Parental recommendations and exercise attitudes in congenital hearts.

BACKGROUND: Children and young people with CHD benefit from regular physical activity. Parents are reported as facilitators and barriers to their children's physical activity. The aim of this study was to explore parental factors, child factors, and their clinical experience on physical activity participation in young people with CHD. METHODS: An online questionnaire was co-developed with parents (n = 3) who have children with CHD. The survey was then distributed in the United Kingdom by social media and CHD networks, between October 2021 and February 2022. Data were analysed using mixed methods. RESULTS: Eighty-three parents/guardians responded (94% mothers). Young people with CHD were 7.3 ± 5.0 years old (range 0-20 years; 53% female) and 84% performed activity. Parental participation in activity (X2(1) = 6.9, P < 0.05) and perceiving activity as important for their child were positively associated with activity (Fisher's Exact, P < 0.05). Some parents (∼15%) were unsure of the safety of activity, and most (∼70%) were unsure where to access further information about activity. Fifty-two parents (72%) had never received activity advice in clinic, and of the 20 who received advice, 10 said it was inconsistent. Qualitative analysis produced the theme "Knowledge is power and comfort." Parents described not knowing what activity was appropriate or the impact of it on their child. CONCLUSION: Parental participation and attitudes towards activity potentially influence their child's activity. A large proportion of young people performed activity despite a lack and inconsistency of activity advice offered by CHD clinics. Young people with CHD would benefit from activity advice with their families in clinics.

Investigating the Accuracy of Quantitative Echocardiographic-Modified Task Force Criteria for Arrhythmogenic Ventricular Cardiomyopathy in Adolescent Male Elite Athletes.

Athlete preparticipation screening focuses on preventing sudden cardiac death (SCD) by detecting diseases such as arrhythmogenic ventricular cardiomyopathy (AVC), which affects primarily the right ventricular myocardium. Diagnosis may be obscured by physiological remodeling of the athlete heart. Healthy athletes may meet the 2010 Task Force Criteria right ventricular outflow tract (RVOT) dimension cut-offs, questioning the suitability of the modified Task Force Criteria (mTFC) in adolescent athletes. In this study, 67 male adolescent footballers undergoing preparticipation screening were reviewed. All athletes underwent a screening for resting ECG and echocardiogram according to the English FA protocol, as well as cardiopulmonary exercise testing, stress ECG, and exercise echocardiography. Athletes' right ventricular outflow tract (RVOT) that met the major AVC diagnostic criteria for dilatation were identified. Of 67 evaluated athletes, 7 had RVOT dilatation that met the major criteria, all in the long axis parasternal view measurement. All had normal right ventricular systolic function, including normal free-wall longitudinal strain (ranging from - 21.5 to - 32.7%). Left ventricular ejection fraction ranged from 52 to 67%, without evidence of structural changes. Resting ECGs and cardiopulmonary exercise tests were normal in all individuals. In a series of healthy athletes meeting the major AVC diagnostic criteria for RVOT dilatation, none had any other pathological changes on a detailed screening including ECG, exercise testing, and echocardiography. This report highlights that current AVC echocardiographic diagnosis criteria have limitations in this population.

Assessment of a Novel, 22-lead Mobile Electrocardiogram in Elite, Adolescent Footballers.

