What is the most appropriate age for the first cardiac screening of athletes?

For sporting organisations that conduct screening of athletes, there are very few consistent guidelines on the age at which to start. Our review found the total rate of sudden cardiac arrest or death is very low between the ages of 8-11 years (less than 1/100,000/year), increasing to 1-2/100,000/year in both elite athletes and community athletes aged 12-15 years and then steadily increases with age. The conditions associated with sudden cardiac death in paediatric athletes and young adult athletes are very similar with some evidence that death from coronary artery abnormalities occurs more frequently in athletes 10-14 years old. The decision when to begin a screening program involves a complex interplay between requirements and usual practices in a country, the rules of different leagues and programs, the age of entry into an elite program, the underlying risk of the population and the resources available. Given the incidence of sudden cardiac arrest or death in young people, we recommend beginning cardiac screening no earlier than 12 years (not later than 16 years). The risk increases with age, therefore, starting a program at any point after age 12 has added value. Importantly, anyone with concerning symptoms (e.g. collapse on exercise) or family history of an inherited cardiac condition should see a physician irrespective of age. Finally, no screening program can capture all abnormalities, and it is essential for organisations to implement a cardiac emergency plan including training on recognition and response to sudden cardiac arrest and prompt access to resuscitation, including defibrillators.

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.

Medical services at the FIFA world cup Qatar 2022.

OBJECTIVE: The Football World Cup is among the biggest sporting events in the world, but data to inform the requirements of medical care for such tournaments are limited. This study describes the athlete and team medical services at the FIFA World Cup Qatar 2022 . METHODS: Three different medical service entities were identified through a needs analysis based on expert advice, team physician interviews and questionnaires prior to the event: 'Team Services' to provide any workforce or equipment needs of the teams, a 'Polyclinic' to manage any acute medical demands, and a 'recovery centre' to improve game readiness throughout the tournament. All services had been set up prior to the tournament and thoroughly tested. RESULTS: Of a total of 832 athletes, ~1300 team delegation and ~130 match officials, 167 individuals including 129 (77%) athletes and 38 (23%) non-athletes were assessed in the polyclinic. For the 129 athletes (median 4 players per team), medical imaging was the most requested service, which peaked during the group phase of the tournament. Most requests were received during normal working hours despite many games finishing late at night. 30 of the 32 participating teams solicited medical services for their players at least once. Three teams made use of the recovery facilities, and 17 teams requested additional medical equipment or clinical assistance. CONCLUSION: Central imaging services was the most used medical resource at the FIFA World Cup Qatar 2022, and over half of teams required additional medical equipment or personnel. These data may inform planning of medical services for similar events in the future.

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.

A New Tool to Aid the Differential Diagnosis of Physiological Remodelling from Cardiac Pathology When Assessing Left Ventricle, Left Atrial and Aortic Structure and Function in Male Arab and Black Paediatric Athletes.

Aim: To determine if published Z-scores for left ventricular (LV), left atrial (LA) and aortic structure as well as indices of LV function (Doppler and TDI) in paediatric athletes and non-athletes are appropriate for application in male Arab and black paediatric athletes. If inappropriate, we aim to provide new nomograms and Z-scores for clinical application. Methods: 417 (297 Arab, 120 black) male paediatric (11-18 years) athletes, were evaluated by 2D echocardiography as per British Society of Echocardiography recommendations, and biological age (by radiological X-ray) assessment. Z-scores were tested by residual and correlation analysis together with visual inspection. New Z-scores involved allometric (a*BSA(b+c*chronological age)) and second-order polynomial (y=a*chronological age2+b*chronological age+c) equations for measures of cardiac size and indices of LV function, respectively. Results: Residual linear regression, correlation analysis and visual inspection revealed published z-scores in white peri-pubertal footballers and paediatric non-athletes to be inappropriate for application in male Arab and black paediatric athletes. Residual linear regression revealed new Z-scores for measures of LV, LA and aortic root size to be independent of BSA, ethnicity, chronological and biological age. Residual linear regression revealed new Z-scores for measures of function to be independent of chronological age. Conclusion: Our new z-scores may aid differential diagnosis of suspected pathology versus physiology remodelling, in cardiac screening of the Arab and black paediatric athlete. Nomograms are provided to assist the tracking of the paediatric athlete necessitating annual follow-up and Excel z-score calculation to facilitate use in day-to-day practice.

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.

