Electrocardiographic findings in patients with arrhythmogenic cardiomyopathy and right bundle branch block ventricular tachycardia.

AIMS: Little is known about patients with right bundle branch block (RBBB)-ventricular tachycardia (VT) and arrhythmogenic cardiomyopathy (ACM). Our aims were: (i) to describe electrocardiogram (ECG) characteristics of sinus rhythm (SR) and VT; (ii) to correlate SR with RBBB-VT ECGs; and (iii) to compare VT ECGs with electro-anatomic mapping (EAM) data. METHODS AND RESULTS: From the European Survey on ACM, 70 patients with spontaneous RBBB-VT were included. Putative left ventricular (LV) sites of origin (SOOs) were estimated with a VT-axis-derived methodology and confirmed by EAM data when available. Overall, 49 (70%) patients met definite Task Force Criteria. Low QRS voltage predominated in lateral leads (n = 37, 55%), but QRS fragmentation was more frequent in inferior leads (n = 15, 23%). T-wave inversion (TWI) was equally frequent in inferior (n = 28, 42%) and lateral (n = 27, 40%) leads. TWI in inferior leads was associated with reduced LV ejection fraction (LVEF; 46 ± 10 vs. 53 ± 8, P = 0.02). Regarding SOOs, the inferior wall harboured 31 (46%) SOOs, followed by the lateral wall (n = 17, 25%), the anterior wall (n = 15, 22%), and the septum (n = 4, 6%). EAM data were available for 16 patients and showed good concordance with the putative SOOs. In all patients with superior-axis RBBB-VT who underwent endo-epicardial VT activation mapping, VT originated from the LV. CONCLUSIONS: In patients with ACM and RBBB-VT, RBBB-VTs originated mainly from the inferior and lateral LV walls. SR depolarization and repolarization abnormalities were frequent and associated with underlying variants.

Corrigendum: Left Ventricular Longitudinal Dyssynchrony by CMR Feature Tracking Is Related to Adverse Prognosis in Advanced Arrhythmogenic Cardiomyopathy.

[This corrects the article DOI: 10.3389/fcvm.2021.712832.].

A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy.

AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.3% reduction of ICD placements with the same proportion of protected patients (P < 0.001). CONCLUSION: Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs (www.arvcrisk.com).

The prevalence of left and right bundle branch block morphology ventricular tachycardia amongst patients with arrhythmogenic cardiomyopathy and sustained ventricular tachycardia: insights from the European Survey on Arrhythmogenic Cardiomyopathy.

AIMS: In arrhythmogenic cardiomyopathy (ACM), sustained ventricular tachycardia (VT) typically displays a left bundle branch block (LBBB) morphology while a right bundle branch block (RBBB) morphology is rare. The present study assesses the VT morphology in ACM patients with sustained VT and their clinical and genetic characteristics. METHODS AND RESULTS: Twenty-six centres from 11 European countries provided information on 954 ACM patients who had ≥1 episode of sustained VT spontaneously documented during patients' clinical course. Arrhythmogenic cardiomyopathy was defined according to the 2010 Task Force Criteria, and VT morphology according to the QRS pattern in V1. Overall, 882 (92.5%) patients displayed LBBB-VT alone and 72 (7.5%) RBBB-VT [alone in 42 (4.4%) or in combination with LBBB-VT in 30 (3.1%)]. Male sex prevalence was 79.3%, 88.1%, and 56.7% in the LBBB-VT, RBBB-VT, and LBBB + RBBB-VT groups, respectively (P = 0.007). First RBBB-VT occurred 5 years after the first LBBB-VT (46.5 ± 14.4 vs 41.1 ± 15.8 years, P = 0.011). An implanted cardioverter-defibrillator was more frequently implanted in the RBBB-VT (92.9%) and the LBBB + RBBB-VT groups (90%) than in the LBBB-VT group (68.1%) (P < 0.001). Mutations in PKP2 predominated in the LBBB-VT (65.2%) and the LBBB + RBBB-VT (41.7%) groups while DSP mutations predominated in the RBBB-VT group (45.5%). By multivariable analysis, female sex was associated with LBBB + RBBB-VT (P = 0.011) while DSP mutations were associated with RBBB-VT (P < 0.001). After a median follow-up of 103 (51-185) months, death occurred in 106 (11.1%) patients with no intergroup difference (P = 0.176). CONCLUSION: RBBB-VT accounts for a significant proportion of sustained VTs in ACM. Sex and type of pathogenic mutations were associated with VT type, female sex with LBBB + RBBB-VT, and DSP mutation with RBBB-VT.