The 12-lead electrocardiogram is a key component of cardiac screening in elite adolescent footballers. Current technology hampers mobile electrocardiogram monitoring that could reduce the time-to-diagnosis in symptomatic athletes. Recently, a 22-lead mobile electrocardiogram monitor, CardioSecur (Personal MedSystems GmbH), has been approved for use in adults. In this study, the differences in parameter accuracy between CardioSecur's 22-lead electrocardiogram and the gold standard 12-lead electrocardiogram were assessed in elite adolescent footballers (n=31) using Bland-Altman and paired t-tests/Wilcoxon analysis. Agreement between the two devices was clinically acceptable for heart rate (bias=- 0.633 bpm), PR Interval (bias=- 1.73 ms), Bazzett's corrected QTc interval (bias=2.03 ms), T-wave axis (bias=6.55°), P-wave duration (bias=- 0.941 ms), Q-wave amplitude (bias=0.0195 mV), Q-wave duration (bias=1.98 ms), rhythm (bias=0.0333), ST-segment (bias=- 0.0629), J-point analysis (bias=- 0.01) and extended T wave and QRS duration analysis. Unsatisfactory agreement was observed in QRS axis (bias=- 19.4°), P-wave axis (bias=- 0.670°), QRS amplitude (bias=- 0.660 mV), P-wave amplitude (bias=0.0400 mV) and T-wave amplitude (bias=- 0.0675 mV). CardioSecur's 22-lead electrocardiogram agrees with the gold standard in rhythm, durations, T-wave determination in all leads assessed, permitting its use in adolescent footballers for immediate pitch- or track-side analysis.

Characterisation of LV myocardial exercise function by 2-D strain deformation imaging in elite adolescent footballers.

PURPOSE: Few data exist on the descriptions of LV myocardial mechanics and reserve during dynamic exercise of adolescent athletes. The aim of this study was to describe the LV myocardial and cardiopulmonary changes during exercise using 2-D strain deformation imaging. METHODS: Elite adolescent male football players (n = 42) completed simultaneous cardiopulmonary exercise testing (CPET) and exercise echocardiography measurement of LV myocardial deformation by 2-D strain imaging. LV longitudinal and circumferential 2-D strain and strain rates were analyzed at each stage during incremental exercise to a work rate of 150 W. Additionally, exercise LV myocardial deformation and its relation to metabolic exercise parameters were evaluated at each exercise stage and in recovery using repeated measures ANOVA, linear regression and paired t tests. RESULTS: LV peak systolic baseline 2-D strain (longitudinal: - 15.4 ± 2.5%, circumferential: - 22.5 ± 3.1%) increased with each exercise stage, but longitudinal strain plateaued at 50 W (mean strain reserve - 7.8 ± 3.0) and did not significantly increase compared to subsequent exercise stages (P > 0.05), whilst circumferential strain (mean strain reserve - 11.6 ± 3.3) significantly increased (P < 0.05) throughout exercise up to 150 W as the dominant mechanism of exercise LV contractility increase. Regression analyses showed LV myocardial strain increased linearly relative to HR, VO2 and O2 pulse (P < 0.05) for circumferential deformation, but showed attenuation for longitudinal deformation. CONCLUSION: This study describes LV myocardial deformation dynamics by 2-D strain and provides reference values for LV myocardial strain and strain rate during exercise in adolescent footballers. It found important differences between LV longitudinal and circumferential myocardial mechanics during exercise and introduces a methodology that can be used to quantify LV function and cardiac reserve during exercise in adolescent athletes.

Recommendations for participation in competitive sport in adolescent and adult athletes with Congenital Heart Disease (CHD): position statement of the Sports Cardiology & Exercise Section of the European Association of Preventive Cardiology (EAPC), the European Society of Cardiology (ESC) Working Group on Adult Congenital Heart Disease and the Sports Cardiology, Physical Activity and Prevention Working Group of the Association for European Paediatric and Congenital Cardiology (AEPC).

Improved clinical care has led to an increase in the number of adults with congenital heart disease (CHD) engaging in leisure time and competitive sports activities. Although the benefits of exercise in patients with CHD are well established, there is a low but appreciable risk of exercise-related complications. Published exercise recommendations for individuals with CHD are predominantly centred on anatomic lesions, hampering an individualized approach to exercise advice in this heterogeneous population. This document presents an update of the recommendations for competitive sports participation in athletes with cardiovascular disease published by the Sports Cardiology & Exercise section of the European Association of Preventive Cardiology (EAPC) in 2005. It introduces an approach which is based on the assessment of haemodynamic, electrophysiological and functional parameters, rather than anatomic lesions. The recommendations provide a comprehensive assessment algorithm which allows for patient-specific assessment and risk stratification of athletes with CHD who wish to participate in competitive sports.

Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial.

