Death In Young Athletes Research Articles

LEFT VENTRICULAR HYPERTROPHY IN ELITE ADOLESCENT ATHLETES: GEORGIAN EXPERIENCE OF PRE-PARTICIPATION SCREENING

BackgroundAthlete's heart should be differentiated from the hypertrophic cardiomyopathy (HCM)-leading cause of exercise related sudden cardiac death in young athletes. Some genetic variations of HCM are characterized by a benign...

Sudden death in young athletes

Sudden and unexpected deaths during sport are rare, but often attract wide media coverage due to the deceased being young and healthy. They often have wide reaching effect not only on the families but also often on the sporting group where the death occurs. International studies have shown that sporting participants are three times more likely than the general population to suffer from undiagnosed cardiac conditions predisposing them to sudden death during sport. The actual rate of sudden death in sports is uncertain as the studies carried out predominantly in the USA and Europe are retrospective and the exact circumstances are not always known. Data also show that young indigenous people are more likely to die of coronary artery disease at an early age than non-indigenous people. It has been suggested in the literature that young indigenous people in sporting activities are at an increased risk of dying due to ischaemic heart disease rather than other cardiac anomalies. This presentation will present preliminary results of a study that aims to determine whether the cause of death in sporting activity in the young in Australia is similar to international data.

Anomalous Aortic Origin of a Coronary Artery: Toward a Standardized Approach

Anomalous aortic origin of a coronary artery (AAOCA) is a congenital abnormality of the origin or course of a coronary artery that arises from the aorta. It is the second most common cause of sudden cardiac death in young athletes. Its exact prevalence, the pathophysiological mechanisms that cause sudden cardiac death, the actual risk of death for the different types of AAOCA, the optimal way to evaluate these patients, and whether any treatment strategies decrease the risk of sudden cardiac death in patients diagnosed with AAOCA are unknown. This article analyzes what is currently known and unknown about this disease. It also describes the creation of a dedicated multidisciplinary coronary anomalies program and the development of a framework in an initial attempt to standardize the evaluation and management of these patients.

Sarcomere Neutralization Therapy in HCM/RCM: Therapeutic Application of the Calcium Desensitizer W7 to Remediate Hyper-Calcium Sensitive Disease States

The sarcomere is the functional unit of the heart. Alterations in sarcomere activation lead to disease states such as hypertrophic and restrictive cardiomyopathy (HCM/RCM). HCM is the most common monogenic cardiovascular disease, affecting 1:500 and is the leading cause of death in young athletes. Mutations in many of the sarcomeric genes are causal for HCM. In most cases, these mutations result in increased calcium sensitivity of the sarcomere giving rise to altered systolic and diastolic function. We show here that sarcomere neutralization therapy, with the calcium desensitizer W7, is a potential therapeutic strategy for HCM/RCM. W7 has previously been shown to decrease calcium sensitivity of the sarcomere through binding to cTnC and altering cTnI-cTnC interactions. Acute treatment of adult cardiac myocytes with W7 caused a dose-dependent (1-10 μM) decrease in contractility, independent of calcium transient changes. Alkalosis was used as an acute model of heightened calcium sensitivity, resulting in increased contractility and decreased baseline sarcomere length. Treatment with W7 in alkalosis remediated both phenotypes. R193H cTnI transgenic (Tg) myocytes were used as a model of RCM. R193H cTnI Tg myocytes display significant decreased baseline sarcomere length and slowed relaxation. Acute treatment with W7 dose-dependently increased baseline sarcomere length and remediated the slowed relaxation. Langendorff perfused whole heart pacing stress was used to study W7 effects at the organ level. R193H cTnI transgenic hearts had elevated end diastolic pressures at all stimulation frequencies compared to nontransgenic mice. Acute treatment with W7 restored normal end diastolic pressures throughout pacing in R193H cTnI transgenic mice. These results provide evidence that drug-based sarcomere neutralization therapy is a potential new approach to remediate hyper-calcium sensitive sarcomere function evident in acquired (respiratory alkalosis) and inherited (HCM/RCM) cardiac diseases.

