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BACKGROUND: Chronic right ventricular (RV) pacing is associated with deleterious effects on cardiac function. OBJECTIVE: In an observational multicentre study in children with isolated atrioventricular (AV) block receiving chronic ventricular pacing, the importance of the ventricular pacing site on left ventricular (LV) function was investigated. METHODS: Demographics, maternal autoantibody status and echocardiographic measurements on LV end-diastolic and end-systolic dimensions and volumes at age <18 years were retrospectively collected from patients undergoing chronic ventricular pacing (>1 year) for isolated AV block. LV fractional shortening (LVFS) and, if possible LV ejection fraction (LVEF) were calculated. Linear regression analyses were adjusted for patient characteristics. RESULTS: From 27 centres, 297 children were included, in whom pacing was applied at the RV epicardium (RVepi, n = 147), RV endocardium (RVendo, n = 113) or LV epicardium (LVepi, n = 37). LVFS was significantly affected by pacing site (p = 0.001), and not by maternal autoantibody status (p = 0.266). LVFS in LVepi (39 ± 5%) was significantly higher than in RVendo (33 ± 7%, p < 0.001) and RVepi (35 ± 8%, p = 0.001; no significant difference between RV-paced groups, p = 0.275). Subnormal LVFS (LVFS < 28%) was seen in 16/113 (14%) RVendo-paced and 21/147 (14%) RVepi-paced children, while LVFS was normal (LVFS >= 28%) in all LVepi-paced children (p = 0.049). These results are supported by the findings for LVEF (n = 122): LVEF was <50% in 17/69 (25%) RVendo- and in 10/35 (29%) RVepi-paced patients, while LVEF was >= 50% in 17/18 (94%) LVepi-paced patients. CONCLUSION: In children with isolated AV block, permanent ventricular pacing site is an important determinant of LV function, with LVFS being significantly higher with LV pacing than with RV pacing. [ABSTRACT FROM AUTHOR]

'The Doubles,' funded by the National Institute on Drug Abuse, is a seven-episode series of media tools designed to teach thirdand fourth-grade students about the science of drug addiction. The program's curriculum was delivered through a set of DVDs, interactive CD-ROMs, workbooks, or an Internet Site. Results indicate that although the interventions were successful in teaching students about the biological bases of substance abuse, they had little effect on student attitudes. Implications for future studies are discussed. [ABSTRACT FROM AUTHOR]

Space Scouts, funded by the National Institute on Drug Abuse (NIDA) and produced by the Missouri Institute of Mental Health (MIMH), is a three-episode series of media tools designed to teach fourth-, fifth-, and sixth-grade children from African American churches about the science of drug addiction. This article examines the effectiveness of the principles of practice for community-based participatory research used to create Space Scouts. Academic researchers at MIMH collaborated with an inter-faith agency-Committed Caring Faith Communities (CCFC)-and solicited feedback from members of the target audience, their pastors, and other church staff, substance abuse researchers, and curriculum development specialists in order to ensure that the final program would meet the needs of all involved parties. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this systematic review was to summarize national and international guidelines that made recommendations for monitoring patients diagnosed with low-risk cancer. It appraised the quality of guidelines and determined whether the guidelines adequately identified patients for monitoring, specified which tests to use, defined monitoring intervals, and stated triggers for further intervention. It then assessed the evidence to support each recommendation., Study Design and Setting: Following the Preferred Reporting Items for Systematic Reviews and Meta-analyses, we searched PubMed and Turning Research into Practice databases for national and international guidelines' that were written in English and developed or updated between 2012 and 2023. Quality of individual guidelines was assessed using the AGREE II tool., Results: Across the 41 published guidelines, 48 different recommendations were identified: 15 (31%) for prostate cancer, 11 (23%) for renal cancer, 6 (12.5%) for thyroid cancer, and 10 (21%) for blood cancer. The remaining 6 (12.5%) were for brain, gastrointestinal, oral cavity, bone and pheochromocytoma and paraganglioma cancer. When combining all guidelines, 48 (100%) stated which patients qualify for monitoring, 31 (65%) specified which tests to use, 25 (52%) provided recommendations for surveillance intervals, and 23 (48%) outlined triggers to initiate intervention. Across all cancer sites, there was a strong positive trend with higher levels of evidence being associated with an increased likelihood of a recommendation being specific (P = 0.001) and the evidence for intervals was based on expert opinion or other guidance., Conclusion: With the exception of prostate cancer, the evidence base for monitoring low-risk cancer is weak and consequently recommendations in clinical guidelines are inconsistent. There is a lack of direct evidence to support monitoring recommendations in the literature making guideline developers reliant on expert opinion, alternative guidelines, or indirect or nonspecific evidence., Competing Interests: Declaration of competing interest None of the authors have any competing interests to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Clinical guidelines include monitoring blood test abnormalities to identify patients at increased risk of undiagnosed cancer. Noting blood test changes over time may improve cancer risk stratification by considering a patient's individual baseline and important changes within the normal range. We aimed to review the published literature to understand the association between blood test trends and undiagnosed cancer. MEDLINE and EMBASE were searched until 15 May 2023 for studies assessing the association between blood test trends and undiagnosed cancer. We used descriptive summaries and narratively synthesised studies. We included 29 articles. Common blood tests were haemoglobin (24%, n = 7), C-reactive protein (17%, n = 5), and fasting blood glucose (17%, n = 5), and common cancers were pancreatic (29%, n = 8) and colorectal (17%, n = 5). Of the 30 blood tests studied, an increasing trend in eight (27%) was associated with eight cancer types, and a decreasing trend in 17 (57%) with 10 cancer types. No association was reported between trends in 11 (37%) tests and breast, bile duct, glioma, haematological combined, liver, prostate, or thyroid cancers. Our review highlights trends in blood tests that could facilitate the identification of individuals at increased risk of undiagnosed cancer. For most possible combinations of tests and cancers, there was limited or no evidence.