BACKGROUND: Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome. METHODS: We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK. Individuals aged 6-40 years with clinically confirmed Marfan syndrome were eligible for inclusion. Study participants were all given 75 mg open label irbesartan once daily, then randomly assigned to 150 mg of irbesartan (increased to 300 mg as tolerated) or matching placebo. Aortic diameter was measured by echocardiography at baseline and then annually. All images were analysed by a core laboratory blinded to treatment allocation. The primary endpoint was the rate of aortic root dilatation. This trial is registered with ISRCTN, number ISRCTN90011794. FINDINGS: Between March 14, 2012, and May 1, 2015, 192 participants were recruited and randomly assigned to irbesartan (n=104) or placebo (n=88), and all were followed for up to 5 years. Median age at recruitment was 18 years (IQR 12-28), 99 (52%) were female, mean blood pressure was 110/65 mm Hg (SDs 16 and 12), and 108 (56%) were taking ß blockers. Mean baseline aortic root diameter was 34·4 mm in the irbesartan group (SD 5·8) and placebo group (5·5). The mean rate of aortic root dilatation was 0·53 mm per year (95% CI 0·39 to 0·67) in the irbesartan group compared with 0·74 mm per year (0·60 to 0·89) in the placebo group, with a difference in means of -0·22 mm per year (-0·41 to -0·02, p=0·030). The rate of change in aortic Z score was also reduced by irbesartan (difference in means -0·10 per year, 95% CI -0·19 to -0·01, p=0·035). Irbesartan was well tolerated with no observed differences in rates of serious adverse events. INTERPRETATION: Irbesartan is associated with a reduction in the rate of aortic dilatation in children and young adults with Marfan syndrome and could reduce the incidence of aortic complications. FUNDING: British Heart Foundation, the UK Marfan Trust, the UK Marfan Association.

High g-Force Rollercoaster Rides Induce Sinus Tachycardia but No Cardiac Arrhythmias in Healthy Children.

Theme park operators and medical professionals advise children with heart conditions against using rollercoaster rides, but these recommendations are not evidence-based. The underlying assumption is that the combination of adrenergic stimulation through stress and acceleration might trigger arrhythmias in susceptible individuals. We conducted a cross-sectional observational study to assess heart rate and rhythm in healthy children during commercial rollercoaster rides. Twenty healthy children (9 male) aged 11-15 (mean 13.3 ± 1.4) years underwent continuous heart rate and rhythm monitoring (2-lead ECG) from 5 min before until 10 min after each of 4 high speed (>50 km h-1), high g-force (>4) commercial rollercoaster rides. Total recording time was 13 h 20 min. No arrhythmic events were detected. Resting heart rate was 81 ± 10 b min-1 and increased to 158 ± 20 b·min-1 during rides. The highest mean HR (165 ± 23 b min-1) was observed on the ride with the lowest g-force (4.5 g), but one of the highest speeds (100 km h-1). Anticipatory tachycardia (126 ± 15 b min-1) within 5 min was frequently observed. A 10 min recovery HR (124 ± 17 b min-1) was 56 % greater than resting HR. The speed and g-force experienced on roller coasters induce sinus tachycardia but do not elicit pathological arrhythmias in healthy children.

Genome-wide association study identifies loci on 12q24 and 13q32 associated with tetralogy of Fallot.

We conducted a genome-wide association study to search for risk alleles associated with Tetralogy of Fallot (TOF), using a northern European discovery set of 835 cases and 5159 controls. A region on chromosome 12q24 was associated (P = 1.4 × 10(-7)) and replicated convincingly (P = 3.9 × 10(-5)) in 798 cases and 2931 controls [per allele odds ratio (OR) = 1.27 in replication cohort, P = 7.7 × 10(-11) in combined populations]. Single nucleotide polymorphisms in the glypican 5 gene on chromosome 13q32 were also associated (P = 1.7 × 10(-7)) and replicated convincingly (P = 1.2 × 10(-5)) in 789 cases and 2927 controls (per allele OR = 1.31 in replication cohort, P = 3.03 × 10(-11) in combined populations). Four additional regions on chromosomes 10, 15 and 16 showed suggestive association accompanied by nominal replication. This study, the first genome-wide association study of a congenital heart malformation phenotype, provides evidence that common genetic variation influences the risk of TOF.