An internship experience researching sudden death in young athletes and gaining exposure to clinical cardiology

/n summer 2012 I had the opportunity to death in athletes. In 1993, Dr. Maron brought the work as a research intern at the MinneapNational Registry of Sudden Death in Young olis Heart Institute Foundation (MHIF). Athletes to MHIF. The Registry is an autopsy MHIF is a nonprofit organization with the based database that records athlete deaths meeting mission of improving cardiovascular health five criteria: the athlete must have been related to through research and education. The internship the USA in some capacity, such as an American they offer is an effective extension of this athlete or a foreign athlete on American soil; a mission: future physicians (the interns) are victim of sports-related sudden death-, under the educated about healthcare through a collection age Qfforty, working towards improvement and of diverse experiences, and in return the interns excellence in their sport; and they must have died work with physicians to advance clinical in 1980 or later (Maron et al., 2009). The research projects. The internship program at database currently holds about 2300 cases of MHIF is primarily designed for pre-medical athlete deaths, and an additional 225 cases of students in the latter stages of undergraduate aborted death, which is defined as a sudden education (juniors, seniors, and graduated cardiac arrest that would have resulted in death if students). In existence since 2002, the program nQt for cardiopUlmonaiy resuscitation (CPR) or has three main purposes for participating automated external defibrillator (AED) use. Since interns: to gain experience with clinical rethere is no mandatory reporting of athlete deaths search, to learn more about cardiovascular h the USA, the Registiy is responsible for many healthcare, and to develop an accurate understatistics ()n ^ |ssue of sudden aftlete deaths and standing of medicine as a career (Willenbring et . r . , r , 1 20081 1S ore an imPortant resource for physicians, administrators, and public health officials. The most recent statistical analysis of the Clinical research Registry was published in 2009, and this analysis is still considered accurate (the per The most valuable part of the internship was centages do not change much over time). This gaining exposure to clinical research. I was analysis offers many useful figures conceming assigned to work under Dr. Bany J. Maron, who the demographics of sudden death in athletes, holds a special interest in researching sudden For example% the most recent years of the database suggest a sudden death incidence rate in athletes of 99 deaths per year (Maron et al., Correspondence to: cjmurphy2@students.nwc.edu 2009). The age of these athletes at death was

Allele-Specific Silencing of Mutant Myh6 Transcripts in Mice Suppresses Hypertrophic Cardiomyopathy

Dominant mutations in sarcomere proteins such as the myosin heavy chains (MHC) are the leading genetic causes of human hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy. We found that expression of the HCM-causing cardiac MHC gene (Myh6) R403Q mutation in mice can be selectively silenced by an RNA interference (RNAi) cassette delivered by an adeno-associated virus vector. RNAi-transduced MHC(403/+) mice developed neither hypertrophy nor myocardial fibrosis, the pathologic manifestations of HCM, for at least 6 months. Because inhibition of HCM was achieved by only a 25% reduction in the levels of the mutant transcripts, we suggest that the variable clinical phenotype in HCM patients reflects allele-specific expression and that partial silencing of mutant transcripts may have therapeutic benefit.

Sports preparticipation cardiac screening: what about children?

Sudden cardiac death in young athletes is a devastating event. Screening programs have been proposed to prevent sudden cardiac death in young athletes. Mortality rates and causes of death differ among young adults and children. Children have a considerably lower incidence of sudden cardiac death. Data lack to compare athletes and non-athletes in childhood, but 40-50 % of sudden cardiac death in this age group seems to be related to exercise. Screening programs including history and physical exam are not very sensitive or specific and will result in important numbers of false-positives and false-negatives. Especially, interpretation of ECG in children is different from ECG in adults, with less accurate diagnoses as a consequence. Secondary prevention by widespread education of simple resuscitation techniques and use of automatic external defibrillators if available will probably save as many lives as any screening program. Sufficient data are lacking to support general preparticipation screening with history, physical exam, and ECG in competitive children. Nevertheless, the impact of such a program, together with secondary preventive measures, should be evaluated in large prospective studies.