Background: Misinformation in Spanish on social media platforms has contributed to COVID-19 vaccine hesitancy among Latino parents. Brigada Digital de Salud was established to disseminate credible, science-based information about COVID-19 in Spanish on social media., Objective: This study aims to assess participants' reactions to and engagement with Brigada Digital content that sought to increase COVID-19 vaccine uptake among US Latino parents and their children., Methods: We conducted a 5-week intervention in a private, moderator-led Facebook (Meta Platforms, Inc) group with Spanish-speaking Latino parents of children aged ≤18 years (N=55). The intervention participants received 3 to 4 daily Brigada Digital posts and were encouraged to discuss the covered topics through comments and polls. To assess participants' exposure, reactions, and engagement, we used participants' responses to a web-based survey administered at 2 time points (baseline and after 5 weeks) and Facebook analytics to calculate the average number of participant views, reactions, and comments. Descriptive statistics were assessed for quantitative survey items, qualitative responses were thematically analyzed, and quotes were selected to illustrate the themes., Results: Overall, 101 posts were published. Most participants reported visiting the group 1 to 3 times (22/55, 40%) or 4 to 6 (18/55, 33%) times per week and viewing 1 to 2 (23/55, 42%) or 3 to 4 (16/55, 29%) posts per day. Facebook analytics validated this exposure, with 36 views per participant on average. The participants reacted positively to the intervention. Most participants found the content informative and trustworthy (49/55, 89%), easy to understand, and presented in an interesting manner. The participants thought that the moderators were well informed (51/55, 93%) and helpful (50/55, 91%) and praised them for being empathic and responsive. The participants viewed the group environment as welcoming and group members as friendly (45/55, 82%) and supportive (19/55, 35%). The 3 most useful topics for participants were the safety and efficacy of adult COVID-19 vaccines (29/55, 53%), understanding child risk levels (29/55, 53%), and the science behind COVID-19 (24/55, 44%). The preferred formats were educational posts that could be read (38/55, 69%) and videos, including expert (28/55, 51%) and instructional (26/55, 47%) interviews. Regarding engagement, most participants self-reported reacting to posts 1 to 2 (16/55, 29%) or 3 to 4 (15/55, 27%) times per week and commenting on posts 1 to 2 (16/55, 29%) or <1 (20/55, 36%) time per week. This engagement level was validated by analytics, with 10.6 reactions and 3 comments per participant, on average, during the 5 weeks. Participants recommended more opportunities for engagement, such as interacting with the moderators in real time., Conclusions: With adequate intervention exposure and engagement and overall positive participant reactions, the findings highlight the promise of this digital approach for COVID-19 vaccine-related health promotion., (©Anna I González-Salinas, Elizabeth L Andrade, Lorien C Abroms, Kaitlyn Gómez, Carla Favetto, Valeria M Gómez, Karen K Collins. Originally published in JMIR Formative Research (https://formative.jmir.org), 14.03.2024.)

Competing Interests: The authors report no conflicts of interest for the published content. No funding information was provided.