The relationship between biventricular myocardial performance and metabolic parameters during incremental exercise and recovery in healthy adolescents.

Background left ventricular (LV) and right ventricular (RV) myocardial reserve during exercise in adolescents has not been directly characterized. The aim of this study was to quantify myocardial performance response to exercise by using two-dimensional (2-D) speckle tracking echocardiography and describe the relationship between myocardial reserve, respiratory, and metabolic exercise parameters. A total of 23 healthy boys and girls (mean age 13.2 ± 2.7 yr; stature 159.1 ± 16.4 cm; body mass 49.5 ± 16.6 kg; BSA 1.47 ± 0.33 m(2)) completed an incremental cardiopulmonary exercise test (25 W · 3 min increments) with simultaneous acquisition of 2-D transthoracic echocardiography at rest, each exercise stage up to 100 W, and in recovery at 2 min and 10 min. Two-dimensional LV (LV Sl) and RV (RV Sl) longitudinal strain and LV circumferential strain (LV Sc) were analyzed to define the relationship between myocardial performance reserve and metabolic exercise parameters. Participants achieved a peak oxygen uptake (V̇o 2peak) of 40.6 ± 8.9 ml · kg(-1) · min(-1) and a work rate of 154 ± 42 W. LV Sl and LV Sc and RV Sl increased significantly across work rates (P < 0.05). LV Sl during exercise was significantly correlated to resting strain, V̇o 2peak, oxygen pulse, and work rate (0.530 ≤ r ≤ 0.784, P < 0.05). This study identifies a positive and moderate relationship between LV and RV myocardial performance and metabolic parameters during exercise by using a novel methodology. Relationships detected present novel data directly describing myocardial adaptation at different stages of exercise and recovery that in the future can help directly assess cardiac reserve in patients with cardiac pathology.

Adaptations of aortic and pulmonary artery flow parameters measured by phase-contrast magnetic resonance angiography during supine aerobic exercise.

PURPOSE: Increased oxygen uptake and utilisation during exercise depend on adequate adaptations of systemic and pulmonary vasculature. Recent advances in magnetic resonance imaging techniques allow for direct quantification of aortic and pulmonary blood flow using phase-contrast magnetic resonance angiography (PCMRA). This pilot study tested quantification of aortic and pulmonary haemodynamic adaptations to moderate aerobic supine leg exercise using PCMRA. METHODS: Nine adult healthy volunteers underwent pulse gated free breathing PCMRA while performing heart rate targeted aerobic lower limb exercise. Flow was assessed in mid ascending and mid descending thoracic aorta (AO) and main pulmonary artery (MPA) during exercise at 180 % of individual resting heart rate. Flow sequence analysis was performed by experienced operators using commercial offline software (Argus, Siemens Medical Systems). RESULTS: Exercise related increase in HR (rest: 69 ± 10 b min(-1), exercise: 120 ± 13 b min(-1)) resulted in cardiac output increase (from 6.5 ± 1.4 to 12.5 ± 1.8 L min(-1)). At exercise, ascending aorta systolic peak velocity increased from 89 ± 14 to 122 ± 34 cm s(-1) (p = 0.016), descending thoracic aorta systolic peak velocity increased from 104 ± 14 to 144 ± 33 cm s(-1) (p = 0.004), MPA systolic peak velocity from 86 ± 18 to 140 ± 48 cm s(-1) (p = 0.007), ascending aorta systolic peak flow rate from 415 ± 83 to 550 ± 135 mL s(-1) (p = 0.002), descending thoracic aorta systolic peak flow rate from 264 ± 70 to 351 ± 82 mL s(-1) (p = 0.004) and MPA systolic peak flow rate from 410 ± 80 to 577 ± 180 mL s(-1) (p = 0.006). CONCLUSION: Quantitative blood flow and velocity analysis during exercise using PCMRA is feasible and detected a steep exercise flow and velocity increase in the aorta and MPA. Exercise PCMRA can serve as a research and clinical tool to help quantify exercise blood flow adaptations in health and disease and investigate patho-physiological mechanisms in cardio-pulmonary disease.