Left Ventricular Longitudinal Dyssynchrony by CMR Feature Tracking Is Related to Adverse Prognosis in Advanced Arrhythmogenic Cardiomyopathy.

Objectives: Left ventricular (LV) involvement has been associated with unfavorable prognosis in arrhythmogenic cardiomyopathy (ACM). We aim to evaluate LV mechanics by cardiovascular magnetic resonance-feature tracking (CMR-FT) in ACM patients with right ventricular (RV) dysfunction. Methods: We retrospectively recruited ACM patients diagnosed according to the revised Task Force Criteria (rTFC) from January 2015 to July 2017. All patients underwent CMR examinations and collections of clinical, electrocardiographic data. The strain and dyssynchrony parameters of LV and RV were analyzed. These patients were followed, and primary study outcome was defined as a composite of cardiovascular events (arrhythmic events and heart transplantation), secondary study outcome included arrhythmic events. Results: Eighty-nine ACM patients (40.40 ± 13.98 years, 67.42% male) were included. LV and RV ejection fractions were 49.12 ± 12.02% and 22.28 ± 10.11%, respectively. During a median (IQR) follow-up for 18.20 (11.60-30.04) months, 30 patients experienced cardiovascular events which included 22 patients who experienced arrhythmic events. Patients with cardiovascular events had impaired LV global longitudinal strain (-10.82 ± 2.77 vs. -12.61 ± 3.18%, p = 0.010), impaired LV global circumferential strain (-11.81 ± 2.40 vs. -13.04 ± 2.83%, p = 0.044), and greater LV longitudinal dyssynchrony (LVLD) (80.98 ± 30.98 vs. 64.23 ± 25.51 ms, p = 0.012) than those without. After adjusting for age, sex, and other confounding factors, LVLD ≥89.15 ms was an independent risk factor for cardiovascular events (HR: 4.50, 95% CI: 1.94 to 10.42; p = 0.001) and for arrhythmic events (HR: 4.79, 95% CI: 1.74 to 13.20; p = 0.003). Conclusions: LVLD by CMR-FT was an independent risk factor for cardiovascular and arrhythmic events in ACM patients in advanced stage, which could provide prognostic value for this subtype.

Facial expressions of Asian people exposed to constructed urban forests: Accuracy validation and variation assessment.

An outcome of building sustainable urban forests is that people's well-being is improved when they are exposed to trees. Facial expressions directly represents one's inner emotions, and can be used to assess real-time perception. The emergence and change in the facial expressions of forest visitors are an implicit process. As such, the reserved character of Asians requires an instrument rating to accurately recognize expressions. In this study, a dataset was established with 2,886 randomly photographed faces from visitors at a constructed urban forest park and at a promenade during summertime in Shenyang City, Northeast China. Six experts were invited to choose 160 photos in total with 20 images representing one of eight typical expressions: angry, contempt, disgusted, happy, neutral, sad, scared, and surprised. The FireFACE ver. 3.0 software was used to test hit-ratio validation as an accuracy measurement (ac.) to match machine-recognized photos with those identified by experts. According to the Kruskal-Wallis test on the difference from averaged scores in 20 recently published papers, contempt (ac. = 0.40%, P = 0.0038) and scared (ac. = 25.23%, P = 0.0018) expressions do not pass the validation test. Both happy and sad expression scores were higher in forests than in promenades, but there were no difference in net positive response (happy minus sad) between locations. Men had a higher happy score but lower disgusted score in forests than in promenades. Men also had a higher angry score in forests. We conclude that FireFACE can be used for analyzing facial expressions in Asian people within urban forests. Women are encouraged to visit urban forests rather than promenades to elicit more positive emotions.

Internal, external and repeated-sprint demands in small-sided games: A comparison between bouts and age groups in elite youth soccer players.

This study investigated the activity profile during small-sided games (SSG) in elite youth soccer players. Internal load (IL) including heart rate (HR) and external load (EL) such as distance covered in different speed-zones (SZ) were collected from forty-eight players of three different teams (U15, U16, U18). The investigation included a total of eighteen 5vs.5 SSGs, each consisting of four 2-minute bouts on a 40x32m pitch during spring season. Total group results (n = 48) showed a reduction in total-distance (p = 0.001; [Formula: see text] = 0.12), high-intensity-running (p = 0.009; [Formula: see text] = 0.09), and low-intensity-running distance (p = 0.028; [Formula: see text] = 0.07) between bouts. Similarly, a reduction in the number of both acceleration-low (p = 0.001; [Formula: see text] = 0.12) and deceleration-high (p = 0.003; [Formula: see text] = 0.11) values was observed. Additionally, time spent in HR-zones 3 and 4 (p≤0.007; [Formula: see text] ≥ 0.10), increased, with a reduction in HR-zone 1 (p = 0.000, [Formula: see text] = 0.25). Age group comparison showed less distance covered in SZ 1 (p≤0.000; [Formula: see text] = 0.56) and greater deceleration-high values (p≤0.038; [Formula: see text] = 0.32) in U15 players compared to other age groups. Further, U15 showed lower values in low-intensity-running compared to U18 (p = 0.038; [Formula: see text] = 0.22). No age-related differences were found for IL and repeated sprint ability (RSA) values. The higher EL in younger age groups should be taken into account when implementing soccer specific SSGs. In addition, HRmean values between 80-85% of HRmax and RSA numbers, which are similar to match-play data, indicate SSGs as an effective training tool to prepare youth soccer athletes for the demands of competition.