Active Surveillance of Sudden Cardiac Death in Young Athletes by Periodic Internet Searches

The authors hypothesized that prospective, systematic Internet searches could identify occurrences of sudden cardiac death (SCD) in athletes and would be useful for establishing a system of active surveillance. Weekly advanced Google searches of the Internet were conducted for cases of SCD in young athletes during a 12-month period (2007-2008). Athletes ages 11-30 years who collapsed during a game, practice, or within an hour of exercise were included in the study. Individuals with known histories of cardiac issues and events occurring outside the United States were excluded. Verification of SCD was by autopsy reports and death certificates from county coroner offices and vital record agencies. Initially, 71 events were identified. Verification for the cause of death by coroner reports was possible in 45 cases, 43 (96 %) of which were confirmed to be SCDs. A total of 69 individuals 11-30 years of age (mean 17 ± 5 years) died suddenly of cardiovascular causes while participating in 15 different organized sports and a variety of nonorganized physical activities. The most common cause of death was hypertrophic cardiomyopathy (30 %), followed by coronary artery anomalies (9 %), and myocarditis (9 %). The incidence of athlete SCD, the types of sports involved, and the cardiac causes of death in our study were comparable with those of previous reports. Readily available Internet searches have the potential to be a powerful tool for identifying occurrences of athlete SCD. An active surveillance system using Google searches followed by coroner report verification can provide important epidemiologic and clinical information.

Transgenic Mouse α- and β-Cardiac Myosins Containing the R403Q Mutation Show Isoform-dependent Transient Kinetic Differences

Familial hypertrophic cardiomyopathy (FHC) is a major cause of sudden cardiac death in young athletes. The discovery in 1990 that a point mutation at residue 403 (R403Q) in the β-myosin heavy chain (MHC) caused a severe form of FHC was the first of many demonstrations linking FHC to mutations in muscle proteins. A mouse model for FHC has been widely used to study the mechanochemical properties of mutated cardiac myosin, but mouse hearts express α-MHC, whereas the ventricles of larger mammals express predominantly β-MHC. To address the role of the isoform backbone on function, we generated a transgenic mouse in which the endogenous α-MHC was partially replaced with transgenically encoded β-MHC or α-MHC. A His6 tag was cloned at the N terminus, along with R403Q, to facilitate isolation of myosin subfragment 1 (S1). Stopped flow kinetics were used to measure the equilibrium constants and rates of nucleotide binding and release for the mouse S1 isoforms bound to actin. For the wild-type isoforms, we found that the affinity of MgADP for α-S1 (100 μM) is ~ 4-fold weaker than for β-S1 (25 μM). Correspondingly, the MgADP release rate for α-S1 (350 s(-1)) is ~3-fold greater than for β-S1 (120 s(-1)). Introducing the R403Q mutation caused only a minor reduction in kinetics for β-S1, but R403Q in α-S1 caused the ADP release rate to increase by 20% (430 s(-1)). These transient kinetic studies on mouse cardiac myosins provide strong evidence that the functional impact of an FHC mutation on myosin depends on the isoform backbone.

099 MYOCARDIAL CONTRAST ECHOCARDIOGRAPHY DISTINGUISHES PHYSIOLOGICAL FROM PATHOLOGICAL GREY-ZONE LEFT VENTRICULAR HYPERTROPHY