Exertional syncope has been suggested to correlate with a cardiac aetiology, particularly when occurring in mid-stride. The aim of the study is to evaluate the incidence of cardiac disease among children presenting with exertional syncope, determine the influence of timing within activity, and determine the utility of genetic testing and implantable event monitors in the evaluation of cardiac syncope. The patients ≤18 years old with exertional syncope who underwent exercise stress testing between 2008 and 2019 were retrospectively included. Patients were assessed to be in one of three groups: mid-exertion (mid-stride syncope), peri-exertion (syncope during activity but not moving), and post-exertion (within minutes of the activity). A total of 334 patients were included; 46 % were mid-exertion, 18 % were peri-exertion, and 36 % were post-exertion. Thirteen patients (3.8 %) were diagnosed with cardiac syncope; n = 9 (69 %) mid-exertion. Only mid-exertional syncope was significantly associated with a cardiac diagnosis (OR: 2.6). Cardiac diagnoses included inherited arrhythmia syndromes (n = 9), abnormal coronary origins (n = 2), and supraventricular tachycardia (n = 2). Only catecholaminergic polymorphic ventricular tachycardia (n = 5) was associated with mid-exertional syncope (OR: 1.4). The definitive diagnostic test was exercise testing (n = 8), echocardiogram (n = 2), genetic testing (n = 1), ambulatory monitor (n = 1), and EKG (n = 1). Mid-stride syncope was more likely to result in a cardiac diagnosis, and exercise testing is the most common definitive test as catecholaminergic polymorphic ventricular tachycardia was the primary aetiology of exertional syncope in our cohort. Implantable event monitors and genetic testing could be helpful in ruling out cardiac disease.

Adequate nutrition is central to well-being and health and can enhance recovery during illness. Although it is well known that malnutrition, both undernutrition and overnutrition, poses an added challenge for patients with cancer diagnoses, it remains unclear when and how to intervene and if such nutritional interventions improve clinical outcomes. In July 2022, the National Institutes of Health convened a workshop to examine key questions, identify related knowledge gaps, and provide recommendations to advance understanding about the effects of nutritional interventions. Evidence presented at the workshop found substantial heterogeneity among published randomized clinical trials, with a majority rated as low quality and yielding mostly inconsistent results. Other research cited trials in limited populations that showed potential for nutritional interventions to reduce the adverse effects associated with malnutrition in people with cancer. After review of the relevant literature and expert presentations, an independent expert panel recommends baseline screening for malnutrition risk using a validated instrument following cancer diagnosis and repeated screening during and after treatment to monitor nutritional well-being. Those at risk of malnutrition should be referred to registered dietitians for more in-depth nutritional assessment and intervention. The panel emphasizes the need for further rigorous, well-defined nutritional intervention studies to evaluate the effects on symptoms and cancer-specific outcomes as well as effects of intentional weight loss before or during treatment in people with overweight or obesity. Finally, although data on intervention effectiveness are needed first, robust data collection during trials is recommended to assess cost-effectiveness and inform coverage and implementation decisions., (© The Author(s) 2023. Published by Oxford University Press.)

Background: The Advisor™ HD Grid mapping catheter (Abbott Laboratories; Chicago, IL) allows for bipolar electrogram collection in both orthogonal and perpendicular planes, unique when compared to traditional and branch catheters. Experience in pediatric patients and congenital heart disease (CHD) is limited. The purpose of this work was to evaluate the utility and safety of the Advisor™ HD Grid mapping catheter in pediatric and CHD populations., Methods: Retrospective review of all pediatric patients and those with CHD (regardless of age) at Children's Hospital Colorado and University of Colorado undergoing electrophysiologic study in which the Advisor™ HD Grid mapping catheter was utilized., Results: Sixty-five procedures in 60 patients (N = 31 female (47.6%), median age 17 years (15-24.1)) were included. Patients had CHD in 30 procedures (46.1%). Eight-eight arrhythmia substrates were mapped including atrial flutter/intra-atrial reentrant tachycardia (N = 33), focal atrial tachycardia (N = 20), isolated PVCs (N = 10), accessory pathways (N = 9), atrioventricular nodal reentrant tachycardia (N = 7), right ventricular substrate mapping (N = 7), and ventricular tachycardia (N = 2). Median time per map was 11.8 (7.5-20.1) min with 3.2 (± 1.7) maps per procedure and a median of 2634 (1767-7654) points used per map. Patients with CHD required more maps (p < 0.001) and points per map (p < 0.001). Ablation was successful in 92.4% of procedures., Conclusions: The Advisor™ HD Grid mapping catheter is safe and effective in the pediatric and congenital heart disease population. A wide variety of arrhythmia substrates can be mapped with high point density and low mapping time., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: The authors report no conflicts of interest for the published content. No funding information was provided.