The Role of Speckle-Tracking Echocardiography in Predicting Mortality and Morbidity in Patients With Congenital Heart Disease: A Systematic Review and Meta-analysis.

BACKGROUND: Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD. METHODS: The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis. Meta-analysis of association of right ventricle and left ventricle strain (RV Sl and LV Sl, respectively) with major adverse cardiovascular events (MACEs) was performed in atrial switch transposition of the great arteries (asTGA)/congenitally corrected TGA (ccTGA), tetralogy of Fallot (ToF), and congenital aortic stenosis (cAS)/bicuspid aortic valve (BAV). P-value combination analysis was additionally performed for all CHD groups. RESULTS: A total of 33 studies (30 cohorts, n = 8,619 patients, children, and adults) were included. Meta-analysis showed the following parameters as being associated with MACE: RV Sl in asTGA/ccTGA (hazard ratio [HR] = 1.1/%; CI, [1.03; 1.18]), RV Sl and LV Sl in ToF (HR = 1.14/%; CI, [1.03; 1.26] and HR = 1.14/%; CI, [1.08; 1.2], respectively), and LV Sl in cAS/BAV (HR = 1.19/%; CI, [1.15; 1.23]). The RV Sl and strain rate were associated with outcomes also in single ventricle/hypoplastic left heart syndrome (at all palliation stages except before Norwood stage 1) and LV Sl in Ebstein's anomaly. CONCLUSIONS: This systematic review and meta-analysis showed that biventricular strain and strain rate were associated with outcomes in a variety of CHD, highlighting the need for updated recommendations on the use of STE in the current guidelines, specific to disease types.

A new protocol for a single-stage combined cardiopulmonary and echocardiography exercise test: a pilot study.

Aims: The value of cardiopulmonary exercise testing (CPET) and exercise stress echocardiography (ESE) in managing cardiac disease is well known, but no standard CPET-ESE protocol is currently recommended. This pilot study aims to compare feasibility and cardiac function responses between a new high-intensity single-stage combined test (CPET-hiESE) and a standard maximal ESE (smESE). Methods and results: After screening and maximal CPET, all volunteers (n = 21) underwent three ESE modalities: (i) based on the gas exchange threshold (hiESE-GET, 40% of peak-GET, 6 min), (ii) based on heart rate (HR) (hiESE-HR, 80% of peak HR, 6 min), and (iii) smESE (85% of predicted peak HR for age, 3 min). Speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI) were measured at each step. There was superior image quality and data completeness for the right ventricle strain for both hiESE modalities compared with smESE (71.4 and 76.2 vs. 42.9%, P = 0.07). Left ventricular STE data completeness was similar for all three conditions. Despite systematically higher HR, work rate and levels of exertion in the smESE compared with hiESE, STE and TDI parameters were not systematically different. Concordance correlation coefficients ranged from 0.56 to 0.88, lowest for strain rate parameters and mean difference from -0.34 to 1.53, highest for TDI measurements. Conclusion: The novel CPET-hiESE protocol allowed for better data completeness, at lower levels of exertion compared with smESE, without systematically different cardiac reserve measurements in healthy participants. This single-stage protocol can be individualized to clinical populations, which would provide practical advantages to standard testing.

Low-frequency intermediate penetrance variants in the ROCK1 gene predispose to Tetralogy of Fallot.

