A novel tool for arrhythmic risk stratification in desmoplakin gene variant carriers.

BACKGROUND AND AIMS: Pathogenic desmoplakin (DSP) gene variants are associated with the development of a distinct form of arrhythmogenic cardiomyopathy known as DSP cardiomyopathy. Patients harbouring these variants are at high risk for sustained ventricular arrhythmia (VA), but existing tools for individualized arrhythmic risk assessment have proven unreliable in this population. METHODS: Patients from the multi-national DSP-ERADOS (Desmoplakin SPecific Effort for a RAre Disease Outcome Study) Network patient registry who had pathogenic or likely pathogenic DSP variants and no sustained VA prior to enrolment were followed longitudinally for the development of first sustained VA event. Clinically guided, step-wise Cox regression analysis was used to develop a novel clinical tool predicting the development of incident VA. Model performance was assessed by c-statistic in both the model development cohort (n = 385) and in an external validation cohort (n = 86). RESULTS: In total, 471 DSP patients [mean age 37.8 years, 65.6% women, 38.6% probands, 26% with left ventricular ejection fraction (LVEF) < 50%] were followed for a median of 4.0 (interquartile range: 1.6-7.3) years; 71 experienced first sustained VA events {2.6% [95% confidence interval (CI): 2.0, 3.5] events/year}. Within the development cohort, five readily available clinical parameters were identified as independent predictors of VA and included in a novel DSP risk score: female sex [hazard ratio (HR) 1.9 (95% CI: 1.1-3.4)], history of non-sustained ventricular tachycardia [HR 1.7 (95% CI: 1.1-2.8)], natural logarithm of 24-h premature ventricular contraction burden [HR 1.3 (95% CI: 1.1-1.4)], LVEF < 50% [HR 1.5 (95% CI: .95-2.5)], and presence of moderate to severe right ventricular systolic dysfunction [HR 6.0 (95% CI: 2.9-12.5)]. The model demonstrated good risk discrimination within both the development [c-statistic .782 (95% CI: .77-.80)] and external validation [c-statistic .791 (95% CI: .75-.83)] cohorts. The negative predictive value for DSP patients in the external validation cohort deemed to be at low risk for VA (<5% at 5 years; n = 26) was 100%. CONCLUSIONS: The DSP risk score is a novel model that leverages readily available clinical parameters to provide individualized VA risk assessment for DSP patients. This tool may help guide decision-making for primary prevention implantable cardioverter-defibrillator placement in this high-risk population and supports a gene-first risk stratification approach.

Long-Term Arrhythmic Follow-Up and Risk Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular Cardiomyopathy.

Background: Patients with likely pathogenic/pathogenic desmoplakin (DSP) variants are poorly characterized. Some of them meet diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC), but it is unclear how risk stratification strategies for ARVC perform in this setting. Objectives: The purpose of this study was to characterize arrhythmic outcomes and to test the performance of the recently validated ARVC risk calculator in patients with DSP likely pathogenic/pathogenic variants fulfilling definite 2010 ARVC Task Force Criteria (DSP-TFC+). Methods: DSP-TFC+ patients were enrolled from 20 institutions across 3 continents. Ventricular arrhythmias (VA), defined as a composite of sustained ventricular tachycardia (VT), appropriate implantable cardioverter defibrillator therapies, and ventricular fibrillation/sudden cardiac death events in follow-up, were reported as the primary outcome. We tested the performance of the ARVC risk calculator for VA prediction, reporting c-statistics. Results: Among 252 DSP-TFC+ patients (age 39.6 ± 16.9 years, 35.3% male), 94 (37.3%) experienced VA over 44.5 [IQR: 19.6-78.3] months. Patients with left ventricle involvement (n = 194) were at higher VA risk (log-rank P = 0.0239). History of nonsustained VT (aHR 2.097; P = 0.004) showed the strongest association with VA occurrence during the first 5-year follow-up. Neither age (P = 0.723) nor male sex (P = 0.200) was associated with VAs at follow-up. In 204 patients without VA at diagnosis, incident VA rate was high (32.8%; 7.37%/y). The ARVC risk calculator performed poorly overall (c-statistic 0.604 [0.594-0.614]) and very poorly in patients with left ventricular disease (c-statistic 0.558 [0.556-0.560]). Conclusions: DSP-TFC+ patients are at substantial risk for VAs. The ARVC risk calculator performs poorly in DSP-TFC+ patients suggesting need for a gene-specific risk algorithm. Meanwhile, DSP-TFC+ patients with nonsustained VT should be considered as high-risk.