Protecting the stars of tomorrow: do international cardiovascular preparticipation screening policies account for the paediatric athlete? A systematic review and quality appraisal.

OBJECTIVE: (1) Identify and review current policies for the cardiovascular screening of athletes to assess their applicability to the paediatric population and (2) evaluate the quality of these policy documents using the Appraisal of Guidelines for Research & Evaluation II (AGREE II) tool. DESIGN: Systematic review and quality appraisal of policy documents. DATA SOURCES: A systematic search of PubMed, MEDLINE, Scopus, Web of Science, SportDiscus and CINAHL. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: An article was included if it was a policy/position statement/guideline/consensus or recommendation paper relating to athletes and cardiovascular preparticipation screening. RESULTS AND SUMMARY: Of the 1630 articles screened, 13 met the inclusion criteria. Relevance to paediatric athletes was found to be high in 3 (23%), moderate in 6 (46%) and low in 4 (31%), and only 2 provide tailored guidance for the athlete aged 12-18 years. A median 5 related citations per policy investigated solely paediatric athletes, with study designs most commonly being retrospective (72%). AGREEII overall quality scores ranged from 25% to 92%, with a median of 75%. The lowest scoring domains were rigour of development; (median 32%) stakeholder involvement (median 47%) and Applicability (median 52%). CONCLUSION: Cardiac screening policies for athletes predominantly focus on adults, with few providing specific recommendations for paediatric athletes. The overall quality of the policies was moderate, with more recent documents scoring higher. Future research is needed in paediatric athletes to inform and develop cardiac screening guidelines, to improve the cardiac care of youth athletes.

How to evaluate resting ECG and imaging in children practising sport: a critical review and proposal of an algorithm for ECG interpretation.

The athlete's heart is a well-known phenomenon in adults practising competitive sports. Unfortunately, to date, most of the studies on training-induced cardiac remodelling have been conducted in adults and the current recommendations refer mainly to adult individuals. However, an appropriate interpretation of resting ECG and imaging in children practising sports is crucial, given the possibility of early detect life-threatening conditions and managing therapy and eligibility to sports competitions in the rapidly growing paediatric athlete population. While several articles have been published on this topic in adult athletes, a practical guide for the clinical evaluation of paediatric athletes is still missing. In this critical review, we provided a comprehensive description of the current evidence on training-induced remodelling in paediatric athletes with a practical approach for clinicians on how to interpret the resting 12-lead ECG and cardiac imaging in the paediatric athlete. Indeed, given that training may mimic potential cardiovascular disorders, clinicians evaluating children practising sports should pay attention to the risk of missing a diagnosis of a life-threatening condition. However, this risk should be balanced with the risk of overdiagnosis and unwarranted disqualification from sports practice, when interpreting an ECG as pathological while, on the contrary, it may represent a physiological expression of athlete's heart. Accordingly, we proposed an algorithm for the evaluation of normal, borderline, and abnormal ECG findings that can be useful for the readers for their daily clinical practice.

Indications and utility of cardiac genetic testing in athletes.

Sports Cardiology practice commonly involves the evaluation of athletes for genetically determined cardiac conditions that may predispose to malignant arrhythmias, heart failure, and sudden cardiac death. High-level exercise can lead to electrical and structural cardiac remodelling which mimics inherited cardiac conditions (ICCs). Differentiation between 'athlete's heart' and pathology can be challenging and often requires the whole armamentarium of available investigations. Genetic studies over the last 30 years have identified many of the genetic variants that underpin ICCs and technological advances have transformed genetic testing to a more readily available and affordable clinical tool which may aid diagnosis, management, and prognosis. The role of genetic testing in the evaluation and management of athletes with suspected cardiac conditions is often unclear beyond the context of specialist cardio-genetics centres. This document is aimed at physicians, nurses, and allied health professionals involved in the athlete's care. With the expanding role and availability of genetic testing in mind, this document was created to address the needs of the broader sports cardiology community, most of whom work outside specialized cardio-genetics centres, when faced with the evaluation and management of athletes with suspected ICC. The first part of the document provides an overview of basic terminology and principles and offers guidance on the appropriate use of genetic testing in the assessment of such athletes. It outlines key considerations when contemplating genetic testing, highlighting the potential benefits and pitfalls, and offers a roadmap to genetic testing. The second part of the document presents common clinical scenarios in Sports Cardiology practice, outlining the diagnostic, prognostic, and therapeutic implications of genetic testing, including impact on exercise recommendations. The scope of this document does not extend to a comprehensive description of the genetic basis, investigation, or management of ICCs.

