intervals, and repolarization dispersion were constructed. Activation was normal in all patients. However, recovery times and activation-recovery intervals were prolonged relative to control, indicating delayed repolarization and abnormally long action potential duration (312±30 ms versus 235±21 ms in control). Activation-recovery interval prolongation was spatially heterogeneous, with repolarization gradients much steeper than control (119±19 ms/cm versus 2.0±2.0 ms/cm). There was variability in steepness and distribution of repolarization gradients between and within LQTS types. Repolarization gradients were steeper in symptomatic patients (130±27 ms/cm in 12 symptomatic patients versus 98±19 ms/cm in 13 asymptomatic patients; P<0.05). CONCLUSIONS: LQTS patients display regions with steep repolarization dispersion caused by localized action potential duration prolongation. This defines a substrate for reentrant arrhythmias, not detectable by surface ECG. Steeper dispersion in symptomatic patients suggests a possible role for ECG imaging in risk stratification.

Assessment of intrathoracic impedance algorithm in the pediatric and adult congenital population.

BACKGROUND: Decreased intrathoracic impedance has been used in adults to predict heart failure (HF) exacerbations. A commercial algorithm, OptiVol® (Medtronic Inc., Minneapolis, MN, USA), identifies patients with decreased impedance. We sought to determine the specificity, sensitivity, and positive predictive value (PPV) of OptiVol for predicting HF exacerbation or increased arrhythmia burden in pediatric and adult congenital heart disease (CHD) patients. METHODS: A multicenter retrospective chart review was undertaken. Inclusion criteria were: (1) <19 years or CHD adults, (2) an implanted device with OptiVol capability, (3) implanted between April 9 and September 6, and (4) follow-up of >30 days postimplant. Clinical events were defined as clinical HF exacerbation/hospital admission, initiation/uptitration of medication, or increased arrhythmia burden. RESULTS: Seventy-two patients (19 ± 9 years) were identified with the following indications: 20% dilated cardiomyopathy (DCM), 11% hypertrophic cardiomyopathy (HCM), 43% CHD, 15% channelopathy, and 11% other. Thirty-nine had 122 OptiVol crossings (median 2, range 1-11); 30% were linked to a cause. The remaining 33 had no crossing, though 17 had 89 clinical events. The clinical event rate was 19% greater in patients with crossings, though not statistically significant (P = 0.4). The algorithm had a 59% sensitivity, 52% specificity, and 62% PPV. Clinical HF exacerbation and arrhythmia burden did not significantly correlate with decreased impedance though uptitration or initiation of HF medication did correlate significantly (P = 0.03). CONCLUSION: The algorithm sensitivity for pediatric DCM, HCM, CHD, and adult CHD was equivalent to the general adult population. Further studies are warranted to assess whether inaccuracy in prediction is secondary to the algorithm or to differences in the clinical response of pediatric/CHD patients.

The potential of artificial intelligence to revolutionize health care delivery, research, and education in cardiac electrophysiology.

The field of electrophysiology (EP) has benefited from numerous seminal innovations and discoveries that have enabled clinicians to deliver therapies and interventions that save lives and promote quality of life. The rapid pace of innovation in EP may be hindered by several challenges including the aging population with increasing morbidity, the availability of multiple costly therapies that, in many instances, confer minor incremental benefit, the limitations of healthcare reimbursement, the lack of response to therapies by some patients, and the complications of the invasive procedures performed. To overcome these challenges and continue on a steadfast path of transformative innovation, the EP community must comprehensively explore how artificial intelligence (AI) can be applied to healthcare delivery, research, and education and consider all opportunities in which AI can catalyze innovation; create workflow, research, and education efficiencies; and improve patient outcomes at a lower cost. In this white paper, we define AI and discuss the potential of AI to revolutionize the EP field. We also address the requirements for implementing, maintaining, and enhancing quality when using AI and consider ethical, operational, and regulatory aspects of AI implementation. This manuscript will be followed by several perspective papers that will expand on some of these topics.

Promises and Perils of Consumer Mobile Technologies in Cardiovascular Care: JACC Scientific Statement.

