Sudden Arrhythmic Death Syndrome Research Articles

Sports-Related Sudden Cardiac Arrest in Germany

BackgroundKnowledge about causes of sports-related sudden cardiac arrest (SrSCA) may influence national strategies to prevent such events. Therefore, we established a prospective registry on SrSCA to estimate the incidence and in particular describe the etiologies of SrSCA in the general population in Germany. MethodsThe registration of SrSCA based upon 4 pillars: a web-based platform to record SrSCA cases in competitive and recreational athletes, media-monitoring, cooperation with the German Resuscitation Registry, and 15 institutes of forensic medicine. ResultsAfter an observation period of 6 years, a total of 349 cases was recorded (mean age 48.0 ± 12.7 years); 109 subjects survived. Most of the cases occurred during nonelite competitive or recreational sports. Bystander cardiopulmonary resuscitation (CPR) was initiated in 262 cases (75%); however, rhythm analysis and defibrillation (if indicated) was mainly performed by medical services. In patients ≤ 35 years of age, premature coronary artery disease (CAD) and sudden arrhythmic death syndrome (SADS) prevailed, followed by myocarditis. In athletes ≥ 35 years of age, CAD predominated. ConclusionsCountry-specific registries are necessary to define the national screening and prevention strategy optimally. In Germany, premature CAD, SADS, and myocarditis are the leading causes of SrSCA in young athletes, reinforcing the great disparity of the prevalence of cardiac diseases among different countries. Extension of on-site SCD-prevention campaigns, with training of CPR and explanation of the efficient use of automated external defibrillators (AEDs), may decrease the burden of SrSCD.

Sudden cardiac death in children and young adults without structural heart disease: a comprehensive review

Sudden cardiac death (SCD) is a rare clinical encounter in pediatrics, but its social impact is immense because of its unpredicted and catastrophic nature in previously healthy individuals. Unlike in adults where the primary cause of SCD is related to ischemic heart disease, the etiology is diverse in young SCD victims. Although certain structural heart diseases may be identified during autopsy in some SCD victims, autopsy-negative SCD is more common in pediatrics, which warrants the diagnosis of sudden arrhythmic death syndrome (SADS) based upon the assumption that the usual heart rhythm is abruptly replaced by lethal ventricular arrhythmia. Despite current advances in molecular genetics, the causes of more than half of SADS cases remain unanswered even after postmortem genetic testing. Moreover, the majority of these deaths occur at rest or during sleep even in the young. Recently, sudden unexpected death in epilepsy (SUDEP) has emerged as another etiology of SCD in children and adults, suggesting critical involvement of the central nervous system (CNS) in SCD. Primary cardiac disorders may not be solely responsible for SCD; abnormal CNS function may also contribute to the unexpected lethal event. In this review article, we provide an overview of the complex pathogenesis of SADS and its diverse clinical presentation in the young and postulate that SADS is, in part, induced by unfortunate miscommunication between the heart and CNS via the autonomic nervous system.

Editorial introductions

Editorial introductions

NOS1AP SNPs related to sudden arrhythmic death syndrome

Between 12–20 % of all Sudden Cardiac Death (SDC) occurs in individuals without previous symptoms of cardiac disease, which is known as Sudden Arrhythmic Death Syndrome (SADS). Recent studies identified the NOS1AP gene as new genetic marker to increase the risk of SDC. Here, we analyzed five SNPs of the NOS1AP gene by PCR-HRM in order to evaluate its ability as diagnostic and predictive biomarkers of the SADS.

4100Exercise related sudden cardiac death in the young in Sweden 2000–2010 – a nationwide study showing decreasing incidence in competitive athletes