Cardiac screening prior to return to play after SARS-CoV-2 infection: focus on the child and adolescent athlete: A Clinical Consensus Statement of the Task Force for Childhood Health of the European Association of Preventive Cardiology.

Cardiac sequelae after COVID-19 have been described in athletes, prompting the need to establish a return-to-play (RTP) protocol to guarantee a safe return to sports practice. Sports participation is strongly associated with multiple short- and long-term health benefits in children and adolescents and plays a crucial role in counteracting the psychological and physical effects of the current pandemic. Therefore, RTP protocols should be balanced to promote safe sports practice, particularly after an asymptomatic SARS-CoV-2 infection that represents the common manifestation in children. The present consensus document aims to summarize the current evidence on the cardiac sequelae of COVID-19 in children and young athletes, providing key messages for conducting the RTP protocol in paediatric athletes to promote a safe sports practice during the COVID-19 era.

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.

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.

The role of cardiopulmonary exercise testing in predicting mortality and morbidity in people with congenital heart disease: a systematic review and meta-analysis.

AIMS: The role of cardiopulmonary exercise testing (CPET) in predicting major adverse cardiovascular events (MACE) in people with congenital heart disease (ConHD) is unknown. A systematic review with meta-analysis was conducted to report the associations between CPET parameters and MACE in people with ConHD. METHODS AND RESULTS: Electronic databases were systematically searched on 30 April 2020 for eligible publications. Two authors independently screened publications for inclusion, extracted study data, and performed risk of bias assessment. Primary meta-analysis pooled univariate hazard ratios across studies. A total of 34 studies (18 335 participants; 26.2 ± 10.1 years; 54% ± 16% male) were pooled into a meta-analysis. More than 20 different CPET prognostic factors were reported across 6 ConHD types. Of the 34 studies included in the meta-analysis, 10 (29%), 23 (68%), and 1 (3%) were judged as a low, medium, and high risk of bias, respectively. Primary univariate meta-analysis showed consistent evidence that improved peak and submaximal CPET measures are associated with a reduce risk of MACE. This association was supported by a secondary meta-analysis of multivariate estimates and individual studies that could not be numerically pooled. CONCLUSION: Various maximal and submaximal CPET measures are prognostic of MACE across a variety of ConHD diagnoses. Further well-conducted prospective multicentre cohort studies are needed to confirm these findings.

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.

Self-regulation in Barth syndrome: a qualitative perspective of adolescents, adults and parents in the U.K.

BACKGROUND: Barth syndrome (BS) is a life-threatening genetic disease caused by abnormal lipids in the mitochondria of cells and mostly affects young males. Those living with BS have severe exercise intolerance, lethargy and fatigue due to muscle disease which affect their daily life. Previous research suggests a need for qualitative exploration of self-regulation in BS and the inter-personal processes at play in family life. Therefore this study aimed to explore self-regulation and coping strategies and inter-personal responses in individuals and families affected by Barth syndrome. A multi-perspective qualitative study based on face to face, semi-structured, in-depth interviews with 11 participants (9-27 years, mean 15 years) with BS and/or their parents participating in a randomised double-blind clinical drug trial (CARDIOMAN). Interviews were transcribed verbatim and managed in NVivo prior to conducting a thematic analysis (AS and GH). RESULTS: Four key themes were identified: diagnosis and treatment, social support, identity and social integration, symptoms and self-regulation. The present findings suggest that self-regulation and coping in boys with BS was interpersonal and contingent on parental awareness such that parents were aware that their child had a limited energy reserve and that had to be managed due to the implications of fatigue for daily living. CONCLUSION: The findings support previous quantitative work demonstrating that children and parents tend to share a coherent view of BS. However, there is a need for greater awareness from others within the wider context of social and employment networks to minimise adverse implications for future life choices.

Treatment of Barth Syndrome by Cardiolipin Manipulation (CARDIOMAN) With Bezafibrate: Protocol for a Randomized Placebo-Controlled Pilot Trial Conducted in the Nationally Commissioned Barth Syndrome Service.