A Natural History Study of Timothy Syndrome.

Timothy syndrome (OMIM #601005) is a rare disease caused by variants in the gene CACNA1C . Timothy syndrome patients were first identified as having a cardiac presentation of Long QT and syndactyly of the fingers and/or toes, and an identical variant in CACNA1C , Gly406Arg. However, since this original identification, more individuals harboring diverse variants in CACNA1C have been identified and have presented with various cardiac and extra-cardiac symptoms. Furthermore, it has remained underexplored whether individuals harboring canonical Gly406Arg variants in mutually exclusive exon 8A (Timothy syndrome 1) or exon 8 (Timothy syndrome 2) have additional symptoms. Here, we describe the first Natural History Study for Timothy syndrome, providing a thorough resource describing the current understanding of disease manifestation in Timothy syndrome patients. Parents of Timothy syndrome children were queried regarding a wide-ranging set of symptoms and features via a survey. Importantly, we find that in addition to cardiac concerns, Timothy syndrome patients commonly share extra-cardiac features including neurodevelopmental impairments, hypoglycemia, and respiratory problems. Our work expands the current understanding of the disorder to better inform the care of Timothy syndrome patients.

Honor Values as Identity Content: Evidence From a Three-Wave Longitudinal Study.

Reputation refers to the set of judgments a community makes about its members. In cultures of honor, reputation constitutes one of the most pressing concerns of individuals. Reputational concerns are intimately intertwined with people's social identities. However, research has yet to address the question of how honor-related reputational concerns are structured at the within-person level vis-à-vis individuals' identification with relevant group memberships. The present longitudinal study investigated the association between social identification and reputational concerns in southern Italy (N1st-wave = 1,173), a little-studied culture of honor. Specifically, using a random intercept cross-lagged panel model, we tested whether reputational concerns predict, are predicted by, or are bidirectionally linked to individuals' identification with their region, a group membership relevant for the endorsement of honor. Findings revealed a positive association at the within-person level between group identification and subsequent honor-related concerns. Longitudinal paths from reputational concerns to identification were not significant. Implications of the findings and directions for future research are discussed.

Hypertrophic Cardiomyopathy and Ventricular Preexcitation in the Young: Cause and Accessory Pathway Characteristics.

BACKGROUND: The cause of hypertrophic cardiomyopathy (HCM) in the young is highly varied. Ventricular preexcitation (preexcitation) is well recognized, yet little is known about the specificity for any cause and the characteristics of the responsible accessory pathways (AP). METHODS: Retrospective cohort study of patients <21 years of age with HCM/preexcitation from 2000 to 2022. The cause of HCM was defined as isolated HCM, storage disorder, metabolic disease, or genetic syndrome. Atrioventricular AP (true AP) were distinguished from fasciculoventricular fibers (FVF) using standard invasive electrophysiology study criteria. AP were defined as high risk if any of the following were <250 ms: shortest preexcited RR interval in atrial fibrillation, shortest paced preexcited cycle length, or anterograde AP effective refractory period. RESULTS: We identified 345 patients with HCM and 28 (8%) had preexcitation (isolated HCM, 10/220; storage disorder, 8/17; metabolic disease, 5/19; and genetic syndrome, 5/89). Six (21%) patients had clinical atrial fibrillation (1 with shortest preexcited RR interval <250 ms). Twenty-two patients underwent electrophysiology study which identified 23 true AP and 16 FVF. Preexcitation was exclusively FVF mediated in 8 (36%) patients. Five (23%) patients had AP with high-risk conduction properties (including ≥1 patient in each etiologic group). Multiple AP were seen in 8 (36%) and AP plus FVF in 10 (45%) patients. Ablation was acutely successful in 13 of 14 patients with recurrence in 3. One procedure was complicated by complete heart block after ablation of a high-risk midseptal AP. There were significant differences in QRS amplitude and delta wave amplitude between groups. There were no surface ECG features that differentiated AP from FVF. CONCLUSIONS: Young patients with HCM and preexcitation have a high likelihood of underlying storage disease or metabolic disease. Nonisolated HCM should be suspected in young patients with large QRS and delta wave amplitudes. Surface ECG is not adequate to discriminate preexcitation from a benign FVF from that secondary to potentially life-threatening AP.

Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry.

BACKGROUND: The development of left ventricular systolic dysfunction (LVSD) in hypertrophic cardiomyopathy (HCM) is rare but serious and associated with poor outcomes in adults. Little is known about the prevalence, predictors, and prognosis of LVSD in patients diagnosed with HCM as children. METHODS: Data from patients with HCM in the international, multicenter SHaRe (Sarcomeric Human Cardiomyopathy Registry) were analyzed. LVSD was defined as left ventricular ejection fraction <50% on echocardiographic reports. Prognosis was assessed by a composite of death, cardiac transplantation, and left ventricular assist device implantation. Predictors of developing incident LVSD and subsequent prognosis with LVSD were assessed using Cox proportional hazards models. RESULTS: We studied 1010 patients diagnosed with HCM during childhood (<18 years of age) and compared them with 6741 patients with HCM diagnosed as adults. In the pediatric HCM cohort, median age at HCM diagnosis was 12.7 years (interquartile range, 8.0-15.3), and 393 (36%) patients were female. At initial SHaRe site evaluation, 56 (5.5%) patients with childhood-diagnosed HCM had prevalent LVSD, and 92 (9.1%) developed incident LVSD during a median follow-up of 5.5 years. Overall LVSD prevalence was 14.7% compared with 8.7% in patients with adult-diagnosed HCM. Median age at incident LVSD was 32.6 years (interquartile range, 21.3-41.6) for the pediatric cohort and 57.2 years (interquartile range, 47.3-66.5) for the adult cohort. Predictors of developing incident LVSD in childhood-diagnosed HCM included age <12 years at HCM diagnosis (hazard ratio [HR], 1.72 [CI, 1.13-2.62), male sex (HR, 3.1 [CI, 1.88-5.2), carrying a pathogenic sarcomere variant (HR, 2.19 [CI, 1.08-4.4]), previous septal reduction therapy (HR, 2.34 [CI, 1.42-3.9]), and lower initial left ventricular ejection fraction (HR, 1.53 [CI, 1.38-1.69] per 5% decrease). Forty percent of patients with LVSD and HCM diagnosed during childhood met the composite outcome, with higher rates in female participants (HR, 2.60 [CI, 1.41-4.78]) and patients with a left ventricular ejection fraction <35% (HR, 3.76 [2.16-6.52]). CONCLUSIONS: Patients with childhood-diagnosed HCM have a significantly higher lifetime risk of developing LVSD, and LVSD emerges earlier than for patients with adult-diagnosed HCM. Regardless of age at diagnosis with HCM or LVSD, the prognosis with LVSD is poor, warranting careful surveillance for LVSD, especially as children with HCM transition to adult care.

Gene Therapy for Catecholaminergic Polymorphic Ventricular Tachycardia.

Over the last three decades, the genetic basis of various inherited arrhythmia syndromes has been elucidated, providing key insights into cardiomyocyte biology and various regulatory pathways associated with cellular excitation, contraction, and repolarisation. As varying techniques to manipulate genetic sequence, gene expression, and different cellular pathways have become increasingly defined and understood, the potential to apply various gene-based therapies to inherited arrhythmia has been explored. The promise of gene therapy has generated significant interest in the medical and lay press, providing hope for sufferers of seemingly incurable disorders to imagine a future without repeated medical intervention, and, in the case of various cardiac disorders, without the risk of sudden death. In this review, we focus on catecholaminergic polymorphic ventricular tachycardia (CPVT), discussing the clinical manifestations, genetic basis, and molecular biology, together with current avenues of research related to gene therapy.