Abstract Background Sudden cardiac death (SCD) is uncommon, but accounts for a majority of natural, unexpected sudden death in the young population. Exercise, although clearly health promoting for the vast majority of individuals, is also historically well known as a potential trigger of SCD. Previous studies have reported varying results concerning the comparative risk of SCD in competitive athletes versus non-athletes. Purpose The aim was to study all exercise related SCD in 10–35 years old in Sweden during the 11-year period 2000–2010, regarding the frequency, etiology, prevalence of symptoms, family history and/or ECG-changes; and to estimate the SCD incidence in the young and specifically in competitive athletes and to compare it with data from the previous decade. Methods We have previously identified all cases of SCD in 1–35 years old during 2000–2010 by systematic search in the Swedish Cause of Death Registry and the database of Forensic Medicine (n=552). From this cohort, we identified 514 cases in 10–35-years old, 373 (73%) men, and 141 (27%) women. For each case, information on circumstances at death, pathogenesis, athletic activity, medical history, symptoms, substance use, and heredity was assembled from autopsy-, police- and medical records. Results Exercise-related SCD occurred in 12% (62/514), a majority being men (56/62; 90%). Two thirds were recreational athletes, one-third competitive athletes. In total, 48% had either previous cardiac disease (27%), family history (27%), symptoms (32%), or an abnormal ECG (45%) before death. The most prevalent diagnosis was sudden arrhythmic death syndrome (SADS) (15/62; 24%). Exercise-related SCD was more frequent in men with hypertrophic cardiomyopathy (p=0.0008) and arrhythmogenic right ventricular cardiomyopathy (p=0.001), compared to non-exercise related SCD. The SCD incidence in competitive athletes was 9.4/million per year in men, and 0.6/million per year in women, which was around half the incidence compared to the years 1992–99. The SCD incidence in the general population was 22.3/million per year in men and 9.6/million per year in women. However, the risk of exercise-related SCD was twice as high in competitive athletes compared to recreational athletes. Conclusions 12% of SCD cases in the young Swedish population are exercise-related, with SADS being the most common etiology. The risk of exercise-related SCD is higher for competitive athletes compared to non-athletes. Specifically, exercise seems to trigger SCD in men with ARVC and HCM. About 50% have a pre-mortal risk profile. Importantly, the incidence of SCD in competitive athletes has been approximately halved in the 2000's compared to the 1990's. Theoretically, increased awareness, safety measures and cardiac screening may all have attributed to this positive development. We advocate a uniform evaluation of all exercise-related SCD in young athletes, including optimal histology and genetic analysis at autopsy. Acknowledgement/Funding Grants from Marcus Borgström, Uppsala University, the Swedish Medical Society, and from Norrbotten County Council

4250Sports-related sudden cardiac arrest in Germany

Abstract Background Knowledge about causes of sports-related sudden cardiac arrest (SrSCA) may influence national strategies to prevent such events. Purpose We established a prospective registry on SrSCA to estimate the incidence and in particular describe the etiologies of SrSCA in the general population in Germany. Methods The registration of SrSCA based upon 4 pillars: a) a web-based platform to record SrSCA cases in competitive and recreational athletes, b) media-monitoring and c) a cooperation with the German Resuscitation Registry as well as d) 15 institutes of forensic medicine. Results After an observation period of 6 years, a total of 349 cases was recorded (mean age 48.0±12.7 years) of which 109 subjects survived. Most of the cases occurred during non-elite competitive or recreational sports. Bystander cardiopulmonary resuscitation (CPR) was initiated in 262 cases (75%), however rhythm analysis and defibrillation (if indicated) was mainly performed by medical service. In subjects ≤35 years, premature coronary artery disease (CAD) and sudden arrhythmic death syndrome (SADS) prevailed, followed by myocarditis. In athletes ≥35 years, CAD predominated. Conclusion The prevalence of cardiac pathologies in young athletes seems to vary across different countries. CAD represents the most common cause of SrSCA in the general population of Germany, highlighting the need for a targeted cardiovascular risk evaluation including young athletes. Public education on basic life support including the appropriate use of an automated external defibrillator (AED) may further decrease the burden of sudden cardiac death. Acknowledgement/Funding Funding from the German Heart Foundation

Exercise related sudden cardiac death (SCD) in the young — Pre-mortal characterization of a Swedish nationwide cohort, showing a decline in SCD among athletes