Background It remains difficult to distinguish an athlete9s heart (physiological left ventricular hypertrophy (LVH) from hypertrophic cardiomyopathy (HCM) (pathological LVH) especially when subjects fall into Maron9s grey zone ventricular wall thickness of 12–15 mm. Pathological LVH is one of the common causes of sudden death in young athletes. We hypothesised that pathological LVH due to HCM will have more fibrosis and reduced myocardial blood flow reserve (MBFR) compared to athletes. Capillary blood volume (CBV) which is reduced in fibrosis and MBFR can be assessed at the bedside by flash-replenishment myocardial contrast echocardiography (MCE). Methods 25 subjects with genetically proven septal HCM and 25 athletes all with grey zone septal hypertrophy (13.7±1.2 mm) were recruited. There were no significant differences in age (p=0.57, 33±8 years/32±8 years), gender(males-HCM:21, Atheletes:25) and degree of septal LVH (p=0.71, 14±0.89 vs 13.7±1.03) between HCM and athletes. All subjects underwent rest and stress vasodilator myocardial contrast echocardiography and quantitative analysis of CBV (dâ), blood velocity (dâ/s), myocardial blood flow (db/s 2 ) and MBFR(stress MBF/rest MBF) of the septum and apex was performed. Results Patients with HCM had significantly lower CBV (15.56±12.45 vs 18.30±12.6; p value=0.01) at rest and significantly lower resting MBF (14.10±15 vs 36.1±14.7; p=0.032) compared to athletes. MBFR was also significantly reduced compared to athletes (2.49 vs 5.67; p value=0.027). The receiver-operator characteristics(ROC) curve for CBV and MBFR demonstrated areas under the curve of 0.65 and 0.84 respectively. A cut-off of MBFR of 4.8 provides a sensitivity and specificity of 99% and 81% respectively for the detection of pathological LVH. The positive predictive value for predicting pathological LVH was 88% with a negative predictive value of 92%. Conclusions Quantitative MCE reliably distingisuishes physiological from pathological LVH in patients with grey zone hypertrophy who pose a diagnostic dilemma.

Differentiating Athlete's Heart from Inherited Cardiac Pathology: The Challenge of Repolarisation Abnormalities Presenting during Anaesthesia

This case report describes an asymptomatic healthy male professional athlete who underwent general anaesthesia for a routine orthopaedic operation. Peri-procedure, pronounced ST elevation suggestive of myocardial ischaemia manifested on the electrocardiogram lasting for four hours post-procedure, upon which the athlete developed deep and diffuse inferolateral T-wave inversion. These changes resolved spontaneously and the patient remained clinically stable throughout. This case demonstrates the clinical conundrum facing anaesthetists attempting to differentiate between repolarisation anomalies that are commonly observed in high-level athletes and those of inherited cardiac pathology, namely hypertrophic cardiomyopathy, which is the leading cause of sudden cardiac death in young athletes.

FUNCTIONAL ASSESSMENT BY STRESS EXERCISE IN PATIENTS WITH ANOMALOUS AORTIC ORIGIN OF A CORONARY ARTERY: EXPERIENCE OVER 17 YEARS

Anomalous aortic origin of coronary arteries (AAOCA) is the second most common cause of sudden cardiac death in young athletes and remains a challenging diagnosis with variable clinical and surgical approaches to management. We sought to review our 17-year institutional experience with functional

Sudden Cardiac Death in Young Athletes: Preparticipation Screening for Underlying Cardiovascular Abnormalities and Approaches to Prevention

The study of sudden cardiac death (SCD) in athletes has received more interest in the medical and lay press over the past few years. Professional athletes represent ideals of fitness and health, and the sudden death of prominent athletes can come as a shock. Underlying occult cardiovascular disorders are the most common cause of SCD in athletes. Unfortunately, because these disorders rarely present clinically, their initial manifestation is often a fatal event. Due to this, much attention has turned to both primary and secondary prevention. Primary prevention includes preparticipation screening and secondary prevention includes having automatic external defibrillators available at sporting events. This article summarizes the most common causes of athletic-related cardiac arrest and evaluates the screening methods used to screen for these conditions. The general sentiment is that we need to more effectively identify athletes who are at risk for SCD, but how to do so using an efficient screening system and in a cost-effective manner have not been determined.