Direct-to-consumer (D2C) wearables are becoming increasingly popular in cardiovascular health management because of their affordability and capability to capture diverse health data. Wearables may enable continuous health care provider-patient partnerships and reduce the volume of episodic clinic-based care (thereby reducing health care costs). However, challenges arise from the unregulated use of these devices, including questionable data reliability, potential misinterpretation of information, unintended psychological impacts, and an influx of clinically nonactionable data that may overburden the health care system. Further, these technologies could exacerbate, rather than mitigate, health disparities. Experience with wearables in atrial fibrillation underscores these challenges. The prevalent use of D2C wearables necessitates a collaborative approach among stakeholders to ensure effective integration into cardiovascular care. Wearables are heralding innovative disease screening, diagnosis, and management paradigms, expanding therapeutic avenues, and anchoring personalized medicine.

Artificial Intelligence in Cardiovascular Care-Part 2: Applications: JACC Review Topic of the Week.

Recent artificial intelligence (AI) advancements in cardiovascular care offer potential enhancements in effective diagnosis, treatment, and outcomes. More than 600 U.S. Food and Drug Administration-approved clinical AI algorithms now exist, with 10% focusing on cardiovascular applications, highlighting the growing opportunities for AI to augment care. This review discusses the latest advancements in the field of AI, with a particular focus on the utilization of multimodal inputs and the field of generative AI. Further discussions in this review involve an approach to understanding the larger context in which AI-augmented care may exist, and include a discussion of the need for rigorous evaluation, appropriate infrastructure for deployment, ethics and equity assessments, regulatory oversight, and viable business cases for deployment. Embracing this rapidly evolving technology while setting an appropriately high evaluation benchmark with careful and patient-centered implementation will be crucial for cardiology to leverage AI to enhance patient care and the provider experience.

Artificial Intelligence for Cardiovascular Care-Part 1: Advances: JACC Review Topic of the Week.

Recent artificial intelligence (AI) advancements in cardiovascular care offer potential enhancements in diagnosis, treatment, and outcomes. Innovations to date focus on automating measurements, enhancing image quality, and detecting diseases using novel methods. Applications span wearables, electrocardiograms, echocardiography, angiography, genetics, and more. AI models detect diseases from electrocardiograms at accuracy not previously achieved by technology or human experts, including reduced ejection fraction, valvular heart disease, and other cardiomyopathies. However, AI's unique characteristics necessitate rigorous validation by addressing training methods, real-world efficacy, equity concerns, and long-term reliability. Despite an exponentially growing number of studies in cardiovascular AI, trials showing improvement in outcomes remain lacking. A number are currently underway. Embracing this rapidly evolving technology while setting a high evaluation benchmark will be crucial for cardiology to leverage AI to enhance patient care and the provider experience.

Duplication of 20p12.3 associated with familial Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White (WPW) syndrome is caused by preexcitation of the ventricular myocardium via an accessory pathway which increases the risk for paroxysmal supraventricular tachycardia. The condition is often sporadic and of unknown etiology in the majority of cases. Autosomal dominant inheritance and association with congenital heart defects or ventricular hypertrophy were described. Microdeletions of 20p12.3 have been associated with WPW syndrome with either cognitive dysfunction or Alagille syndrome. Here, we describe the association of 20p12.3 duplication with WPW syndrome in a patient who presented with non-immune hydrops. Her paternal uncle carries the duplication and has attention-deficit hyperactivity disorder and electrocardiographic findings consistent with WPW. The 769 kb duplication was detected by the Affymetrix Whole Genome-Human SNP Array 6.0 and encompasses two genes and the first two exons of a third gene. We discuss the potential role of the genes in the duplicated region in the pathogenesis of WPW and possible neurobehavioral abnormalities. Our data provide additional support for a significant role of 20p12.3 chromosomal rearrangements in the etiology of WPW syndrome.

Use of a mixed reality system for navigational mapping during cardiac electrophysiological testing does not prolong case duration: A subanalysis from the Cardiac Augmented REality study.

