Ethnicity and Sudden Cardiac Death in Athletes: Insights from a Large United Kingdom Registry.

BACKGROUND AND AIMS: The relationship between ethnicity and causes of sudden cardiac death (SCD) in athletes is poorly understood. OBJECTIVES: To investigate etiology of SCD among different ethnicities in a large cohort of athletes. METHODS: Between 1994 and November 2022, 7880 cases of SCD were consecutively referred from all over the United Kingdom to our national cardiac pathology centre; 848 (11%) were athletes. All cases underwent detailed autopsy evaluation by expert cardiac pathologists. Clinical information was obtained from referring coroners. RESULTS: Most of athletes were white (n = 758; 89%). Black and Asian athletes were 51 (6%) and 39 (5%) respectively. A structurally normal heart, indicative of sudden arrhythmic death syndrome (SADS) was the most common autopsy finding (n = 385, 45%), followed by myocardial diseases (n = 275; 32%) cases, atherosclerotic coronary artery disease (CAD) (n = 58, 7%) and coronary artery anomalies (n = 29, 3%). In most of cases, death occurred during exercise (n = 737; 87%) . Arrhythmogenic cardiomyopathy (ACM) was more common in black (n = 13; 25%) than in white (n = 109; 14%) and Asian (n = 3; 8%) athletes (p = 0.03 between black and white athletes; p = 0.04 between black and Asian athletes); in contrast, CAD was more common in Asians (n = 6; 15% vs n = 51; 7% in whites vs 2% n = 1; in blacks, p = 0.02 between Asian and black athletes). Among white athletes, ACM was more common in individuals who died during exercise than in the ones who died at rest (p = 0.005). Such a difference was not observed in Asian and black athletes. In Asian athletes, CAD was the diagnosis at autopsy in 18% of individuals who died during exercise and in none of individuals who died at rest. CONCLUSIONS: A structurally normal heart at autopsy and myocardial diseases are the most common findings in athletes who died suddenly. While ACM is more common in black athletes, atherosclerotic CAD is more common in Asian athletes, with a strong association with exercise-induced SCD. ACM appears to be a driver of exercise-induced SCD in white athletes, however this is not the case in black and Asian athletes.

A sudden death in an athlete is an uncommon but highly tragic event which appears almost paradoxical, as athletes epitomize the healthiest segment of society. Inherited and familiar cardiac conditions are the main causes of sudden death in young individuals and athletes. Studies on this matter have mainly focused on Caucasian athletes and the frequency or the causes of sudden death in athletes of other ethnicities is largely unknown. Our study focusses on this aspect and reveals that causes of sudden death may highly vary among athletes of different race. The circumstances of death differ significantly among various ethnicities, even looking at the same underlying cardiac condition.

Current Insights and Novel Cardiovascular Magnetic Resonance-Based Techniques in the Prognosis of Non-Ischemic Dilated Cardiomyopathy.

Cardiac magnetic resonance (CMR) imaging has an important emerging role in the evaluation and management of patients with cardiomyopathies, especially in patients with dilated cardiomyopathy (DCM). It allows a non-invasive characterization of myocardial tissue, thus assisting early diagnosis and precise phenotyping of the different cardiomyopathies, which is an essential step for early and individualized treatment of patients. Using imaging techniques such as late gadolinium enhancement (LGE), standard and advanced quantification as well as quantitative mapping parameters, CMR-based tissue characterization is useful in the differential diagnosis of DCM and risk stratification. The purpose of this article is to review the utility of CMR in the diagnosis and management of idiopathic DCM, as well as risk prediction and prognosis based on standard and emerging CMR contrast and non-contrast techniques. This is consistent with current evidence and guidance moving beyond traditional prognostic markers such as ejection fraction.

Coronary artery anomalies and the role of echocardiography in pre-participation screening of athletes: a practical guide.