Background Electromechanical dyssynchrony is a well described comorbidity in pulmonary arterial hypertension (PAH). ECG-derived measurements reflective of diastolic dysfunction and electromechanical imaging markers are yet to be investigated. In this study we investigated the ECG- derived marker of repolarization dispersion, interval between the peak and end of T wave (TpTe), in pediatric patients with PAH and left ventricular (LV) diastolic dysfunction. Methods and Results We measured TpTe from a standard 12-lead ECG and in 30 children with PAH and matched control subjects. All participants underwent same-day echocardiography and myocardial strain analysis to calculate the diastolic electromechanical discoordination marker diastolic relaxation fraction. When compared with control subjects, patients with PAH had increased TpTe (93±15 versus 81±12 ms, P =0.001) and elevated diastolic relaxation fraction (0.33±0.10 versus 0.27±0.03, P =0.001). Patients with PAH with LV diastolic dysfunction had significantly increased TpTe when compared with patients with PAH without diastolic dysfunction ( P =0.012) and when compared with control group ( P <0.001). Similarly, patients with PAH with LV diastolic dysfunction had increased diastolic relaxation fraction when compared with PAH patients without diastolic dysfunction ( P =0.007) and when compared with control group ( P <0.001). A 10-ms increase in TpTe was significantly associated with 0.023 increase in diastolic relaxation fraction ( P =0.008) adjusting for body surface area, heart rate, right ventricular volumes, and function. Conclusions Prolonged myocardial repolarization and abnormal LV diastolic electromechanical discoordination exist in parallel in children with PAH and are associated with worse LV diastolic function and functional class.

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

Background: Bicuspid aortic valves (BAVs) are associated with accelerated valvular dysfunction. Increasing rates of conduction system disease are seen in patients with calcific tricuspid aortic valves (TAVs). However, little is known regarding the extent of conduction disorders in BAV patients. We sought to determine the extent of infra-hisian conduction pathology among patients with BAVs undergoing EP studies., Methods: We prospectively analyzed patients presenting to the EP laboratory from 2006 to 2017 at our institution. Thirty-three BAV patients had measured HV intervals. Each individual was matched by age and gender to two control patients. Clinical characteristics were collected and compared, and patients followed for outcomes., Results: The BAV cohort had a mean age of 47.8 ± 17.2 years (range 19-76 years). Indications for referral to the EP lab in the BAV cohort included SVT ablation (n = 16), VT ablation (n = 10), and EP study for syncope, pre-syncope, or palpitations (n = 29). Patients with BAVs had a mean HV interval of 58.7 ms ± 18.6 ms, compared to a mean of 47.2 ms ± 9.6 ms for controls (p value = 0.0001). Over a 10-year follow-up period, 9 BAV patients (27%) went on to require permanent pacing compared to 6 patients (9%) in the control group (p value = 0.03)., Conclusion: Compared to patients with TAVs presenting for EP evaluation, individuals with BAVs have longer HV intervals and a significantly increased requirement for pacemaker therapy over long-term follow-up. Closer monitoring of progressive conduction system disease in BAV patients may be warranted.

Background: The ability of transesophageal three-dimensional echocardiography (3DE) to aid in pacemaker lead extraction has not yet been evaluated. 3DE provides real-time evaluation of intracardiac anatomy and the location of pacemaker leads in greater detail than either fluoroscopy or -two-dimensional echocardiography (2DE), aiding in the extraction of such leads, which can be potentially dangerous. We sought to investigate the feasibility and utility of 3DE to visualize intracardiac anatomy and pacemaker leads, and to assist in lead extraction procedures., Methods: We utilized 3DE in nine encounters for eight different patients, to visualize intracardiac anatomy and leads before, during, and after extraction to evaluate the feasibility and utility to aid in the procedure and evaluate for potential sequelae., Results: 3DE was able to identify pertinent intracardiac anatomy and leads in all cases. 3DE detected procedural complications or altered management in five of nine encounters (five of eight patients); this included detection of an avulsed papillary muscle, tricuspid valve leaflet damage, and cast/thrombus after lead removal, as well as adjustment of excess lead slack to avoid future valve damage, or risk stratification of lead removal., Conclusion: 3DE is feasible and adds utility to lead extraction cases by visualizing intracardiac anatomy and leads beyond fluoroscopy or 2DE, providing real-time information during extraction, and identifying potential complications., (© 2021 Wiley Periodicals LLC.)

Objectives: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease., Background: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease., Methods: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed., Results: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%., Conclusions: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations., Competing Interests: Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2020 American College of Cardiology Foundation. All rights reserved.)

Competing Interests: The author reports no conflicts of interest for the published content.