Current management of inherited arrhythmia syndromes associated with the cardiac ryanodine receptor.

PURPOSE OF REVIEW: Gain-of-function variants in the gene encoding the cardiac ryanodine receptor ( RYR2 ) are associated with catecholaminergic polymorphic ventricular tachycardia (CPVT). The exercise stress test (EST) has long been fundamental in diagnosis and management, but recent work has further explored its role. A new entity termed calcium release deficiency syndrome (CRDS) has been associated with loss-of-function RYR2 variants and a different arrhythmic phenotype. RECENT FINDINGS: Standard EST is not perfectly reproducible with regards to provocation of arrhythmia in CPVT. A newly described burst EST protocol may be more sensitive in this regard. Nadolol is the most effective beta blocker in CPVT, though arrhythmic events remain frequent and dual therapy with flecainide and/or left cardiac sympathetic denervation may add protection. A recent report renews debate regarding the use of implantable defibrillator therapy in CPVT. CRDS is characterized by later age of presentation, normal/near normal EST, and ventricular arrhythmia induced by a novel ventricular stimulation protocol. SUMMARY: Burst EST may aid in the diagnosis and management of CPVT. Nadolol is the preferred beta blocker in CPVT, and consideration should be given to early dual therapy. CRDS should be suspected in patients with arrhythmic events, rare RYR2 variants, and a phenotype inconsistent with CPVT.

Cascade testing for inherited cardiac conditions: Risk perception and screening after a negative genetic test result.

First-degree relatives of a proband with an inherited cardiac condition (ICC) are offered predictive genetic testing for the pathogenic or likely pathogenic (P/LP) cardiac gene variant (CGV) to clarify their risk for the familial condition. Relatives who test negative for a familial P/LP CGV typically do not require longitudinal cardiac surveillance. To our knowledge, no previous study has investigated adjustment to risk reduction and subsequent screening practices in genotype-negative relatives from an ICC population. We thus investigated risk perception and ongoing screening practices in genotype-negative adults who received cardiac genetic counseling. Correlations between clinical and demographic variables and risk perception and screening practices were also investigated. On average, participants (n = 71) reported a perceived 19.5% lifetime risk of developing the ICC in their family, despite their negative genetic test result. The majority (54%) of participants reported having undergone cardiac screening after disclosure of their negative result. There were no significant correlations between clinical and demographic variables and risk perception or screening practices. Furthermore, risk perception was not found to impact the likelihood of cardiac screening. These findings suggest that even with comprehensive cardiac genetic counseling, a proportion of this population did not accurately comprehend or recall their cardiac disease risk. Additional interventions beyond traditional result disclosure should be explored to help genotype-negative individuals adjust to their reduction in risk for a familial ICC.

Radiofrequency Catheter Ablation for Pediatric Atrioventricular Nodal Reentrant Tachycardia: Impact of Age on Procedural Methods and Durable Success.