Transthoracic echocardiography is an essential and widely available diagnostic tool for assessing individuals reporting cardiovascular symptoms, monitoring those with established cardiac conditions and for preparticipation screening of athletes. While its use is well-defined in hospital and clinic settings, echocardiography is increasingly being utilised in the community, including in the rapidly expanding sub-speciality of sports cardiology. There is, however, a knowledge and practical gap in the challenging area of the assessment of coronary artery anomalies, which is an important cause of sudden cardiac death, often in asymptomatic athletic individuals. To address this, we present a step-by-step guide to facilitate the recognition and assessment of anomalous coronary arteries using transthoracic echocardiography at the bedside; whilst recognising the importance of performing dedicated cross-sectional imaging, specifically coronary computed tomography (CTCA) where clinically indicated on a case-by-case basis. This guide is intended to be useful for echocardiographers and physicians in their routine clinical practice whilst recognising that echocardiography remains a highly skill-dependent technique that relies on expertise at the bedside.

Seasonal Variation in Sudden Cardiac Death: Insights from a Large United Kingdom Registry.

BACKGROUND: Sudden cardiac death (SCD) is relatively common and may occur in apparently healthy individuals. The role of seasonal variation as a risk factor for SCD is poorly understood. The aim of this study was to investigate whether SCD exhibits a predilection for specific seasons. METHODS: We reviewed a database of 4751 cases of SCD (mean age 38 ± 17 years) referred to our Center for Cardiac Pathology at St George's University of London between 2000 and 2018. Clinical information was obtained from referring coroners who were asked to complete a detailed questionnaire. All cases underwent macroscopic and histological evaluation of the heart, by expert cardiac pathologists. RESULTS: SCD was more common during winter (26%) and rarer during summer (24%), p = 0.161. Significant seasonal variation was not observed among cases of sudden arrhythmic death syndrome (SADS, 2910 cases) in which the heart is structurally normal. In contrast, a significant difference in seasonal distribution among decedents exhibiting cardiac structural abnormalities at the post-mortem examination (n = 1841) was observed. In this subgroup, SCDs occurred more frequently during winter (27 %) compared to summer (22%) (p = 0.007). In cases diagnosed with a myocardial disease (n = 1399), SCD was most common during the winter (27%) and least common during the summer (22%) (p = 0.027). CONCLUSIONS: While SADS occurs throughout the year with no seasonal variation, SCD due to structural heart disease appears to be more common during the winter. Bio-meteorological factors may be potential triggers of SCD in individuals with an underlying structural cardiac abnormality.

Yield of molecular autopsy in sudden cardiac death in athletes: data from a large registry in the UK.

AIMS: Sudden cardiac death (SCD) may occur in apparently healthy individuals, including athletes. The aim was to investigate the diagnostic role of post-mortem genetic testing, molecular autopsy (MA), in elucidating the cause of SCD in athletes. METHODS AND RESULTS: We reviewed a database of 6860 consecutive cases of SCD referred to our specialist cardiac pathology centre. All cases underwent detailed cardiac autopsy, and 748 were deemed to be athletes. Of these, 42 (6%) were investigated with MA (28 using a targeted sequencing, 14 exome sequencing). Variants were classified as pathogenic, likely pathogenic, or variant of unknown significance using international guidelines. Clinical information was obtained from referring coroners who completed a detailed health questionnaire. Out of the 42 decedents (average age 35 years old, 98% males) who were investigated with MA, the autopsy was in keeping with a structurally normal heart [sudden arrhythmic death syndrome (SADS)] in n = 33 (78%) cases, followed by arrhythmogenic cardiomyopathy (ACM) in eight (19%) individuals and idiopathic left ventricular fibrosis in one (2%). Death occurred during exercise and at rest in 26 (62%) and 16 (38%) individuals, respectively. Variants that were adjudicated clinically actionable were present in seven cases (17%). There was concordance between the genetic and phenotypic findings in two cases of ACM (in FLNC and TMEM43 genes). None of the variants identified in SADS cases were previously linked to channelopathies. Clinically actionable variants in cardiomyopathy-associated genes were found in five cases of SADS. CONCLUSION: The yield of MA in athletes who died suddenly is 17%. In SADS cases, clinically actionable variants were found in cardiomyopathy-associated genes and not in channelopathy-associated genes. Arrhythmogenic cardiomyopathy is a common cause of SCD in athletes, and one in four decedents with this condition had a clinically actionable variant in FLNC and TMEM43 genes.