Hjertescreening av unge idrettsutøvere

Young athletes are at an increased risk of sudden cardiac death compared to others. Cardiac screening has been proposed to prevent deaths. We wished to review the evidence for cardiac screening of young athletes. We have conducted a literature search in PubMed on sudden cardiac death in young athletes, using a combination of search terms related to screening, incidence, cost efficiency and recommendations, supplemented by secondary references and articles from our own archive. Published studies utilise a variety of definitions of athlete and sudden death, and some studies also include cardiac arrest with subsequent successful resuscitation. Retrospective studies, often based on media searches, remain the most common form. The cause of death is not invariably determined by an autopsy. Recommendations in favour of screening are based on studies of limited quality and on the personal, often regional, experiences of experts. The differences in study methods result in uncertain incidence figures. The estimates of cost efficiency are therefore questionable. To improve the quality of knowledge, standardised methods need to be devised, ideally also including a register of cardiac arrest in children and young people. To date, we have insufficient knowledge to recommend mandatory cardiac screening with ECG in Norway. Should this be introduced, it should be differentiated according to gender, type of sport and competition level. Cost efficiency could probably be improved with the aid of standardised questionnaires and a standardised interpretation of ECG among athletes.

Synthetic Peptides Model Instability of Cardiac Myosin Subfragment-2

Hypertrophic cardiomyopathy (HCM), a heart-related abnormality, is the most prevalent cause of sudden death in young athletes at sporting events. A cluster of cardiomyopathy mutations are localized in β-cardiac myosin at the N-terminal region of subfragment-2. Using resonance energy transfer probes, a synthetic peptide model system was developed to study stability of the coiled coil (S2 fragment) structure by determining monomer-dimer equilibrium of the peptide. Fluorescence resonance energy transfer and MacroModel software suite were used to obtain distance measurements along with measurement of coiled coil formation. The model peptide was used to characterize the effects of disease-causing-mutations and examine potential candidate drugs (polyamines) to counteract effects of mutations causing HCM. Distance measurements between donor and acceptor probes obtained by computational simulation and fluorescence resonance energy transfer (FRET) were consistent. Measurements also agreed with simulations of unlabeled wildtype, indicating coiled coil structural stability of the peptide. Interaction of the site-specific antibody with the peptide strongly inhibited dimerization and destabilized coiled coil structure of the peptide. Presence of negatively charged glutamate residues in the region of subfragment-2 strongly suggested a potential interaction site for positively charged polyamines. Binding of certain polyamines, such as poly-L-Lysine 11 residues and poly-D-Lysine 17 residues, demonstrated the ability to enhance dimerization and improve stability of the coiled coil structure, while some other polyamines were shown to have insignificant impact on the structure. In an attempt to characterize the effect of HCM-causing-mutations, peptides containing E924K mutation and lethal mutation E930 deletion were synthesized. Fluorescence resonance probes were conjugated to the mutant peptides to determine coiled coil formation. Results obtained from both dynamic simulations and resonance energy transfer experiments indicated that these mutations strongly inhibit dimerization, and thus, destabilize coiled coil structure of the peptide. Further experiments were conducted using heterodimers containing a chain of wildtype and a chain of mutant peptide. Both E924K & Edel930 mutations destabilized coiled coil formation and prevented dimerization. This peptide model system would provide a promising tool for drug development targeting HCM-causing-mutations along the S2 region of myosin.

Electrocardiographic Findings Suggestive of Cardiomyopathy

Cardiomyopathies are the leading cause of sudden cardiac death in young athletes. The electrocardiogram (ECG) is utilized as a first-line screening and diagnostic tool for detecting conditions associated with sudden death. Fundamental to the appropriate evaluation of athletes undergoing ECG is an understanding of what ECG findings are abnormal and may suggest the presence of a pathologic cardiac disorder. Multiple findings such as T-wave inversion, ST-segment depression, and pathologic Q waves are present in patients with cardiomyopathy. This article reviews the ECG findings in the four most common cardiomyopathies afflicting young athletes - hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, left ventricular noncompaction, and idiopathic dilated cardiomyopathy. ECG changes resulting from physiologic cardiac adaptation to regular exercise (athlete's heart) are presented also. This article provides a framework for distinguishing normal from abnormal ECG findings and outlines appropriate steps for further evaluation of ECG abnormalities in athletes suspected of having a cardiomyopathy.