AimsTo study the frequency, etiology, and premortal abnormalities in exercise-related sudden cardiac death (SCD) in the young in Sweden. MethodsAll subjects with SCD in 10–35-year olds in Sweden during 2000–10, were included (n = 514). Information about each case was retrieved from death certifications, autopsy- and medical records. The number of SCD in athletes was compared to national figures from 1992-99. ResultsExercise-related SCD occurred in 12% (62/514) of the SCD-population, a majority being men (56/62; 90%). Cardiopulmonary resuscitation (CPR) was started in 87% (54/62). In total, 48% (30/62), had a cardiac diagnosis, symptoms, family history and/or ECG-changes, before the fatal event. The most prevalent autopsy diagnosis was sudden arrhythmic death syndrome (15/62; 24%). The frequency of hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC) was significantly higher in exercise-related SCD compared to non-exertional SCD. Exercise-related SCD was more common in athletes (21/29) than in non-athletes (41/485) (P < 0.0001). The total number of SCDs/year in athletes 15–35 years old, are approximately halved in 2000-10 compared to the years 1992–99. ConclusionThe increased risk of exercise-related SCD in HCM and ARVC underlines the importance of early detection and eligibility recommendations. There is a major reduction in deaths among athletes in the 2000s, compared to the previous decade. These results may partly be explained by improved acute preparedness for sudden cardiac arrest (CPR, defibrillation), but as a substantial percentage have preceding risk factors, such as symptoms and ECG-abnormalities, increased cardiac screening and increased general awareness, may also play a role.

Sudden Cardiac Death: A Pediatrician’s Role

1. Benjamin H. Hammond, MD* 2. Kenneth G. Zahka, MD* 3. Peter F. Aziz, MD* 1. *Department of Pediatric Cardiology, Cleveland Clinic Children’s, Pediatric Institute, Cleveland Clinic Foundation, Cleveland, OH * Abbreviations: ACC: : American College of Cardiology AED: : automated external defibrillator ARVC: : arrhythmogenic right ventricular cardiomyopathy CPVT: : catecholaminergic polymorphic ventricular tachycardia ECG: : electrocardiogram HCM: : hypertrophic cardiomyopathy ICD: : implantable cardioverter-defibrillator LQTS: : long QT syndrome LVNC: : left ventricular noncompaction cardiomyopathy LVOT: : left ventricular outflow tract RV: : right ventricle SADS: : sudden arrhythmic death syndrome SCA: : sudden cardiac arrest SCD: : sudden cardiac death TdP: : torsade de pointes VA: : ventricular arrhythmia VT: : ventricular tachycardia 1. There is a broad differential diagnosis to be considered in cases of sudden cardiac arrest, sudden cardiac death. 2. Timely diagnosis can circumvent progression to cardiac arrest in at-risk individuals. 3. All children should be screened and testing should be reserved for those with increased risk. After completing this article, readers should be able to: 1. Assess risk of sudden cardiac arrest/sudden cardiac death (SCA/SCD) with a screening history and physical examination. 2. Identify the mechanisms of distinct etiologies of SCA/SCD. 3. Recognize findings consistent with risk of SCA/SCD on a 12-lead electrocardiogram. 4. Address the concerns and questions of individuals and families after SCA/SCD. Sudden cardiac death (SCD) or sudden cardiac arrest (SCA) may occur in populations known to be at high risk or in individuals previously unrecognized to harbor underlying disease. This review focuses on the pediatrician’s role in identifying the latter group. SCA is a “severe malfunction or cessation of the electrical and mechanical activity of the heart, resulting in almost instantaneous loss of consciousness and collapse” that precedes SCD. (1) SCD is defined as rapid, unexpected death from cardiac causes that occurs within 1 hour of symptoms. (2) SCD that is known to be secondary to a primary arrhythmia is termed sudden arrhythmic death syndrome (SADS) . SCA is a common cause of death in adults, with the annual incidence estimated to be approximately 100 per 100,000, with variation seen with age, sex, and race. (3) These deaths are predominantly associated with coronary artery disease. In individuals younger than 18 years, SCA is much less common, with an estimated annual incidence of out-of-hospital cardiac arrest of 8.3 per 100,000 person-years. (4 …

Whole genome and transcriptome sequencing of post-mortem cardiac tissues from sudden cardiac death victims identifies a gene regulatory variant in NEXN.