Sudden Cardiac Death in Young Athletes: JACC State-of-the-Art Review.

Athletes epitomize the healthiest segment of society. Despite this premise, sudden cardiac death may occur in apparently healthy athletes, attracting significant attention not only in the medical community but also in laypersons and media. The incidence of sudden cardiac death is variably reported, and epidemiological burden differs among cohorts. Athletes appear to be at risk of developing fatal arrhythmias when harboring a quiescent cardiac disorder. Primary cardiomyopathies, ion channelopathies, and coronary artery anomalies are prevalent causes in young individuals. Cardiac assessment of athletes can be challenging because these individuals exhibit a plethora of electrical, structural, and functional physiological changes that overlap with cardiac pathology. A diagnosis of cardiac disease in a young athlete is not necessarily an indication to terminate competition and sports participation. International guidelines, traditionally focused on disqualification of individuals with cardiac disease, have recently adopted a more liberal attitude, based on a careful assessment of the risk and on a shared-decision making approach.

The Acute Impact of Endurance Exercise on Right Ventricular Structure and Function: A Systematic Review and Meta-analysis.

There have been many studies since the late 1980s investigating the effect of endurance exercise on the left ventricle. More recently, attention has shifted to the right heart, with suggestions that endurance exercise may have a detrimental effect on the right ventricle. This systematic review and meta-analysis summarizes and critiques 26 studies, including 649 athletes, examining the acute impact of endurance exercise on the right ventricle. We also present a subanalysis contrasting ultraendurance with endurance exercise. Finally, we identify areas for future research, such as the influence of sex, ethnicity, and age.

Aortic regurgitation in athletes: the challenges of echocardiographic interpretation.

BACKGROUND: Bicuspid aortic valve (BAV) is the most common congenital cardiac defect and prone to premature degeneration causing aortic regurgitation (AR). The assessment of AR in athletic individuals poses several challenges as the pathological left ventricle (LV) remodelling caused by AR may overlap with the physiological remodelling of intense exercise. The purpose of this study is to highlight these challenges, review the existing literature and discuss how to tackle these conundrums. As a real-world example, we compare the resting transthoracic echocardiographic (TTE) findings in a cohort of individuals with BAV and AR, sub-grouped into "highly active" or "lightly active". METHODS: Adult male subjects with an index TTE performed at a tertiary referral centre between 2019 and 2022 were included if the TTE confirmed a BAV and at least moderate AR. Further strict inclusion criteria were applied and parameters of valve disease severity was made in accordance with existing guidelines. Subjects completed a physical activity questionnaire over the telephone, and were classified into either group 1: "highly active" or group 2: "lightly active" based on their answers. Demographics and TTE parameters were compared between the two groups. RESULTS: 30 male subjects (mean age 44 ± 13 years) with BAV-AR were included - 17 were highly active, and 13 lightly active. There was no significant difference in age (group 1, 45 ± 12.7 years vs. group 2, 42 ± 17 years; p = 0.49), height (p = 0.45), weight (p = 0.268) or severity of AR, when quantitative assessment was possible. Group 1 had a significantly higher stroke volume (131 ± 17mls vs. 102 ± 13 mls; p = 0.027), larger LV volumes, diastolic dimensions and significantly larger bi-atrial and right ventricular size. This LV dilatation in the context of AR and athleticism poses a diagnostic and management conundrum. Despite this, none of these 17 highly active individuals demonstrated any of the traditional criteria used to consider surgery. CONCLUSION: There is significant overlap between the physiological adaptations to exercise and those caused by AR. Multi-modality imaging and stress testing can aid clinicians in diagnostic and management decisions in exercising individuals when there is discordance between AR severity and symptoms.