Sudden cardiac death in young athletes

To review the recent literature and recommendations for cardiovascular screening in young athletes. The primary purpose of the preparticipation examination is to detect the cardiovascular disorders known to cause sudden cardiac arrest in the athlete. Studies demonstrate that the traditional history and physical-based examination has a limited sensitivity, does not detect the majority of athletes with at-risk conditions, and may provide false reassurance for athletes with disorders that remain undetected. Electrocardiogram (ECG) screening increases the sensitivity of the examination to detect disease, and cost modeling suggests protocols inclusive of ECG are the only screening strategies to be cost-effective. Proper ECG interpretation that distinguishes physiologic cardiac adaptations in athletes from findings suggestive of underlying cardiac pathology is essential to avoid high false-positive rates. The goal of cardiovascular screening is to maximize athlete safety. This includes the detection of underlying cardiac disease associated with sudden cardiac death and reduction of risk through both medical management and activity modification. Greater physician education and research are needed to improve the preparticipation examination in athletes.

Autonomic Response to Hypoxia and Isometric Exercise in Sickle Cell Trait Subjects

AbstractAbstract 3241Loss of cardiac beat to beat variability reflected by high frequency power (HFP) is a strong predictor of death in patients with cardiovascular disease. Similar autonomic dysfunction is found in patients with Sickle Cell Anemia (SCA) and can be induced by a 30 second period of hypoxia during five breath nitrogen exposure (1,2). Some data suggest that Sickle Cell Trait (SCT) carriers also exhibit autonomic dysfunction. In light of the recent attention to sudden death in young athletes with SCT we examined the autonomic response to transient hypoxia and isometric exercise in subjects with SCT and matched controls.Autonomic responses to hypoxia induced by a five-breath nitrogen exposure and intense isometric exercise resulting from handgrip to exhaustion were measured. Autonomic parasympathetic tone and sympathetic-vagal balance were measured using respiratory-adjusted time-varying heart rate variability (HRV). High frequency power (HFP) reflects parasympathetic tone and low-high ratio (LHR), sympathetic-vagal balance (1,2).Ten African American SCT carriers (7 female, 35.7 yrs + 2.8) and 11 age, and ethnicity matched controls (CTRL, 8 female, 30.5 yrs + 2.8) were studied. No difference in resting heart rate (HR, SCT 67.1 + 5.1, CTRL 65.9 + 5.5), cardiac output (CO, SCT 6.17 + .57, CTRL 7.31 + .52), mean arterial pressure (MAP, SCT 101.49 + 4.61, CTRL 101.49 + 4.96) or LHR (SCT, 1.16 + .38, SCT 1.44 + .37) was observed.Five-breath nitrogen exposure produced nearly identical minimal arterial saturation (SaO2, SCT 84.1% ± 1.3%, CTRL 83.1% ± 1.3%) with compensatory tachycardia (DRRI, SCT −6.5% ± 1.2%, CTRL −4.79 ± 1.15%) and increased cardiac output (DCO, SCT 4.7% ± 1.7, CTRL 6.0% ± 1.6%). No change from baseline was observed in the population means of HFP or LHR for CTRLs or SCT subjects.Following ten minutes of recovery, handgrip to exhaustion was performed at 60% maximum voluntary contraction. No differences were observed in maximum handgrip or duration between SCT and CTRL. Men produced higher maximal response but women maintained contraction nearly twice as long, independent of diagnosis. Handgrip exercise increased heart rate, cardiac output (SCT +13.4% ± 5.4%, CTRL +13.9% ± 5.0%), mean arterial pressure (SCT +19.6% ± 2.5%, CTRL +16.0% ± 2.3%), systolic pressure (SCT +14.6% ± 2.2%, CTRL +10.7% ± 2.1%), and diastolic pressure (SCT +22.5% ± 2.9%, CTRL +18.5% ± 2.6%). No population differences were observed in these responses. LHR increased similarly in both populations as well (SCT +78.9% ± 35.7%, CTRL +68.5% ± 32.9%), but SCT carriers had a much larger drop in HFP that was significantly different from baseline and trended lower than the control population (SCT −58% ± 18%, CTRL −9% ± 17%, p<.063).Our laboratory has previously demonstrated hyper-reflexic parasympathetic withdrawal in response to transient hypoxia in patients with SCA (1,2). SCT subjects did not demonstrate parasympathetic withdrawal to hypoxia. However, we found exaggerated parasympathetic withdrawal in response to handgrip exercise in SCT subject and not in matched controls. Even though the biophysical properties of the sickle trait red cell are not sufficient to result in major hemolysis or vaso-occlusion, they are sufficient to alter autonomic balance. It will be important to determine whether this response impacts the distribution of ventricular repolarization, potentially increasing risk of arrhythmia, or is reproduced in other autonomic axes such as mental stress. [Display omitted] [Display omitted] Disclosures:Coates:Apopharma: Consultancy; Novartis: Speakers Bureau. Wood:Novartis: Honoraria, Research Funding; Apotex: Consultancy, Honoraria; Shire: Consultancy; Ferrokin Biosciences: Consultancy.