Background: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events., Objective: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation., Methods: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases. Nonpersistent preexcitation was defined as absence/sudden loss of preexcitation on electrocardiogram, Holter monitoring, or exercise stress test. RC-AF was defined as clinical preexcited atrial fibrillation with shortest preexcited R-R interval (SPERRI) ≤ 250 ms. AP effective refractory period (APERP), SPERRI at EPS , and shortest preexcited paced cycle length (SPPCL) were collected. High-risk APs were defined as APERP, SPERRI, or SPPCL ≤ 250 ms., Results: Of 1589 patients, 244 (15%) had nonpersistent preexcitation and 1345 (85%) had persistent preexcitation. There were no differences in sex (58% vs 60% male; P=.49) or age (13.3±3.6 years vs 13.1±3.9 years; P=.43) between groups. Although APERP (344±76 ms vs 312±61 ms; P<.001) and SPPCL (394±123 ms vs 317±82 ms; P<.001) were longer in nonpersistent vs persistent preexcitation, there was no difference in SPERRI at EPS (331±71 ms vs 316±73 ms; P=.15). Nonpersistent preexcitation was associated with fewer high-risk APs (13% vs 23%; P<.001) than persistent preexcitation. Of 61 patients with SCA or RC-AF, 6 (10%) had nonpersistent preexcitation (3 SCA, 3 RC-AF)., Conclusion: Nonpersistent preexcitation was associated with fewer high-risk APs, though it did not exclude the risk of SCA or RC-AF in children with WPW., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

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.

Purpose: Dietary assessment is vital to inform individualized nutrition care and to evaluate the success of interventions aimed at improving diet for participants in cardiac rehabilitation (CR) programs. The purpose of this study was to assess the validity and reliability of an instrument developed to reflect current evidence-informed dietary recommendations advocated to reduce cardiovascular risk., Methods: This study was conducted at a single CR program at the University of North Carolina, Chapel Hill. Two dietary assessments were administered: Picture Your Plate (PYP) and a reference instrument, the Harvard/Willett Food Frequency Questionnaire (HWFFQ). The PYP is a modification of a previously validated instrument, the Dietary Risk Assessment-New Leaf (DRA-New Leaf). Concurrent validity was assessed by comparing the PYP total score with 3 diet quality indexes (Alternative Health Eating Index [AHEI], Dietary Approaches to Stop Hypertension [DASH], and Alternative Mediterranean Diet [aMED]) calculated from the HWFFQ and by assessment of agreement in tertile cross-classification. An intraclass correlation (ICC) was calculated to assess test-retest reliability., Results: Among the 108 participants, crude and adjusted Spearmen correlation coefficients between the PYP and 3 indexes of dietary quality were AHEI-2010 (0.71-0.72), DASH (0.70-0.71), and aMED (0.52-0.58) (P < .0001, all comparisons). Agreement of tertiles comparing PYP and AHEI-2010 was 67% and the score in opposite tertiles was 6%. The weighted kappa value (κw) = 0.71. The test-retest ICC was 0.91 (95% CI, 0.85-0.93; n = 91)., Conclusions: Results support the PYP as a valid and reliable dietary assessment tool for use in CR programs. Continued research in additional CR program populations is recommended.

Background: Children with Wolff-Parkinson-White Syndrome (WPW) are at risk for sudden death. The gold standard for risk stratification in this population is the shortest pre-excited RR interval during atrial fibrillation (SPERRI)., Objective: The purpose of this study was to determine how closely measurements made in the electrophysiology laboratory in patients with WPW compared to SPERRI obtained during an episode of clinical pre-excited atrial fibrillation (Clinical-SPERRI)., Methods: This was a subgroup analysis of a multicenter study of children with WPW. Subjects in our study (N = 49) were included if they had Clinical-SPERRI measured in addition to 1 or more of 3 surrogate measurements: SPERRI obtained during electrophysiological study (EP-SPERRI), accessory pathway effective refractory period (APERP), or shortest pre-excited paced cycle length with 1:1 conduction (SPPCL)., Results: Seventy percent of electrophysiological measurements were made with patients under general anesthesia. Clinical-SPERRI moderately correlated with EP-SPERRI (r = 0.495; P = .012). However, 24% of our patients with Clinical-SPERRI ≤250 ms would have been misclassified as having a low-risk pathway based on EP-SPERRI >250 ms. Clinical-SPERRI did not correlate with APERP or SPPCL (r < 0.3; P >.1). Mean EP-SPERRI, APERP, and SPPCL all were greater than Clinical-SPERRI., Conclusion: Electrophysiology laboratory measurements of pathway characteristics made with patients under general anesthesia do not correlate well with Clinical-SPERRI. Of APERP, SPPCL, and EP-SPERRI, only EP-SPERRI had moderate correlation with Clinical-SPERRI. This study questions the predictive ability of invasive risk stratification with patients under general anesthesia, given that 24% of patients with high-risk Clinical-SPERRI (≤250 ms) had EP-SPERRI that may be considered low risk (>250 ms)., (Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

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

The feasibility of transesophageal 3-dimensional echocardiography to reduce fluoroscopy in pacemaker lead placement has not yet been evaluated. This clinical vignette demonstrates the ability of 3-dimensional echocardiography to visualize intracardiac anatomy and the pacemaker lead and to guide positioning of the lead into the right atrial appendage, thus reducing fluoroscopy by nearly 50%. ( Level of Difficulty: Advanced. ).