Comparison of echocardiographic methods for calculating left ventricular mass in elite rugby football league athletes and the impact on chamber geometry.

Background: Recommendations for the echocardiographic assessment of left ventricular (LV) mass in the athlete suggest the use of the linear method using a two-tiered classification system (2TC). The aims of this study were to compare the linear method and the area-length (A-L) method for LV mass in elite rugby football league (RFL) athletes and to establish how any differences impact the classification of LV geometry using 2TC and four-tier (4TC) classification systems. Methods: Two hundred and twenty (220) male RFL athletes aged 25 ± 5 (14-34 years) were recruited. All athletes underwent echocardiography and LV mass was calculated by the American Society of Echocardiography (ASE) corrected Linear equation (2D) and the A-L method. Left ventricular mass Index (LVMi) was used with relative wall thickness to determine geometry in the 2TC and with concentricity and LV end diastolic volume index for the 4TC. Method specific recommended cut-offs were utilised. Results: Higher values of absolute (197 ± 34 vs. 181 ± 34 g; p < 0.0001) and indexed (92 ± 13 vs. 85 ± 13 g/m2; p < 0.0001) measures of LV mass were obtained from A-L compared to the linear method. Normal LV geometry was demonstrated in 98.2% and 80% of athletes whilst eccentric hypertrophy in 1.4% and 19.5% for linear and A-L respectively. Both methods provided 0.5% as having concentric remodelling and 0% as having concentric hypertrophy. Allocation to the 4TC resulted in 97% and 80% with normal geometry, 0% and 8.6% with eccentric dilated hypertrophy, 0% and 7.7% with eccentric non-dilated hypertrophy, 1.4% and 0.5% with concentric remodelling and 1.4% and 3% with concentric non-dilated hypertrophy for linear and A-L methods respectively. No participants had concentric dilated hypertrophy from either methods. Conclusion: The linear and A-L method for calculation of LV mass in RFL athletes are not interchangeable with significantly higher values obtained using A-L method impacting on geometry classification. More athletes present with eccentric hypertrophy using 2TC and eccentric dilated/non-dilated using 4TC. Further studies should be aimed at establishing the association of A-L methods of LV mass and application of the 4TC to the multi-factorial demographics of the athlete.

Seasonal variation of cardiac structure and function in the elite rugby football league athlete.

BACKGROUND: Pre-participation cardiac screening (PCS) of "Super-League" rugby football league (RFL) athletes is mandatory but may be completed at any time point. The aim of this study was to assess cardiac electrical, structural and functional variation across the competitive season. METHODS: Elite, male, RFL athletes from a single Super-League club underwent cardiac evaluation using electrocardiography (ECG), 2D echocardiography and speckle tracking echocardiography (STE) at four time points across the RFL season; (1) End pre-season (ENDPRE), (2) mid-season (MIDCOMP), (3) end-season (ENDCOMP) and (4) End off-season (ENDOFF). Training loads for each time point were also determined. One-way ANOVA with post-hoc Bonferroni were used for statistical analyses. RESULTS: Total workload undertaken by athletes was lower at both MIDCOMP and ENDCOMP compared to ENDPRE (P < 0.001). ECG patterns were normal with training-related changes that were largely consistent across assessments. Structural data did not vary across assessment points. Standard functional data was not different across assessment points but apical rotation and twist were higher at ENDPRE (9.83˚ and 16.55˚, respectively compared to all other time points (MIDCOMP, 6.13˚ and 12.62˚; ENDCOMP, 5.84˚ and 12.12˚; ENDOFF 6.60˚ and 12.35˚). CONCLUSIONS: Despite some seasonal variation in training load, the athletes' ECG and cardiac structure were stable across a competitive season. Seasonal variation in left ventricular (LV) apical rotation and twist, associated with higher training loads, should be noted in the context of PCS.