BACKGROUND: Epidemiological studies indicate a substantial excess familial recurrence of non-syndromic Tetralogy of Fallot (TOF), implicating genetic factors that remain largely unknown. The Rho induced kinase 1 gene (ROCK1) is a key component of the planar cell polarity signalling pathway, which plays an important role in normal cardiac development. The aim of this study was to investigate the role of genetic variation in ROCK1 on the risk of TOF. RESULTS: ROCK1 was sequenced in a discovery cohort of 93 non-syndromic TOF probands to identify rare variants. TagSNPs were selected to capture commoner variation in ROCK1. Novel variants and TagSNPs were genotyped in a discovery cohort of 458 TOF cases and 1331 healthy controls, and positive findings were replicated in a further 209 TOF cases and 1290 healthy controls. Association between genotypes and TOF was assessed using LAMP.A rare SNP (c.807C > T; rs56085230) discovered by sequencing was associated with TOF risk (p = 0.006) in the discovery cohort. The variant was also significantly associated with the risk of TOF in the replication cohort (p = 0.018). In the combined cohorts the odds ratio for TOF was 2.61 (95% CI 1.58-4.30); p < 0.0001. The minor allele frequency of rs56085230 in the cases was 0.02, and in the controls it was 0.007. The variant accounted for 1% of the population attributable risk (PAR) of TOF. We also found significant association with TOF for an uncommon TagSNP in ROCK1, rs288979 (OR 1.64 [95% CI 1.15-2.30]; p = 1.5x10⁻5). The minor allele frequency of rs288979 in the controls was 0.043, and the variant accounted for 11% of the PAR of TOF. These association signals were independent of each other, providing additional internal validation of our result. CONCLUSIONS: Low frequency intermediate penetrance (LFIP) variants in the ROCK1 gene predispose to the risk of TOF.

The use of 2-D speckle tracking echocardiography in assessing adolescent athletes with left ventricular hypertrabeculation meeting the criteria for left ventricular non-compaction cardiomyopathy.

BACKGROUND: Current echocardiographic criteria cannot accurately differentiate exercise induced left ventricular (LV) hypertrabeculation in athletes from LV non-compaction cardiomyopathy (LVNC). This study aims to evaluate the role of speckle tracking echocardiography (STE) in characterising LV myocardial mechanics in healthy adolescent athletes with and without LVNC echocardiographic criteria. METHODS: Adolescent athletes evaluated at three sports academies between 2014 and 2019 were considered for this observational study. Those meeting the Jenni criteria for LVNC (end-systolic non-compacted/compacted myocardium ratio > 2 in any short axis segment) were considered LVNC+ and the rest LVNC-. Peak systolic LV longitudinal strain (Sl), circumferential strain (Sc), rotation (Rot), corresponding strain rates (SRl/c) and segmental values were calculated and compared using a non-inferiority approach. RESULTS: A total of 417 participants were included, mean age 14.5 ± 1.7 years, of which 6.5% were LVNC+ (n = 27). None of the athletes showed any additional LVNC clinical criteria. All average Sl, SRl Sc, SRc and Rot values were no worse in the LVNC+ group compared to LVNC- (p values range 0.0003-0.06), apart from apical SRc (p = 0.2). All 54 segmental measurements (Sl/Sc SRl/SRc and Rot) had numerically comparable means in both LVNC+ and LVNC-, of which 69% were also statistically non-inferior. CONCLUSIONS: Among healthy adolescent athletes, 6.5% met the echocardiographic criteria for LVNC, but showed normal LV STE parameters, in contrast to available data on paediatric LVNC describing abnormal myocardial function. STE could better characterise the myocardial mechanics of athletes with LV hypertrabeculation, thus allowing the transition from structural to functional LVNC diagnosis, especially in suspected physiological remodelling.

The use of 2-D speckle tracking echocardiography in differentiating healthy adolescent athletes with right ventricular outflow tract dilation from patients with arrhythmogenic cardiomyopathy.