Sudden cardiac death (SCD) is a major public health problem and constitutes a diagnostic and preventive challenge in forensic pathology, especially for cases with structural normal hearts at autopsy, so-called sudden arrhythmic death syndrome (SADS). The identification of new genetic risk factors that predispose to SADS is important, because they may contribute to establish the diagnosis and increase the understanding of disease pathways underlying SADS. Pathogenic mutations in the protein coding regions of cardiac genes were found in relation to SADS. However, much remains unknown about variants in non-coding regions of the genome. In this study, we explored the potential of whole genome sequencing (WGS) and whole transcriptome sequencing (WTS) to find DNA variants in SCD victims with structural normal hearts. With focus on the non-coding regulatory regions, we re-examined a cohort of 13 SADS and sudden unexplained death in infancy (SUDI) victims without disease causing DNA variants in recognized cardiac genes. The genetic re-examination of DNA was carried out using frozen tissue samples and WTS was carried out using five distinct formalin fixed and paraffin embedded (FFPE) cardiac tissue samples from each individual, including anterior and posterior walls of the left ventricle, ventricular papillary muscle, septum, and the right ventricle. We identified 23 candidate variants in regulatory sequences of cardiac genes, including a variant in the promotor region of NEXN, c.-194A>G, that was found to be statistically significantly (p < 0.05) associated with decreased expression of NEXN and cardiac hypertrophy. With the use of post-mortem FFPE tissues, we highlight the potential of using WTS investigations and compare gene expression levels with DNA variation in regulatory non-coding regions of the genome for a better understanding of the genetics of cardiac diseases leading to SCD.

Next-generation sequencing using microfluidic PCR enrichment for molecular autopsy

BackgroundWe aimed to determine the mutation yield and clinical applicability of “molecular autopsy” following sudden arrhythmic death syndrome (SADS) by validating and utilizing low-cost high-throughput technologies: Fluidigm Access Array PCR-enrichment with Illumina HiSeq 2000 next generation sequencing (NGS).MethodsWe validated and optimized the NGS platform with a subset of 46 patients by comparison with Sanger sequencing of coding exons of major arrhythmia risk-genes (KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, RYR2). A combined large multi-ethnic international SADS cohort was sequenced utilizing the NGS platform to determine overall molecular yield; rare variants identified by NGS were subsequently reconfirmed by Sanger sequencing.ResultsThe NGS platform demonstrated 100% sensitivity for pathogenic variants as well as 87.20% sensitivity and 99.99% specificity for all substitutions (optimization subset, n = 46). The positive predictive value (PPV) for NGS for rare substitutions was 16.0% (27 confirmed rare variants of 169 positive NGS calls in 151 additional cases). The overall molecular yield in 197 multi-ethnic SADS cases (mean age 22.6 ± 14.4 years, 68% male) was 5.1% (95% confidence interval 2.0–8.1%), representing 10 cases carrying pathogenic or likely pathogenic risk-mutations.ConclusionsMolecular autopsy with Fluidigm Access Array and Illumina HiSeq NGS utilizing a selected panel of LQTS/BrS and CPVT risk-genes offers moderate diagnostic yield, albeit requiring confirmatory Sanger-sequencing of mutational variants.

Association between SCN5A and sudden unexplained nocturnal death syndrome in Thai decedents: a case\u2013control study

BackgroundSudden unexplained nocturnal death syndrome (SUNDS) is a genetic disorder that can cause sudden death in young and healthy adults during sleep at night. Cardiac arrhythmia has been proposed as the factor responsible for such deaths. This study was thus aimed to investigate the association between SCN5A, the gene that has been extensively studied and thought to be associated with cardiac conduction system abnormality causing sudden cardiac death, and SUNDS in Thai decedents.ResultsGenomic DNA was extracted using blood samples from 12 unrelated SUNDS subjects and 151 unrelated non-SUNDS control decedents. Subjects were divided into three subgroups as follows: subjects who resided in regions other than Northeastern Thailand (n = 99), age-matched subjects who resided in Northeastern Thailand (n = 28), and subjects older than 40 years who resided in Northeastern Thailand (n = 24). Genomic DNA from SUNDS cases was screened for genetic variations for the entire 28 coding exons of SCN5A. The identified variants were also genotyped in control subjects using high-resolution melting analysis and sequencing.ConclusionsTwelve variants of SCN5A were identified, including six polymorphisms and another six variants previously reported to be related with cardiac conduction defects. Two identified variants (rs1805126 and rs7429945) deserved further study because of their strong odd ratios.