BEAT-IT: A de-novo cardiac screening programme in Maltese adolescents.

AIMS: Sudden cardiac death (SCD) in young individuals is often unexpected, provoking substantial emotional stress for family and friends of the deceased. Cardiac screening may identify individuals who harbour disorders linked to SCD. The feasibility and diagnostic yield of a nationwide cardiac screening programme in adolescents has never been explored. METHODS: All individuals eligible for cardiac screening (students aged 15 years) were systematically invited to enrol. Students were provided with a health questionnaire. ECGs were acquired at school. A physician led consultation was carried out on site. Participants with an abnormal screen were then referred for secondary evaluation to the nation's tertiary centre. Feasibility criteria included a) participation rate >60%, b) adherence to secondary evaluation >80%, and c) cost per individual screened equating to <€100. The diagnostic yield was also evaluated. RESULTS: At the end of enrolment, 2708 students gave consent (mean 15 years, 50.4% male), equating to 67.9% of the eligible cohort. Overall, 109 participants (4.0%) were referred for further evaluation. An abnormal electrocardiogram (ECG) was the most common reason for referral (3.7%). Fifteen individuals (0.6%) were diagnosed with a cardiac condition. Nine (0.3%) had a condition linked to SCD (n = 1 Long-QT syndrome, n = 1 Hypertrophic Cardiomyopathy, n = 5 Wolff-Parkinson White, n = 2 coronary anomalies). The yield was similar in athletes and non-athletes (p = 0.324). The cost per cardiac individual screened equated to €51.15. CONCLUSION: A nationwide systematic cardiac screening programme for adolescent athletes and non-athletes is feasible and cost-efficient, provided that responsible centres have the appropriate infrastructure.

Ethnic and sex-related differences at presentation in apical hypertrophic cardiomyopathy: An observational cross-sectional study.

BACKGROUND: We investigated whether ethnicity and sex are associated with different clinical presentations and cardiovascular magnetic resonance (CMR) findings in individuals with apical hypertrophic cardiomyopathy (ApHCM). METHODS: A retrospective observational cohort study of consecutive ApHCM patients from a large tertiary referral center in the United Kingdom (UK). Demographic, clinical, 12­lead electrocardiogram (ECG) and CMR findings were collected. Participants presented in our clinics between 2010 and 2020. 'Pure' ApHCM was defined as isolated apical hypertrophy and 'mixed' with both apical and septal hypertrophy but with the apical segments of a greater wall thickness. Deep T-wave inversion was defined as ≥5 mm in any electrocardiogram lead. RESULTS: A total of 150 consecutive ApHCM patients (75% men, 25% women; 37% White, 25% Black, 24% Asian and 15% of Mixed/Other ethnicity) were included. Females were diagnosed at an older age compared to men, had less prominent ECG changes, had higher left atrial area index, and were more hypertensive. Black patients had higher left ventricular mass index, more hypertension, and more of the 'mixed' type of ApHCM. The majority of hypertensive male patients showed the 'mixed' phenotype. CONCLUSIONS: Individuals of Black ethnicity and hypertensive male patients are more likely to present with mixed apical and basal hypertrophy, whereas White, Asian and non-hypertensive male patients tend to have hypertrophy limited to the apex. Females present at an older age and are less likely to have deep T wave inversion on ECG.

Competency-based cardiac imaging for patient-centred care. A statement of the European Society of Cardiology (ESC). With the contribution of the European Association of Cardiovascular Imaging (EACVI), and the support of the Association of Cardiovascular Nursing & Allied Professions (ACNAP), the Association for Acute CardioVascular Care (ACVC), the European Association of Preventive Cardiology (EAPC), the European Association of Percutaneous Cardiovascular Interventions (EAPCI), the European Heart Rhythm Association (EHRA), and the Heart Failure Association (HFA) of the ESC.