AIMS: Echocardiographic assessment of adolescent athletes for arrhythmogenic cardiomyopathy (ACM) can be challenging owing to right ventricular (RV) exercise-related remodelling, particularly RV outflow tract (RVOT) dilation. The aim of this study is to evaluate the role of RV 2-D speckle tracking echocardiography (STE) in comparing healthy adolescent athletes with and without RVOT dilation to patients with ACM. METHODS AND RESULTS: A total of 391 adolescent athletes, mean age 14.5 ± 1.7 years, evaluated at three sports academies between 2014 and 2019 were included, and compared to previously reported ACM patients (n = 38 definite and n = 39 borderline). Peak systolic RV free wall (RVFW-Sl), global and segmental strain (Sl), and corresponding strain rates (SRl) were calculated. The participants meeting the major modified Task Force Criteria (mTFC) for RVOT dilation were defined as mTFC+ (n = 58, 14.8%), and the rest as mTFC- (n = 333, 85.2%). Mean RVFW-Sl was -27.6 ± 3.4% overall, -28.2 ± 4.1% in the mTFC+ group and - 27.5 ± 3.3% in the mTFC- group. mTFC+ athletes had normal RV-FW-Sl when compared to definite (-29% vs -19%, p < 0.001) and borderline ACM (-29% vs -21%, p < 0.001) cohorts. In addition, all mean global and regional Sl and SRl values were no worse in the mTFC+ group compared to the mTFC- (p values range < 0.0001 to 0.1, inferiority margin of 2% and 0.1 s-1 respectively). CONCLUSIONS: In athletes with RVOT dilation meeting the major mTFC, STE evaluation of the RV can demostrate normal function and differentiate physiological remodelling from pathological changes found in ACM, improving screening in grey-area cases.

Evaluation oF FactORs ImpacTing CLinical Outcome and Cost EffectiveneSS of the S-ICD: design and rationale of the EFFORTLESS S-ICD Registry.

BACKGROUND: Leads in and on the heart of the transvenous implantable cardioverter defibrillator (ICD) form the Achilles' heel of this system due to potential for peri- and postimplant complications. The S-ICD is a newer generation of the ICD that does not require leads on the heart or in the vasculature. We present the rationale and study design of the Evaluation oF FactORs ImpacTing CLinical Outcome and Cost EffectiveneSS of the S-ICD (EFFORTLESS S-ICD) Registry which was designed to evaluate the long-term performance of the S-ICD including patient quality of life and long-term resource utilization. METHODS: The Registry is an observational, nonrandomized, standard of care evaluation to be conducted at approximately 50 investigational centers in Europe and New Zealand where the S-ICD is approved for use and distribution. Clinical Registry endpoints include perioperative (30 days postimplant) complication-free rate, 360-day complication-free rate, and percentage of inappropriate shocks for atrial fibrillation and supraventricular ventricular tachyarrhythmia. Other endpoints include patient-reported outcomes (e.g., quality of life) and hospital personnel implant and follow-up experience with the S-ICD system. CONCLUSIONS: Results from EFFORTLESS will build on and expand the initial published experience with the S-ICD, which demonstrated that the device successfully and consistently detects and treats episodes of sustained ventricular tachyarrhythmias. The Registry will also evaluate the patients' perspective of how it is to live with an S-ICD as compared to a contemporary transvenous system and track the experience of implanting physicians and personnel performing patient follow-up with a completely subcutaneous system.

Exercise training in paediatric congenital heart disease: fit for purpose?

Exercise and physical activity (PA) have been shown to be effective, safe and feasible in both healthy children and children with congenital heart disease (CHD). However, implementing exercise training as an intervention is still not routine in children with CHD despite considerable evidence of health benefits and well-being. Understanding how children with CHD can safely participate in exercise can boost participation in PA and subsequently reduce inactivity-related diseases. Home-based exercise intervention, with the use of personal wearable activity trackers, and high-intensity interval training have been beneficial in adults' cardiac rehabilitation programmes. However, these remain underutilised in paediatric care. Therefore, the aims of this narrative review were to synthesise prescribed exercise interventions in children with CHD, identify possible limitation to exercise training prescription and provide an overview on how to best integrate exercise intervention effectively for this population into daily practice.

The Role of Speckle Tracking Echocardiography in the Evaluation of Common Inherited Cardiomyopathies in Children and Adolescents: A Systematic Review.