Gas Exchange, Water Use Efficiency, and Biomass Partitioning among Geographic Sources of Acer saccharum Subsp. saccharum and Subsp. nigrum Seedlings in Response to Water Stress.

Responses to water stress were measured for sugar maple (Acer saccharum subsp. saccharum Marshall) sources from Oklahoma (Caddo sugar maple), Missouri, Tennessee, Ontario, and a black maple (Acer saccharum subsp. nigrum F. Michx.) source from Iowa. Seedling sources were selected for differences in temperature and precipitation of their geographic origins. Seedlings were preconditioned through moist (watered daily) or dry (watered every 4-7 days) cycles and then exposed to prolonged water stress. As water stress increased, dry preconditioned 17-week-old sugar maple seedlings from Oklahoma, Missouri, and Tennessee, sources from warmer, and/or drier climates with greater restrained photosynthesis, stomatal conductance, and water use efficiency than those from cooler and moister climates. Under imposed water stress, the Ontario and Iowa sourced seedlings increased their root to shoot ratios and decreased their specific leaf area, mechanisms for drought avoidance. However, no corresponding changes in these values occurred for Oklahoma, Missouri, and Tennessee sources and for the variable of leaf wilting across all sources. Results from this study suggest greater tolerance of water stress in the Oklahoma, Missouri, and Tennessee ecotypes from the western and southern range of sugar maple resulted primarily with water use efficiency (WUE) rather than other water stress coping mechanisms. Findings from this study provide evidence to support selection of sugar maples sources for forestation.

Cadherin 2-Related Arrhythmogenic Cardiomyopathy: Prevalence and Clinical Features.

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) is an inherited cardiac disease characterized by fibrofatty replacement of the right and left ventricle, often causing ventricular dysfunction and life-threatening arrhythmias. Variants in desmosomal genes account for up to 60% of cases. Our objective was to establish the prevalence and clinical features of ACM stemming from pathogenic variants in the nondesmosomal cadherin 2 (CDH2), a novel genetic substrate of ACM. METHODS: A cohort of 500 unrelated patients with a definite diagnosis of ACM and no disease-causing variants in the main ACM genes was assembled. Genetic screening of CDH2 was performed through next-generation or Sanger sequencing. Whenever possible, cascade screening was initiated in the families of CDH2-positive probands, and clinical evaluation was performed. RESULTS: Genetic screening of CDH2 led to the identification of 7 rare variants: 5, identified in 6 probands, were classified as pathogenic or likely pathogenic. The previously established p.D407N pathogenic variant was detected in 2 additional probands. Probands and family members with pathogenic/likely pathogenic variants in CDH2 were clinically evaluated, and along with previously published cases, altogether contributed to the identification of gene-specific features (13 cases from this cohort and 11 previously published, for a total of 9 probands and 15 family members). Ventricular arrhythmic events occurred in most CDH2-positive subjects (20/24, 83%), while the occurrence of heart failure was rare (2/24, 8.3%). Among probands, sustained ventricular tachycardia and sudden cardiac death occurred in 5/9 (56%). CONCLUSIONS: In this worldwide cohort of previously genotype-negative ACM patients, the prevalence of probands with CDH2 pathogenic/likely pathogenic variants was 1.2% (6/500). Our data show that this cohort of CDH2-ACM patients has a high incidence of ventricular arrhythmias, while evolution toward heart failure is rare.

Sudden Cardiac Death Prediction in Arrhythmogenic Right Ventricular Cardiomyopathy: A Multinational Collaboration.

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. METHODS: We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. RESULTS: A total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77-10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69-0.80) and calibration slope of 0.95 (95% CI, 0.94-0.98) indicating minimal over-optimism. CONCLUSIONS: LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

Activity Profile of International Female Lacrosse Players.