Imaging plays an integral role in all aspects of managing heart disease and cardiac imaging is a core competency of cardiologists. The adequate delivery of cardiac imaging services requires expertise in both imaging methodology-with specific adaptations to imaging of the heart-as well as intricate knowledge of heart disease. The European Society of Cardiology (ESC) and the European Association of Cardiovascular Imaging have developed and implemented a successful education and certification programme for all cardiac imaging modalities. This programme equips cardiologists to provide high quality competency-based cardiac imaging services ensuring they are adequately trained and competent in the entire process of cardiac imaging, from the clinical indication via selecting the best imaging test to answer the clinical question, to image acquisition, analysis, interpretation, storage, repository, and results dissemination. This statement emphasizes the need for competency-based cardiac imaging delivery which is key to optimal, effective and efficient, patient care.

Competency-based cardiac imaging for patient-centred care. A statement of the European Society of Cardiology (ESC). With the contribution of the European Association of Cardiovascular Imaging (EACVI), and the support of the Association of Cardiovascular Nursing & Allied Professions (ACNAP), the Association for Acute CardioVascular Care (ACVC), the European Association of Preventive Cardiology (EAPC), the European Association of Percutaneous Cardiovascular Interventions (EAPCI), the European Heart Rhythm Association (EHRA), and the Heart Failure Association (HFA) of the ESC.

Imaging plays an integral role in all aspects of managing heart disease and cardiac imaging is a core competency of cardiologists. The adequate delivery of cardiac imaging services requires expertise in both imaging methodology-with specific adaptations to imaging of the heart-as well as intricate knowledge of heart disease. The European Society of Cardiology (ESC) and the European Association of Cardiovascular Imaging have developed and implemented a successful education and certification programme for all cardiac imaging modalities. This programme equips cardiologists to provide high quality competency-based cardiac imaging services ensuring they are adequately trained and competent in the entire process of cardiac imaging, from the clinical indication via selecting the best imaging test to answer the clinical question, to image acquisition, analysis, interpretation, storage, repository, and results dissemination. This statement emphasizes the need for competency-based cardiac imaging delivery which is key to optimal, effective and efficient, patient care.

The Lancet Commission to reduce the global burden of sudden cardiac death: a call for multidisciplinary action.

Despite major advancements in cardiovascular medicine, sudden cardiac death (SCD) continues to be an enormous medical and societal challenge, claiming millions of lives every year. Efforts to prevent SCD are hampered by imperfect risk prediction and inadequate solutions to specifically address arrhythmogenesis. Although resuscitation strategies have witnessed substantial evolution, there is a need to strengthen the organisation of community interventions and emergency medical systems across varied locations and health-care structures. With all the technological and medical advances of the 21st century, the fact that survival from sudden cardiac arrest (SCA) remains lower than 10% in most parts of the world is unacceptable. Recognising this urgent need, the Lancet Commission on SCD was constituted, bringing together 30 international experts in varied disciplines. Consistent progress in tackling SCD will require a completely revamped approach to SCD prevention, with wide-sweeping policy changes that will empower the development of both governmental and community-based programmes to maximise survival from SCA, and to comprehensively attend to survivors and decedents' families after the event. International collaborative efforts that maximally leverage and connect the expertise of various research organisations will need to be prioritised to properly address identified gaps. The Commission places substantial emphasis on the need to develop a multidisciplinary strategy that encompasses all aspects of SCD prevention and treatment. The Commission provides a critical assessment of the current scientific efforts in the field, and puts forth key recommendations to challenge, activate, and intensify efforts by both the scientific and global community with new directions, research, and innovation to reduce the burden of SCD worldwide.