Competing Interests: The author reports no conflicts of interest for the published content.

Background: An increasing number of adults with congenital heart disease (CHD) are undergoing catheter ablation for atrial fibrillation (AF). Data on ablation strategy and outcomes in CHD are limited. Rhythm control is often believed to be of greater importance among patients with complex CHD., Objective: The purpose of this study was to examine the safety and efficacy of AF ablation in adult patients with CHD., Methods: A multicenter retrospective analysis was performed of CHD patients undergoing AF ablation. Clinical data were collected, including AF and CHD type, procedural data, and outcomes. Patients were divided into 3 groups (simple, moderate, and severe) based on CHD complexity, as defined by the 2014 PACES/HRS (Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society) consensus statement. One-year procedural success was defined as freedom from recurrent AF, off antiarrhythmic drugs (complete) or off/on previously failed antiarrhythmic drugs (partial)., Results: Overall, 84 CHD patients (mean age 51.5 ± 12.1 years; 65.5% male; 45.2% with paroxysmal AF) undergoing AF ablation (51 simple, 22 moderate, 11 severe complexity) were included. Pulmonary vein isolation was performed in 80 (95.2%), of whom 30 (35.7%) underwent pulmonary vein isolation alone. Overall, complete and complete/partial freedom was achieved at 1 year in 53.1% and 71.6%, respectively, with no significant differences between those with simple, moderate, or severe complexity. There were no major complications and 7 minor complications, and 2 patients died during follow-up., Conclusion: There are dramatic differences in the degree of CHD complexity among patients referred for AF ablation. When performed at experienced centers, AF ablation is safe and effective even among patients with the most complex forms of CHD., (Copyright © 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Axillary implant location is an alternative implant location in patients for cardiac implantable electronic devices (CIEDs) for the purposes of improved cosmetic outcome. The impact from the patient's perspective is unknown. The purpose of this study was to compare scar perception scores and quality of life (QOL) in pediatric patients with axillary CIED implant location versus the standard infraclavicular approach., Methods: This is a multicenter prospective study conducted at eight pediatric centers and it includes patients aged from 8 to 18 years with a CIED. Patients with prior sternotomy were excluded. Scar perception and QOL outcomes were compared between the infraclavicular and axillary implant locations., Results: A total of 141 patients (83 implantable cardioverter defibrillator [ICD]/58 pacemakers) were included, 55 with an axillary device and 86 with an infraclavicular device. Patients with an ICD in the axillary position had better perception of scar appearance and consciousness. Patients in the axillary group reported, on average, a total Pediatric QOL Inventory score that was 6 (1, 11) units higher than the infraclavicular group, after adjusting for sex and race (P = 0.02)., Conclusions: QOL is significantly improved in axillary in comparison to the infraclavicular CIED position, regardless of device type. Scar perception is improved in patients with ICD in the axillary position., (© 2018 Wiley Periodicals, Inc.)