ABSTRACT: Hauer, R, Tessitore, A, Hauer, K, and Tschan, H. Activity profile of international female lacrosse players. J Strength Cond Res 35(11): 3207-3212, 2021-This study was designed to investigate the activity profile in international women's lacrosse match-play. Data were collected of 10 female Austrian lacrosse national team players (29.1 ± 6.1 years; 64.2 ± 5.0 kg; and 165.3 ± 4.1 cm) during 4 games of an international tournament using global positioning system and heart rate monitor devices. The mean average heart rate was 75.3 ± 5.0% of maximum heart rate (HRmax) with 23 ± 6.1% of total playing time spent in HRzone 4 (80-90% of HRmax). The mean distance covered during match-play was 3,791.6 ± 554.5 m, of which 38 ± 6.5% were performed in speed zone 1 (<7.0 km·h-1). Ten ±4 sprints per game were conducted with 55 ± 3.8% of accelerations and 59 ± 3.1% of decelerations in zone 1, respectively. Defenders showed greater total distance covered compared with other positions (attack: p = 0.035; d = 0.87; confidence interval [CI] = 0.18-1.56 and midfield: p = 0.006; d = 1.66; CI = 0.93-2.40). Midfielders covered less distance in speed zone 1 (p = 0.032; d = 1.91; CI = 0.53-3.28) compared with attackers, and lower numbers of accelerations (p = 0.042; d = 0.96; CI = 0.20-1.72) and decelerations (p = 0.021; d = 1.79; CI = 0.61-2.97) in zone 3 compared with defenders. Furthermore, attackers showed less distance covered in speed zone 1 (p = 0.09; d = 1.62; CI = 0.86-2.38) compared with defenders. The provided activity data could help coaches and practitioners in designing competition-specific programs for athletes. In addition, positional differences should be taken into account for tactical and physiological aspects.

Lacrosse Athletes Load and Recovery Monitoring: Comparison between Objective and Subjective Methods.

Both objective (OM) and subjective (SM) methods are used in athletic studies, regardless of sport type, to identify and analyze load and recovery status of athletes. As little information exists about the comparison of these two methodologies, the aim of this study is to compare and contrast information that defines the relationship between both methods. Twelve international male lacrosse athletes participated in this study over the course of which participants heart-rate-variability and questionnaire-data were collected. Statistical analysis was performed to evaluate changes over time and correlations between used methods. Comparison between baseline values and competition showed a reduction in root-mean-square of successive differences (RMSSD) (p < 0.01) and the proportion of beat-intervals (NN) that differ by more than 50 ms divided by total number of NNs (pNN50) (p < 0.01). Further, RMSSD values showed differences during competition with large effects (p = 0.02; η2 = 0.24). SM (p < 0.01) showed different progression during competition. Correlation was found for used SM and OM, when considered separately. No evidence for a reliable prediction of OM values using SM could be found. According to these findings, we recommend using a combination of SM and OM data to quantify the physiological stress of training and competition, respectively.

Diagnosing arrhythmogenic right ventricular cardiomyopathy by 2010 Task Force Criteria: clinical performance and simplified practical implementation.

AIMS: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is diagnosed by a complex set of clinical tests as per 2010 Task Force Criteria (TFC). Avoiding misdiagnosis is crucial to prevent sudden cardiac death as well as unnecessary implantable cardioverter-defibrillator implantations. This study aims to validate the overall performance of the TFC in a real-world cohort of patients referred for ARVC evaluation. METHODS AND RESULTS: We included patients consecutively referred to our centres for ARVC evaluation. Patients were diagnosed by consensus of three independent clinical experts. Using this as a reference standard, diagnostic performance was measured for each individual criterion as well as the overall TFC classification. Of 407 evaluated patients (age 38 ± 17 years, 51% male), the expert panel diagnosed 66 (16%) with ARVC. The clinically observed TFC was false negative in 7/66 (11%) patients and false positive in 10/69 (14%) patients. Idiopathic outflow tract ventricular tachycardia was the most common alternative diagnosis. While the TFC performed well overall (sensitivity and specificity 92%), signal-averaged electrocardiogram (SAECG, P = 0.43), and several family history criteria (P ≥ 0.17) failed to discriminate. Eliminating these criteria reduced false positives without increasing false negatives (net reclassification improvement 4.3%, P = 0.019). Furthermore, all ARVC patients met at least one electrocardiogram (ECG) or arrhythmia criterion (sensitivity 100%). CONCLUSION: The TFC perform well but are complex and can lead to misdiagnosis. Simplification by eliminating SAECG and several family history criteria improves diagnostic accuracy. Arrhythmogenic right ventricular cardiomyopathy can be ruled out using ECG and arrhythmia criteria alone, hence these tests may serve as a first-line screening strategy among at-risk individuals.