Current State of Cardiac Implantable Electronic Device Remote Monitoring in Pediatrics and Congenital Heart Disease: A PACES-Sponsored Quality Improvement Initiative.

Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring (RM) data among pediatric and ACHD centers prior to implementation of a Pediatric and Congenital Electrophysiology  Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors (ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.

Extraction and Digitization of ECG Signals from Standard Clinical Portable Document Format Files for the Principal Component Analysis of T-wave Morphology.

INTRODUCTION: T-wave analysis from standard electrocardiogram (ECG) remains one of the most available clinical and research methods for evaluating myocardial repolarization. T-wave morphology was recently evaluated to aid with diagnosis and characterization of diastolic dysfunction. Unfortunately, PDF stored ECG datasets limit additional numerical post-processing of ECG waveforms. In this study, we apply a simple custom process pipeline to extract and re-digitize T-wave signals and subject them to principal component analysis (PCA) to define primary T-wave shape variations. METHODS: We propose simple pre-processing and digitization algorithms programmable as a MATLAB tool using standard thresholding functions without the need for advanced signal analysis. To validate digitized datasets, we compared clinically standard measurements in 20 different ECGs with the original ECG machine interpreted values as a gold standard. Afterwards, we analyzed 212 individual ECGs for T-wave shape analysis using PCA. RESULTS: The re-digitized signal was shown to preserve the original information as evidenced by excellent agreement between original - machine interpreted and re-digitized clinical variables including heart rate: bias ~ 1 bpm (95% CI: -1.0 to 3.5), QT interval: bias ~ 0.000 ms (95% CI: -0.012 to 0.012), PR interval: bias = -0.015 ms (95% CI: -0.015 to 0.003), and QRS duration: bias = -0.001 ms (95% CI: -0.007 to 0.006). PCA revealed that the first principal component universally modulates the T-wave height or amount of repolarization voltage regardless of the investigated ECG lead. The second and third principal components described variation in the T-wave peak onset and the T-wave peak morphology, respectively. CONCLUSION: This study presents a straightforward method for re-digitizing ECGs stored in the PDF format utilized in many academic electronic medical record systems. This process can yield re-digitized lead specific signals which can be retrospectively analyzed using advanced custom post-processing numerical analysis independent of commercially available platforms.

An International Multicenter Evaluation of Inheritance Patterns, Arrhythmic Risks, and Underlying Mechanisms of CASQ2-Catecholaminergic Polymorphic Ventricular Tachycardia.

BACKGROUND: Genetic variants in calsequestrin-2 (CASQ2) cause an autosomal recessive form of catecholaminergic polymorphic ventricular tachycardia (CPVT), although isolated reports have identified arrhythmic phenotypes among heterozygotes. Improved insight into the inheritance patterns, arrhythmic risks, and molecular mechanisms of CASQ2-CPVT was sought through an international multicenter collaboration. METHODS: Genotype-phenotype segregation in CASQ2-CPVT families was assessed, and the impact of genotype on arrhythmic risk was evaluated using Cox regression models. Putative dominant CASQ2 missense variants and the established recessive CASQ2-p.R33Q variant were evaluated using oligomerization assays and their locations mapped to a recent CASQ2 filament structure. RESULTS: A total of 112 individuals, including 36 CPVT probands (24 homozygotes/compound heterozygotes and 12 heterozygotes) and 76 family members possessing at least 1 presumed pathogenic CASQ2 variant, were identified. Among CASQ2 homozygotes and compound heterozygotes, clinical penetrance was 97.1% and 26 of 34 (76.5%) individuals had experienced a potentially fatal arrhythmic event with a median age of onset of 7 years (95% CI, 6-11). Fifty-one of 66 CASQ2 heterozygous family members had undergone clinical evaluation, and 17 of 51 (33.3%) met diagnostic criteria for CPVT. Relative to CASQ2 heterozygotes, CASQ2 homozygote/compound heterozygote genotype status in probands was associated with a 3.2-fold (95% CI, 1.3-8.0; P=0.013) increased hazard of a composite of cardiac syncope, aborted cardiac arrest, and sudden cardiac death, but a 38.8-fold (95% CI, 5.6-269.1; P<0.001) increased hazard in genotype-positive family members. In vitro turbidity assays revealed that p.R33Q and all 6 candidate dominant CASQ2 missense variants evaluated exhibited filamentation defects, but only p.R33Q convincingly failed to dimerize. Structural analysis revealed that 3 of these 6 putative dominant negative missense variants localized to an electronegative pocket considered critical for back-to-back binding of dimers. CONCLUSIONS: This international multicenter study of CASQ2-CPVT redefines its heritability and confirms that pathogenic heterozygous CASQ2 variants may manifest with a CPVT phenotype, indicating a need to clinically screen these individuals. A dominant mode of inheritance appears intrinsic to certain missense variants because of their location and function within the CASQ2 filament structure.

Intraoperative Epicardial Triventricular Pacing in a Pediatric Patient.

Cardiac resynchronization therapy (CRT) is used as an adjunctive therapy in adults with advanced heart failure but remains less commonly applied in pediatric patients. Further, CRT is traditionally conducted via biventricular transvenous pacing from the right ventricle and coronary sinus to activate the left ventricle and improve electromechanical synchrony; however, triventricular pacing, in which a third ventricular lead is utilized to activate an additional ventricular location, has been shown to be a feasible therapeutic alternative to typical CRT in patients with advanced heart failure or nonresponders. Limited adult studies involving triventricular pacing have been performed to date but no pediatric data are available. Thus, we present the case of a 12-month-old patient with congenital complete heart block and subsequent pacemaker-induced cardiomyopathy in whom triventricular epicardial pacing was applied in an effort to increase the available knowledge.

Atrial undersensing secondary to quiet timer blanking in pediatric and congenital heart disease patients.

BACKGROUND: Atrial undersensing despite normal atrial lead parameters and high amplitude electrograms has been described in adult patients as a consequence of atrial amplifier saturation. Repetitive inputs cause amplifier ringing and undersensing occurs as a consequence of quiet timer blanking. High amplitude ventricular electrograms can also result in atrial blanking secondary to cross-chamber effect. This has not been described in pediatric patients or epicardial pacing systems and specific risk factors are not known. We describe five cases of atrial undersensing in pediatric and congenital heart disease patients with epicardial dual-chamber systems and high ventricular lead outputs. METHODS: This was a retrospective cohort study of all patients with dual-chamber pacing systems cared for at the Heart Institute at Children's Hospital Colorado. RESULTS: Five cases of atrial undersensing with normal atrial lead parameters were found. All were epicardial systems with acceptable lead parameters at implantation and Medtronic (Medtronic, Inc., Minneapolis, MN, USA) generators with unipolar leads. Ventricular pacing outputs were elevated at presentation, median 5.5 V (5-7.5). Presenting symptoms were exercise intolerance (four) and detection on routine monitoring (one). Successful interventions included reduction in ventricular lead pacing output (one), programming of rate response (two), and implantation of an alternative generator manufacturer (two). CONCLUSION: Atrial undersensing secondary to amplifier ringing and quiet timer blanking is an uncommon presentation but may be clinically important in patients with unipolar epicardial atrial leads and high pacing outputs from ventricular leads.