Speckle tracking echocardiography (STE) has gained importance in the evaluation of adult inherited cardiomyopathies, but its utility in children is not well characterized. We conducted a systematic review to evaluate the role of STE in pediatric inherited cardiomyopathies. PubMed, EMBASE, Web of Science, Scopus, CENTRAL and CINAHL databases were searched up to May 2020, for terms related to inherited cardiomyopathies and STE. Included were dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), left ventricular non-compaction (LVNC) and arrhythmogenic cardiomyopathy (ACM). A total of 14 cohorts were identified, of which six were in DCM, four in HCM, three in LVNC and one in ACM. The most commonly reported STE measurements were left ventricular longitudinal strain (Sl), circumferential strain (Sc), radial strain (Sr) and rotation/torsion/twist. Sl, Sc and were abnormal in all DCM and LVNC cohorts, but not in all HCM. Apical rotation and twist/torsion were increased in HCM, and decreased in LVNC. Abnormal STE parameters were reported even in cohorts with normal non-STE systolic/diastolic measurements. STE in childhood cardiomyopathies can detect early changes which may not be associated with changes in cardiac function detectable by non-STE methods. Longitudinal and circumferential strain should be introduced in the cardiomyopathy echocardiography protocol, reflecting current practice in adults.

The adolescent athlete's heart; A miniature adult or grown-up child?

The systematic development of early age talent in sports academies has led to the professionalization of pediatric sport and the sports physician need to be aware of pediatric cardiological problems. Research into the medical cardiac care and assessment of the pediatric athlete are accumulating, but specific pediatric international guidelines are not available yet and reference data for ECG and echocardiography are incomplete, in particular for the age group <12 years of age. This article is an introduction to the physiological and diagnostics specifics of the pediatric athlete. The focus lies in the differences in presentation and diagnosis between pediatric and adult athletes for the most common pathologies. Reference data for electrical and structural adaptations to intensive exercise are sparse particularly in athletes aged below 12 years old. Training related changes include decrease of resting heart rate, increase of cardiac output, ventricular cavity size, and wall thickness. Cardiac hypertrophy is less pronounced in pediatric athletes, as HR mediated cardiac output increase to endurance exercise is the dominant mechanism in peripubertal children. As in adults, the most pronounced cardiovascular adaptations appear in classical endurance sports like rowing, triathlon, and swimming, but the specifics of pediatric ECG and echocardiographic changes need to be considered.

Initial experience of pacing with a lumenless lead system in patients with congenital heart disease.

BACKGROUND: Long-term pacing is frequently necessary in patients with congenital heart disease (CHD). Preservation of ventricular function and avoidance of venous occlusion is important in these patients. Site-selective pacing with a smaller diameter lead is achievable with the model 3830 lead (SelectSecure, Medtronic Inc., Minneapolis, MN, USA), which was specifically designed to target these complications. We describe our initial experience with the Model 3830 lead in patients with CHD. METHODS: Retrospective analysis of all patients undergoing site-selective implantation of a Model 3830 lead(s) from two congenital heart centers (Bristol, UK, and Dublin, Ireland) from October 2004 until February 2008. RESULTS: We implanted 139 SelectSecure leads (atrial n=70; ventricular n=69) in 90 patients (57 male) with CHD. Median age at implantation: 13.4 years (1.1-59.2 years), median weight: 43 kg. Sixty-nine patients (76%) were children (<18 years). Indications for lead implantation included atrioventricular block (n=55), sinus node disease (n=18), implantable cardiac defibrillator (n=12), antitachycardia pacing (n=4), and cardiac resynchronization (n=1). Twenty-two patients underwent pre-existing lead extraction during the same procedure. All the attempted procedures resulted in successful pacing. One patient had a significantly raised threshold at implantation. There was no procedural mortality. There were two procedural complications. Three patients required lead repositioning for increasing thresholds early postprocedure (<6 weeks). Four leads (2.9%) had displaced on median follow-up of 21.8 months (0.5-42 months). CONCLUSIONS: The Model 3830 lead is safe and effective in patients with CHD. This is a technically challenging patient group yet procedural complication and lead displacement rates are acceptable.