Background Catheter-based slow-pathway modification (SPM) is the treatment of choice for symptomatic atrioventricular nodal reentrant tachycardia (AVNRT). We sought to investigate the interactions between patient age and procedural outcomes in pediatric patients undergoing catheter-based SPM for AVNRT. Methods and Results A retrospective cohort study was performed, including consecutive patients undergoing acutely successful SPM for AVNRT from 2008 to 2017. Those with congenital heart disease, cardiomyopathy, and accessory pathways were excluded. Patients were stratified by age quartile at time of SPM. The primary outcome was AVNRT recurrence. A total of 512 patients underwent successful SPM for AVNRT. Age quartile 1 had 129 patients with a median age and weight of 8.9 years and 30.6 kg, respectively. Radiofrequency energy was used in 98% of cases. Follow-up was available in 447 (87%) patients with a median duration of 0.8 years (interquartile range, 0.2-2.5 years). AVNRT recurred in 22 patients. Multivariable Cox proportional hazard modeling identified atypical AVNRT (hazard ratio [HR], 5.83; 95% CI, 2.01-16.96; P=0.001), dual atrioventricular nodal only (HR, 4.09; 95% CI, 1.39-12.02; P=0.011), total radiofrequency lesions (HR, 1.06 per lesion; 95% CI, 1.01-1.12; P=0.032), and the use of a long sheath (HR, 3.52; 95% CI, 1.23-10.03; P=0.010) as predictors of AVNRT recurrence; quartile 1 patients were not at higher risk of recurrence (HR, 0.45; 95% CI, 0.10-1.97; P=0.29). Complete heart block requiring permanent pacing occurred in one quartile 2 patient at 14.9 years of age. Conclusions Pediatric AVNRT can be treated with radiofrequency-SPM with high procedural efficacy and minimal risk of complications, including heart block. Atypical AVNRT and dual atrioventricular nodal physiology without inducible tachycardia remain challenging substrates.

Population Prevalence of Premature Truncating Variants in Plakophilin-2 and Association With Arrhythmogenic Right Ventricular Cardiomyopathy: A UK Biobank Analysis.

BACKGROUND: Truncating variants in the desmosomal gene PKP2 (PKP2tv) cause arrhythmogenic right ventricular cardiomyopathy (ARVC) yet display varied penetrance and expressivity. METHODS: We identified individuals with PKP2tv from the UK Biobank (UKB) and determined the prevalence of an ARVC phenotype and other cardiovascular traits based on clinical and procedural data. The PKP2tv minor allelic frequency in the UKB was compared with a second cohort of probands with a clinical diagnosis of ARVC (ARVC cohort), with a figure of 1:5000 assumed for disease prevalence. In silico predictors of variant pathogenicity (combined annotation-dependent depletion and Splice AI [Illumina, Inc.]) were assessed. RESULTS: PKP2tv were identified in 193/200 643 (0.10%) UKB participants, with 47 unique PKP2tv. Features consistent with ARVC were present in 3 (1.6%), leaving 190 with PKP2tv without manifest disease (UKB cohort; minor allelic frequency 4.73×10-4). The ARVC cohort included 487 ARVC probands with 144 distinct PKP2tv, with 25 PKP2tv common to both cohorts. The odds ratio for ARVC for the 25 common PKP2tv was 0.047 (95% CI, 0.001-0.268; P=2.43×10-6), and only favored ARVC (odds ratio >1) for a single variant, p.Arg79*. In silico variant analysis did not differentiate PKP2tv between the 2 cohorts. Atrial fibrillation was over-represented in the UKB cohort in those with PKP2tv (7.9% versus 4.3%; odds ratio, 2.11; P=0.005). CONCLUSIONS: PKP2tv are prevalent in the population and associated with ARVC in only a small minority, necessitating a more detailed understanding of how PKP2tv cause ARVC in combination with associated genetic and environmental risk factors.

Need satisfaction in intergroup contact: A multinational study of pathways toward social change.

What role does intergroup contact play in promoting support for social change toward greater social equality? Drawing on the needs-based model of reconciliation, we theorized that when inequality between groups is perceived as illegitimate, disadvantaged group members will experience a need for empowerment and advantaged group members a need for acceptance. When intergroup contact satisfies each group's needs, it should result in more mutual support for social change. Using four sets of survey data collected through the Zurich Intergroup Project in 23 countries, we tested several preregistered predictions, derived from the above reasoning, across a large variety of operationalizations. Two studies of disadvantaged groups (Ns = 689 ethnic minority members in Study 1 and 3,382 sexual/gender minorities in Study 2) support the hypothesis that, after accounting for the effects of intergroup contact and perceived illegitimacy, satisfying the need for empowerment (but not acceptance) during contact is positively related to support for social change. Two studies with advantaged groups (Ns = 2,937 ethnic majority members in Study 3 and 4,203 cis-heterosexual individuals in Study 4) showed that, after accounting for illegitimacy and intergroup contact, satisfying the need for acceptance (but also empowerment) is positively related to support for social change. Overall, findings suggest that intergroup contact is compatible with efforts to promote social change when group-specific needs are met. Thus, to encourage support for social change among both disadvantaged and advantaged group members, it is essential that, besides promoting mutual acceptance, intergroup contact interventions also give voice to and empower members of disadvantaged groups. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Sudden Cardiac Arrest in the Paediatric Population.