BACKGROUND: Barth syndrome is a rare, life-threatening, X-linked recessive genetic disease that predominantly affects young males and is caused by abnormal mitochondrial lipid metabolism. Currently, there is no definitive treatment for Barth syndrome other than interventions to ameliorate acute symptoms, such as heart failure, cardiac arrhythmias, neutropenia, and severe muscle fatigue. Previous mechanistic studies have identified the lipid-lowering drug bezafibrate as a promising potential treatment; however, to date, no human trials have been performed in this population. OBJECTIVE: The aim of this study is to determine whether bezafibrate (and resveratrol in vitro) will increase mitochondrial biogenesis and potentially modify the cellular ratio of monolysocardiolipin (MLCL) to tetralinoleoyl-cardiolipin (L4-CL), ameliorating the disease phenotype in those living with the disease. METHODS: The CARDIOMAN (Cardiolipin Manipulation) study is a UK single-center, double-blinded, randomized, placebo-controlled crossover study investigating the efficacy of bezafibrate in participants with Barth syndrome. Treatment was administered in two 15-week phases with a minimum washout period of 1 month between the phases where no treatment was administered. The primary outcome is peak oxygen consumption (VO2 peak). Secondary outcomes include MLCL/L4-CL ratio and CL profile in blood cells, amino acid expression, phosphocreatine to adenosine triphosphate ratio in cardiac muscle and skeletal muscle oxidative function on phosphorus-31 magnetic resonance spectroscopy, quality of life using the Pediatric Quality of Life Inventory questionnaire, absolute neutrophil count, cardiac function and rhythm profiles at rest and during exercise, and mitochondrial organization and function assessments. Outcomes were assessed at baseline and during the final week of each treatment phase. RESULTS: A total of 12 patients were scheduled to participate across three consecutive research clinics between March and April 2019. In total, 11 participants were recruited, and the follow-up was completed in January 2020. Data analysis is ongoing, with publication expected in 2021. CONCLUSIONS: This trial was approved by the United Kingdom National Research Ethics Service Committee and the Medicines and Healthcare products Regulatory Agency. The feasibility of the CARDIOMAN study will help to inform the future conduct of randomized controlled trials in rare disease populations as well as testing the efficacy of bezafibrate as a potential treatment for the disease and advancing the mechanistic understanding of Barth syndrome. TRIAL REGISTRATION: International Standard Randomized Controlled Trial Number (ISRCTN): 58006579; https://www.isrctn.com/ISRCTN58006579. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/22533.

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.

Asymmetric Regional Work Contributes to Right Ventricular Fibrosis, Inefficiency, and Dysfunction in Pulmonary Hypertension versus Regurgitation.

BACKGROUND: Right ventricular (RV) pressure loading from pulmonary hypertension (PH) and volume loading from pulmonary regurgitation (PR) lead to RV dysfunction, a critical determinant of clinical outcomes, but their impact on regional RV mechanics and fibrosis is poorly characterized. The aim of this study was to test the hypothesis that regional myocardial mechanics and efficiency in RV pressure and volume loading are associated with RV fibrosis and dysfunction. METHODS: Eight PH, six PR, and five sham-control rats were studied. The PH rat model was induced using Sugen5416, a vascular endothelial growth factor receptor 2 inhibitor, combined with chronic hypoxia. PR rats were established by surgical laceration of the pulmonary valve leaflets. Six (n = 4) or 9 (n = 4) weeks after Sugen5416 and hypoxia and 12 weeks after PR surgery, myocardial strain and RV pressure were measured and RV pressure-strain loops generated. We further studied RV regional mechanics in 11 patients with PH. Regional myocardial work was calculated as the pressure-strain loop area (mm Hg ∙ %). Regional myocardial work efficiency was quantified through wasted work (ratio of systolic lengthening to shortening work). The relation of regional myocardial work to RV fibrosis and dysfunction was analyzed. RESULTS: In rats, PH and PR induced similar RV dilatation, but fractional area change (%) was lower in PH than in PR. RV lateral wall work was asymmetrically higher in PH compared with sham, while septal work was similar to sham. In PR, lateral and septal work were symmetrically higher versus sham. Myocardial wasted work ratio was asymmetrically increased in the PH septum versus sham. Fibrosis in the RV lateral wall, but not septum, was higher in PH than PR. RV fibrosis burden was linearly related to regional work and to measures of RV systolic and diastolic function but not to wasted myocardial work ratio. Patients with PH demonstrated similar asymmetric and inefficient regional myocardial mechanics. CONCLUSIONS: Asymmetric RV work and increased wasted septal work in experimental PH are associated with RV fibrosis and dysfunction. Future investigation should examine whether assessment of asymmetric regional RV work and efficiency can predict clinical RV failure and influence patient management.