Background: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony., Methods: Fifty-six children with PAH and comprehensive CMR evaluation were stratified based on QRS duration z-score, with electrical dyssynchrony defined as z-score ≥2. Comprehensive biventricular volumetric, dyssynchrony, and strain analysis was performed., Results: Nineteen PAH patients had or developed electrical dyssynchrony. Patients with electrical dyssynchrony had significantly reduced RV ejection fraction (35% vs 50%, p = 0.003) and greater end-diastolic (168 vs 112 ml/m 2 , p = 0.041) and end-systolic (119 vs 57, ml/m 2 , p = 0.026) volumes. Patients with electrical dyssynchrony had reduced RV longitudinal strain (-14% vs -19%, p = 0.007), LV circumferential strain measured at the free wall (-19% vs -22%, p = 0.047), and the LV longitudinal strain in the septal region (-10% vs -15%, p = 0.0268). LV mechanical intraventricular dyssynchrony was reduced in patients with electrical dyssynchrony at the LV free wall (43 vs 19 ms, p = 0.019)., Conclusions: The electrical dyssynchrony is associated with the reduced LV strain, enlarged RV volumes, and reduced biventricular function in children with PAH. CMR assessment of biventricular mechanical function with respect to QRS duration may help to detect pathophysiologic processes associated with progressed PAH., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Ventricular tachyarrhythmias are the most common cause of death in patients with repaired tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. An electrophysiology study (EPS) has been proposed to risk stratify patients with TOF., Objective: We sought to evaluate a perioperative EPS-guided approach to risk stratify patients with TOF undergoing pulmonary valve replacement (PVR) and guide concomitant cryoablation., Methods: A prospective cohort study of patients with TOF undergoing an EPS at the time of PVR from 2006 to 2017 was conducted at 2 centers. Patients inducible at the time of pre-PVR had undergone concomitant cryoablation in addition to PVR. A repeat post-PVR EPS was performed in those initially inducible to guide implantable cardioverter-defibrillator (ICD) implantation., Results: Of 70 patients who underwent a pre-PVR EPS, 34 (49%) had inducible sustained ventricular tachycardia (VT): 25 monomorphic VT and 9 polymorphic VT. Among patients undergoing cryoablation, 14 (45%) had inducible VT and underwent ICD implantation. During a mean follow-up period of 6.1 ± 3.2 years, 3 patients (21%) had appropriate ICD shocks for symptomatic VT. There was an average of 2.3 shocks (range 1-4 shocks), and the mean time to first shock post-device implantation was 3.6 years (range 2.9-4.3 years). Among patients with negative pre- or post-PVR EPS results, 2 had VT requiring radiofrequency ablation and/or subsequent ICD implantation. There were no arrhythmic deaths., Conclusion: A pre-PVR EPS identified patients with higher-risk TOF undergoing PVR. Despite empirical VT cryoablation at the time of PVR, a high percentage of patients remained inducible for VT. In this high-risk cohort, post-PVR EPS evaluation is important to identify patients at risk of VT despite cryoablation., (Copyright © 2018 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population., Background: Children with WPW syndrome are at risk of sudden death., Methods: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects., Results: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia., Conclusions: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an ion channelopathy characterized by ventricular arrhythmia during exertion or stress. Mutations in RYR2-coded Ryanodine Receptor-2 (RyR2) and CASQ2-coded Calsequestrin-2 (CASQ2) genes underlie CPVT1 and CPVT2, respectively. However, prognostic markers are scarce. We sought to better characterize the phenotypic and genotypic spectrum of CPVT, and utilize molecular modelling to help account for clinical phenotypes., Methods and Results: This is a Pediatric and Congenital Electrophysiology Society multicentre, retrospective cohort study of CPVT patients diagnosed at <19 years of age and their first-degree relatives. Genetic testing was undertaken in 194 of 236 subjects (82%) during 3.5 (1.4-5.3) years of follow-up. The majority (60%) had RyR2-associated CPVT1. Variant locations were predicted based on a 3D structural model of RyR2. Specific residues appear to have key structural importance, supported by an association between cardiac arrest and mutations in the intersubunit interface of the N-terminus, and the S4-S5 linker and helices S5 and S6 of the RyR2 C-terminus. In approximately one quarter of symptomatic patients, cardiac events were precipitated by only normal wakeful activities., Conclusion: This large, multicentre study identifies contemporary challenges related to the diagnosis and prognostication of CPVT patients. Structural modelling of RyR2 can improve our understanding severe CPVT phenotypes. Wakeful rest, rather than exertion, often precipitated life-threatening cardiac events., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For Permissions, please email: journals.permissions@oup.com.)

Background: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias., Hypothesis: A measure of atrial dispersion, the P-wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra-atrial re-entrant tachycardia (IART) in a large TOF cohort., Methods: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. History of AFL/IART was documented using electrocardiogram, Holter monitor, exercise stress test, implanted cardiac device, and electrophysiology study. P-R intervals, Pvm, QRS duration, and QRS vector magnitude were assessed from resting sinus-rhythm 12-lead electrocardiograms and identification of those with AFL/IART was determined., Results: Fourteen patients (8.9%) were found to have AFL/IART. Pvm, QRS duration, and QRS vector magnitude significantly differentiated those with AFL/IART from those without on univariate analysis: 0.09 ± 0.04 vs 0.18 ± 0.07 mV, 161.3 ± 21.9 vs 137.7 ± 31.4 ms, and 1.2 (interquartile range, 1.0-1.2) vs 1.6 mV (1.0-2.3), respectively (P < 0.05 for each). The Pvm had the highest area under the ROC curve (0.88) and was the only significant predictor on multivariate analysis, with odds ratio of 0.02 (95% confidence interval: 0.01-0.53). P-R duration, MRI volumes, and right-heart hemodynamics did not significantly differentiate those with vs those without AFL/IART., Conclusions: In TOF patients undergoing pulmonary valve replacement, Pvm has significant value in predicting those with perioperative AFL/IART. These clinical features may help further evaluate TOF patients at risk for perioperative atrial arrhythmias. Prospective studies are warranted., (© 2017 Wiley Periodicals, Inc.)

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., (© 2017 Wiley Periodicals, Inc.)