Sudden cardiac arrest in the young is a rare event with a range of potential causes including cardiomyopathies, ion channelopathies, and autonomic nervous system dysfunction. Investigations into the cause involve a multidisciplinary team, including cardiologists, geneticists, and psychologists. In addition to a detailed medical history, family history and circumstances surrounding the event are important in determining the cause. Clinical investigations including an electrocardiogram are fundamental in diagnosis and should be interpreted cautiously because some children may have atypical presentations and an evolving phenotype. The potential for misdiagnosis exists that could lead to incorrect long-term management strategies. If an inherited condition is suspected, genetic testing of the patient and cascade screening of family members is recommended with genetic counselling and psychological support. Medical management is left to the treating physician acknowledging that a clear diagnosis cannot be made in approximately half of cases. Secondary prevention implantable defibrillators are widely deployed but can be associated with complications in young patients. A plan for safe return to activity is recommended along with a proper transition of care into adulthood. Broad screening of the general population for arrhythmia syndromes is not recommended; preventative measures include screening paediatric patients for risk factors by their primary care physician. Several milestone events or activities that take place in youth could be used as opportunities to promote safety. Further work into risk stratification of this paediatric population through patient registries and greater awareness of cardiopulmonary resuscitation and automated external defibrillator use in saving lives is warranted.

L'arrêt cardiaque subit chez les enfants est un événement rare dont les causes possibles comprennent la cardiomyopathie, la maladie des canaux ioniques ou une dysfonction du système nerveux autonome. Pour cerner la cause exacte, on fait appel à une équipe multidisciplinaire composée notamment de cardiologues, de généticiens et de psychologues. En plus de recueillir les antécédents médicaux complets du patient, il est également important de s'enquérir des antécédents familiaux ainsi que des circonstances entourant l'événement. Les examens cliniques comme un électrocardiogramme sont par ailleurs essentiels pour établir le diagnostic, mais doivent être interprétés avec prudence, car chez certains enfants, le tableau peut être atypique et le phénotype peut évoluer. Une erreur de diagnostic pourrait fausser la stratégie de prise en charge à long terme. Si l'on soupçonne une cause héréditaire, un dépistage génétique est recommandé pour le patient et pour chacun des membres de sa famille de même qu'une consultation génétique et un soutien psychologique. Il revient au médecin traitant de déterminer la conduite à suivre et de ne pas perdre de vue que, dans environ la moitié des cas, il n'est pas possible de poser un diagnostic avec certitude. Les défibrillateurs implantables sont largement employés en prophylaxie secondaire, mais s'accompagnent d'un risque de complications chez les jeunes patients. Un plan de retour prudent à l'activité physique est recommandé, et le jeune patient devra être suivi jusqu'à l'âge adulte. Le dépistage systématique des symptômes de l'arythmie dans la population générale n'est pas recommandé. En revanche, le médecin de première ligne peut, à titre préventif, évaluer les facteurs de risque chez les patients pédiatriques. Plusieurs événements marquants ou étapes clés dans la vie de l'enfant pourraient être l'occasion de promouvoir les mesures de sécurité à cet égard. Enfin, il est nécessaire d'effectuer d'autres études sur la stratification des risques dans la population pédiatrique à l'aide des registres de patients et de promouvoir les manœuvres de réanimation cardiorespiratoire ainsi que le recours aux défibrillateurs externes automatisés pour sauver des vies.

Collective deviance: Scaling up subjective group dynamics to superordinate categories reveals a deviant ingroup protection effect.