Background: CommandEP™ is a mixed reality (MXR) system for cardiac electrophysiological (EP) procedures that provides a real-time 3-dimensional digital image of cardiac geometry and catheter locations. In a previous study, physicians using the system demonstrated improved navigational accuracy. This study investigated the impact of the CommandEP system on EP procedural times compared to the standard-of-care electroanatomic mapping system (EAMS) display. Objective: The purpose of this retrospective case-controlled analysis was to evaluate the impact of a novel MXR interface on EP procedural times compared to a case-matched cohort. Methods: Cases from the Cardiac Augmented REality (CARE) study were matched for diagnosis and weight using a contemporary cohort. Procedural time was compared from the roll-in and full implementation cohort. During routine EP procedures, operators performed tasks during the postablation waiting phase, including creation of cardiac geometry and 5-point navigation under 2 conditions: (1) EAMS first; and (2) CommandEP. Results: From a total of 16 CARE study patients, the 10 full implementation patients were matched to a cohort of 20 control patients (2 controls:1 CARE, matched according to pathology and age/weight). No statistical difference in total case times between CARE study patients vs control group (118 ± 29 minutes vs 97 ± 20 minutes; P = .07) or fluoroscopy times (6 ± 4 minutes vs 7 ± 6 minutes; P = .9). No significant difference in case duration for CARE study patients comparing roll-in vs full-implementation cohort (121 ± 26 minutes vs 118 ± 29 minutes; P = .96). CommandEP wear time during cases was significantly longer in full implementation cases (53 ± 24 minutes vs 24 ± 5 minutes; P = .0009). During creation of a single cardiac geometry, no significant time difference was noted between CommandEP vs EAMS (284 ± 45 seconds vs 268 ± 43 seconds; P = .1) or fluoroscopy use (9 ± 19 seconds vs 6 ± 18 seconds; P = .25). During point navigation tasks, there was no difference in total time (CommandEP 31 ± 14 seconds vs EAMS 28 ± 15 seconds; P = .16) or fluoroscopy time (CommandEP 0 second vs EAMS 0 second). Conclusion: MXR did not prolong overall procedural time compared to a matched cohort. There was no prolongation in study task completion time. Future studies with experienced CommandEP users directly assessing procedural time and task completion time in a randomized study population would be of interest.

Unusually high association of hypertrophic cardiomyopathy and complex heart defects in children with fasciculoventricular pathways.

INTRODUCTION: Fasciculoventricular pathways (FVPs) are rare causes of preexcitation that do not mediate tachycardias. We report a two-center experience of pediatric patients with FVP and an unexpectedly high association of complex congenital heart defects (CHDs), chromosomal anomalies, and hypertrophic cardiomyopathy. METHODS: A retrospective review of the electrophysiology database at two institutions was performed to identify patients with FVP from January 2000 to January 2011. Medical records of these patients were reviewed for clinical history and course, presence of comorbidities, and details of intracardiac electrophysiology (EP) study. RESULTS: A total of 17 patients were identified. The primary indication for EP study was a preexcitation pattern on electrocardiogram. The majority of patients, 12/17 (71%), were found to have associated cardiac and genetic anomalies. Hypertrophic cardiomyopathy was found in 5/17 (29%) patients, with genetic testing in two patients demonstrating the lysosomal-associated membrane protein 2 mutation (Danon syndrome). Underlying complex CHDs were present in 3/17 (18%) patients. One patient (6%) was status post (s/p) cardiac transplant, one patient had hypertension, and another had Trisomy 21. Other electrophysiologic substrates mediating tachycardia were found in 3/17 (18%) patients. Only 5/17 patients (29%) were otherwise healthy with structurally normal hearts. CONCLUSIONS: In this largest reported series of FVP in children, there is an unusually high association of FVP with complex CHDs, chromosomal anomalies, and hypertrophic cardiomyopathy. Any patient with such disorders and manifest preexcitation should be evaluated with a high index of suspicion for a FVP.

Updates on the inherited cardiac ion channelopathies: from cell to clinical.

OPINION STATEMENT: The inherited channelopathies are a rare, heterogeneous group of diseases with widely variable clinical presentations and courses. Systematic clinical and experimental work has led to identification of disease-causing genetic mutations and their biophysical manifestation. The process by which the knowledge base is developed, from genetic mutation, to cardiac myocyte, to whole heart, and finally to clinical presentation, has dramatically expanded our understanding of these diseases. Most importantly, we can now begin to comprehend how small changes at the genetic level can dramatically influence a patient's clinical course.