Point/Mandatory ECG screening of young competitve athletes

e ( The death of a young (12–35 years old) and apparently healthy athlete generates considerable publicity through media headlines pertaining to the circumstantial paradox, the youth of the individual, and the loss of potentially several quality years of life. Apart from the devastating impact on the family, the societal ramifications of these tragedies cannot be underestimated. Most nontraumatic deaths in young athletes are attributable to hereditary and congenital structural or electrical cardiac disorders that usually run a quiescent natural history and favorable prognosis in sedentary individuals. Most disorders can be diagnosed during life, and several therapeutic strategies combined with lifestyle modification are in existence to minimize the risk of death. Hypertrophic cardiomyopathy (HCM) is the commonest cause of sudden cardiac death (SCD) in young athletes worldwide, whereas arrhythogenic right ventricular cardiomyopathy is the leading cause f death in Italy. The physical and metabolic stresses assoiated with exercise serve as triggers for fatal arrhythmias in redisposed individuals, with arrhythmogenic SCD being the rst manifestation in 70%–80% of the victims. Statistics compiled from large reliable systematic regisries indicate a SCD incidence rate of approximately 1 in 0,000. At least 100 young athletes suffer SCD annually in the United States; most lose at least 50 years of normal life expectancy. Whereas a figure of 100 appears trivial compared with 300,000 SCDs in the entire population, this equates to 5000 life-years, making the issue of SCD in athletes an important public health concern.

Counterpoint: Mandatory ECG screening of young competitive athletes

Sudden death (SD) in young sports participants remains a visible and riveting issue that continues to command attention in the general public and medical community. 1 Maron B.J. Sudden death in young athletes. N Engl J Med. 2003; 349: 1064-1075 Crossref PubMed Scopus (1127) Google Scholar While there is a general agreement that primary prevention preparticipation screening for cardiovascular disease in apparently healthy young populations is justified and desirable, determining the most effective and practical strategy has become an intense public health and even societal debate. 2 Corrado D. Pelliccia A. Bjørnstad H.H. et al. Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Eur Heart J. 2005; 26: 516-524 Crossref PubMed Scopus (1010) Google Scholar , 3 Maron B.J. Thompson P.D. Ackerman M.J. et al. Recommendations and considerations related to preparticipation screening for cardiovascular abnormalities in competitive athletes: update 2007. Circulation. 2007; 115: 1643-1655 Crossref PubMed Scopus (847) Google Scholar Based on controversial initiatives in Italy, considerable interest has focused on screening competitive athletes with 12-lead electrocardiograms (ECGs). 4 Corrado D. Basso C. Pavei A. Michieli P. Schiavon M. Thiene G. Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. JAMA. 2006; 296: 1593-1601 Crossref PubMed Scopus (1180) Google Scholar However, such proposals are deceptively complex and have generated misunderstanding.