Six experiments examined responses to groups whose attitudes deviated from wider social norms about asylum and immigration (in the United Kingdom), or about taxation levels (in the U.S.). Subjective group dynamics (SGD) theory states that people derogate in-group individuals who deviate from prescriptive in-group norms. This enables members to sustain the subjective validity of those norms and, hence, a positive social identity. Research also shows that in-group deviants who accentuate the difference between the in-group and out-group norm (e.g., extremists) are derogated less than deviants who attenuate that difference (e.g., a member who veers toward the outgroup's norm; Abrams et al., 2000). We hypothesize that these effects and the associated group dynamics should scale up when people evaluate deviant groups that are part of larger in-categories. Consistent with SGD theory, participants in Experiments 1, 2, and 3 derogated an in-category attenuating deviant group and upgraded an out-category attenuating deviant group relative to groups that consolidated or accentuated the respective norms of those categories-thereby reinforcing in-category norms relative to out-category norms. Across all experiments, this pattern of differential evaluation was associated with greater subjective validity of the in-category norm. We also hypothesized a novel Deviant Ingroup Protection (DIP) effect, wherein people should curtail derogation of an in-category deviant group when that group is their own. Consistent with this hypothesis, participants in Experiments 4, 5, and 6 evaluated an accentuating in-group, or an attenuating in-group, equally to or more positively than other in-category groups. Implications for political and organizational entrenchment are discussed. (PsycInfo Database Record (c) 2022 APA, all rights reserved).

Exercise Recommendations in Pediatric HCM: Variation and Influence of Provider Characteristics.

Pediatric Hypertrophic Cardiomyopathy (HCM) is associated with sudden cardiac death (SCD) that can be related to physical activity. Without pediatric specific guidelines, recommendations for activity restriction may be varied. Therefore, our aim is to determine the current practice and variability surrounding exercise clearance recommendations (ER) in pediatric HCM referral centers as well as provider and patient characteristics that influence them. We designed a survey that was distributed to the Pediatric Heart Transplant Study (PHTS) providers and members of the Pediatric and Adult Congenital Electrophysiology Society (PACES) querying provider demographics and patient variables from 2 patient vignettes. The study is a multicenter survey of current practice of specialized providers caring for pediatric HCM patients. Survey of PHTS and PACES providers via email to the respective listservs with a response rate of 28% and 91 overall completing the entire survey after self-identifying as providers for pediatric HCM patients at their center. ER varies for pediatric HCM and is associated with provider training background as well as personal and professional history. Of the 91 providers who completed the survey, 42% (N = 38) trained in pediatric electrophysiology (EP), and 40% (N = 36) in pediatric heart failure (HF). Responses varied and only 53% of providers cleared for mild to moderate activity for the patient in Vignette 1, which is more in line with recent published adult guidelines. ER in both vignettes was significantly associated with type of training background. EP providers were more likely to recommend no restriction (27.8% vs 5.9%) than HF providers even when controlling for provider age and time out of training. Syncope with exercise was deemed "Most Important" by 81% of providers when making ER. ER for pediatric HCM are variable and the majority of providers make ER outside of previously published adult guidelines. Furthermore, ER are influenced by provider background and experience. Further study is needed for risks and benefits of physical activity in this population to inform the development of pediatric specific guidelines.

The social cohesion investment: Communities that invested in integration programmes are showing greater social cohesion in the midst of the COVID-19 pandemic.

Social cohesion can rise in the aftermath of natural disasters or mass tragedies, but this 'coming together' is often short-lived. The early stages of the COVID-19 pandemic witnessed marked increases in kindness and social connection, but as months passed social tensions re-emerged or grew anew. Thus local authorities faced persistent and evolving challenges. A cross-sectional survey (N = 2,924) examined perceptions of social cohesion while Britain was slowly emerging from its first national lockdown in June 2020 in six English local authorities that have prioritised investment in social cohesion over the last two years (including five 'integration areas') compared with three other areas that have not. We expected that social cohesion programmes would better equip people to tackle the various challenges of the COVID-19 pandemic. We found a greater sense of social cohesion in the six local authorities (at the micro, meso and macro levels) than in other areas. This was manifested as higher levels of reported social activism, interpersonal trust and closer personal relationships, greater political trust and more positive attitudes towards immigrants. Findings are consistent with the proposition that investing in social cohesion underpins stronger and more connected and open communities, better able to cope with crisis situations.