Objectives: This study sought to test the hypothesis that a vectorcardiographic parameter, the QRS vector magnitude (QRSVm), can risk stratify those patients at risk for sustained spontaneous ventricular arrhythmias (VAs) or ventricular arrhythmia inducibility (VAI) in a large cohort of patients with tetralogy of Fallot (TOF)., Background: Patients with TOF have an increased risk of VAs, but predicting those at risk can often be challenging., Methods: Blinded retrospective analyses of 177 TOF patients undergoing pulmonary valve replacement (PVR) between 1997 and 2015 were performed. VAI was evaluated by programmed electrical stimulation in 48 patients. QRS intervals and QRSVm voltage measurements were assessed from resting 12-lead electrocardiograms, and risk of VA was determined. Clinical characteristics, including imaging and cardiac catheterizations, were used for other modality comparisons., Results: Sustained spontaneous VA occurred in 12 patients and inducible VA in 18 patients. Age and QRSVm were significant univariate predictors of VA. QRSVm was the only independent predictor of VAI (p < 0.001). Using a root mean square QRS value of 1.24 mV, the positive and negative predictive values were 47.9% and 97.8%, respectively, for spontaneous sustained VA. For VAI, using a QRSVm cutoff of 1.31 mV, positive and negative predictive values were 63.0% and 95.3%, respectively., Conclusions: In TOF patients undergoing PVR, older age was associated with increased spontaneous VA risk. Lower QRSVm predicted spontaneous VA or VAI risk with high negative predictive values. QRSVm is the only independent predictor of VAI. These clinical features may help further risk stratify TOF patients requiring therapies to prevent sudden death., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Intermittent Wolff-Parkinson-White (WPW) syndrome is considered to have a lower risk of sudden death. Fewer data exist regarding electrophysiologic (EP) characteristics and the natural history of intermittent WPW in children., Methods: All patients with WPW age 1-18 years at a single institution (1996-2013) were reviewed. Patients with intermittent preexcitation were compared to those with loss of preexcitation on Holter/exercise testing and those with persistent preexcitation. High-risk accessory pathway (AP) was defined as AP effective refractory period (APERP), block cycle length, or shortest preexcited RR interval during atrial fibrillation ≤250 ms., Results: A total of 295 patients were included: 226 (76.6%) persistent, 39 (13.2%) intermittent, and 30 (10.2%) loss of preexcitation Holter/exercise. There were no differences in symptoms between groups. Median interquartile range APERP was significantly longer in intermittent WPW (380 [320, 488] ms vs 320 [300, 350] ms persistent, 310 [290, 330] ms loss of preexcitation Holter/exercise; P = 0.0008). At baseline, there was no difference between groups in frequency of high-risk pathways. However, when isoproterenol values were included, high-risk pathways were more frequent among patients with loss of preexcitation on Holter/exercise (54% vs 16% persistent, 11% intermittent; P = 0.005). There was one death in a patient with loss of preexcitation on exercise testing, no EP study, and prior drug use. A second patient with persistent WPW and APERP 270 ms required resuscitation following a methadone overdose., Conclusion: Intermittent preexcitation in children does not connote a lower risk AP by EP criteria or reduced symptoms. The low number of pediatric WPW patients who develop preexcited atrial fibrillation or sudden death warrants larger studies to investigate these outcomes., (©2015 Wiley Periodicals, Inc.)

The Cardiometabolic Think Tank was convened on June 20, 2014, in Washington, DC, as a "call to action" activity focused on defining new patient care models and approaches to address contemporary issues of cardiometabolic risk and disease. Individual experts representing >20 professional organizations participated in this roundtable discussion. The Think Tank consensus was that the metabolic syndrome (MetS) is a complex pathophysiological state comprised of a cluster of clinically measured and typically unmeasured risk factors, is progressive in its course, and is associated with serious and extensive comorbidity, but tends to be clinically under-recognized. The ideal patient care model for MetS must accurately identify those at risk before MetS develops and must recognize subtypes and stages of MetS to more effectively direct prevention and therapies. This new MetS care model introduces both affirmed and emerging concepts that will require consensus development, validation, and optimization in the future., (Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Catecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia., Methods and Results: This is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8-13.2) years with a delay to diagnosis of 0.5 (0-2.6) years. Syncope (P<0.001), cardiac arrest (P<0.001), and treatment failure (P=0.008) occurred more often in probands. β-Blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least 1 treatment failure event. Implantable cardioverter defibrillators were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and left cardiac sympathetic denervation in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years., Conclusions: This study demonstrates a malignant phenotype and lengthy delay to diagnosis in catecholaminergic polymorphic ventricular tachycardia. Probands were typically severely affected. β-Blockers were almost universally initiated; however, treatment failure, noncompliance and subtherapeutic dosing were often reported. Implantable cardioverter defibrillators were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. Left cardiac sympathetic denervation was not uncommon although the indication was variable., (© 2015 American Heart Association, Inc.)