Intergenerational contact during and beyond COVID-19.

Intergenerational contact is crucial for promoting intergenerational harmony and reducing ageism. However, the COVID-19 pandemic has disrupted and changed the nature and frequency of intergenerational contact. In addition, research suggests that both ageism towards older adults and intergenerational threat regarding succession and consumption, have increased. Through the lens of the Temporally Integrated Model of Intergroup Contact and Threat (TIMICAT; Abrams & Eller, 2016), we explore the implications of these changing dynamics on ageism towards older adults during and beyond the COVID-19 pandemic. Our review reveals that research into intergenerational contact needs to articulate both the time course and salience of contact and threats before making predictions about their impacts on prejudice. The implications of understanding how contact and threat combine to affect ageism for policy and practice are discussed in relation to employment, education, and intergenerational contact programs. We highlight that policy makers play a key role in promoting intergenerational harmony through the reduction of narratives that inflame intergenerational tensions and threat.

Distrustful Complacency and the COVID-19 Vaccine: How Concern and Political Trust Interact to Affect Vaccine Hesitancy.

We test the hypothesis that COVID-19 vaccine hesitancy is attributable to distrustful complacency-an interactive combination of low concern and low trust. Across two studies, 9,695 respondents from different parts of Britain reported their level of concern about COVID-19, trust in the UK government, and intention to accept or refuse the vaccine. Multilevel regression analysis, controlling for geographic area and relevant demographics, confirmed the predicted interactive effect of concern and trust. Across studies, respondents with both low trust and low concern were 10%-22% more vaccine hesitant than respondents with either high trust or high concern, and 26%-29% more hesitant than respondents with both high trust and high concern. Results hold equally among White, Black, and Muslim respondents, consistent with the view that regardless of mean-level differences, a common process underlies vaccine hesitancy, underlining the importance of tackling distrustful complacency both generally and specifically among unvaccinated individuals and populations.

Accessory pathway ablation in Ebstein anomaly: A challenging substrate.

BACKGROUND: Catheter ablation of accessory pathways (APs) in Ebstein anomaly (EA) has been associated with a high recurrence risk. OBJECTIVE: The purpose of this study was to compare outcomes of AP ablation in EA in an early (1990-2004) vs a recent (2005-2019) era and identify variables associated with recurrence. METHODS: A retrospective review of all catheter ablations for supraventricular tachycardia in EA at our institution was performed. RESULTS: We identified 76 patients with median (25th-75th quartiles) age 9 (2.6-13.3) years. Of these patients, 52 had AP alone, 12 had atrial flutter, 3 had atrioventricular nodal reentrant tachycardia, and 9 had AP plus at least 1 additional arrhythmia. Of the 61 patients with APs, a total of 78 separate APs were identified: 40 right-sided, 37 septal, and 1 left-sided. Acute success for AP first procedure was 89% and did not differ between early and recent eras (89% vs 88%; P = .48). However, 19 patients (31%) required repeat procedures (average 1.4 per patient) due to AP recurrence or ablation failure at first attempt. In comparison to early era, recent era ablations had significantly lower recurrence rates at 1 year (62% vs 19%; P = .005). At median follow-up of 2.5 (0.2-7) years, ultimate AP elimination after all procedures was 93%. Younger age at time of electrophysiological study (<2 vs 12-47 years: hazard ratio [HR] 7.3; P = .003) and ablation era (early era vs recent era: HR 3.65; P = .009) predicted recurrence. CONCLUSION: Outcomes for AP ablation in patients with EA have improved, but there is still a relatedly high recurrence risk requiring repeat procedures.