Sudden cardiac death associated to substances of abuse and psychotropic drugs consumed by young people: A population study based on forensic autopsies

BackgroundToxic substances are one of the main risk factors for sudden cardiac death (SCD) in young people. However, there is limited information about this matter based on clinical research. The aim of this study was to analyze the use of substances of abuse (legal and illicit) and prescribed psychotropic drugs in young people who died by SCD. MethodsA population-based study performed in 15–35-year-olds who died by SCD in Biscay (Basque-Country) between 1991 and 2016. Cases were analyzed prospectively by a complete autopsy, toxicological and histopathological studies. A case was considered positive for exposure to cardiotoxic substances if smoking status was diagnosed or if toxicological analysis detected any drug associated with increased risk of SCD. ResultsThere were 204 SCD; 98 (48%) were exposed to a cardiotoxic substance, including smoking status (n = 72) and/or positive toxicology (n = 58). Illicit drugs (n = 29, mainly cannabis and cocaine), ethanol (n = 25), and prescribed psychotropic drugs (n = 11) were detected. Positive cases were more frequent in males than in females (54% vs. 19%). They were also more common in subjects who died by acute (86%) and chronic (71%) ischemic heart disease than in myocardial diseases (33%) and sudden arrhythmic death syndrome (36%). All positive cases of illicit drugs were males. Smoking status was very high in deaths due to acute ischemic heart disease. ConclusionsThe proportion of users of substances of abuse was unexpectedly high, even more prevalent than other cardiovascular risk factors. Toxic substances could play an important role as triggers of SCD in young people.

SCN5A gene exome sequencing profile in sudden unexplained nocturnal death syndrome in Thai population

SCN5A gene exome sequencing profile in sudden unexplained nocturnal death syndrome in Thai population

Blunted Nighttime Sympathetic Nervous System Response to Stress Among Thai Men with Positive Family History of Sudden Unexplained Nocturnal Death Syndrome.

Sudden unexplained nocturnal death syndrome (SUNDS) is prominent among northeast Thai men. This study tests the hypothesis that Thai men with positive family history of SUNDS display abnormal diurnal, autonomic nervous system responses to stress. Healthy northeast Thai men (20-49 years old) lived in the same rural area were divided into two groups based on their positive (PF) or negative family (NF1) history of SUNDS. A second control included Thai men with an NF history of SUNDS from a non-endemic area (NF2). All data were collected at 4:00-6:00 AM (nighttime) and 4:00-6:00 PM (daytime). All three groups displayed nighttime decreases in mean arterial pressure, heart rate, and blood glucose. Furthermore, all subjects displayed similar glucose tolerance and electrolyte balance. The tachycardic responses to a four-minute step test were similar among groups in the daytime, but the nighttime responses were significantly blunted in the PF group compared to either control group (about 20 bpm less). Tachycardic responses to a cold pressor test tended to decrease more during the nighttime in the PF compared to NF1 and NF2 groups, but the difference was not significant. Arterial pressure responses to the exercise were similar among the three groups during the nighttime, whereas in the NF2, daytime mean arterial pressures increased more than those in the other groups. The present data suggest that Thai men with a PF history of SUNDS display blunted sympathetic nervous system responses to stress during the nighttime, a potential factor that may trigger cardiac arrhythmias and contribute to SUNDS.

Sudden arrhythmia death syndrome in young victims: a five-year retrospective review and two-year prospective molecular autopsy study by next-generation sequencing and clinical evaluation of their first-degree relatives.

Sudden arrhythmia death syndrome (SADS) accounts for about 30% of causes of sudden cardiac death (SCD) in young people. In Hong Kong, there are scarce data on SADS and a lack of experience in molecular autopsy. We aimed to investigate the value of molecular autopsy techniques for detecting SADS in an East Asian population. This was a two-part study. First, we conducted a retrospective 5-year review of autopsies performed in public mortuaries on young SCD victims. Second, we conducted a prospective 2-year study combining conventional autopsy investigations, molecular autopsy, and cardiac evaluation of the first-degree relatives of SCD victims. A panel of 35 genes implicated in SADS was analysed by next-generation sequencing. There were 289 SCD victims included in the 5-year review. Coronary artery disease was the major cause of death (35%); 40% were structural heart diseases and 25% were unexplained. These unexplained cases could include SADS-related conditions. In the 2-year prospective study, 21 SCD victims were examined: 10% had arrhythmogenic right ventricular cardiomyopathy, 5% had hypertrophic cardiomyopathy, and 85% had negative autopsy. Genetic analysis showed 29% with positive heterozygous genetic variants; six variants were novel. One third of victims had history of syncope, and 14% had family history of SCD. More than half of the 11 first-degree relatives who underwent genetic testing carried related genetic variants, and 10% had SADS-related clinical features. This pilot feasibility study shows the value of incorporating cardiac evaluation of surviving relatives and next-generation sequencing molecular autopsy into conventional forensic investigations in diagnosing young SCD victims in East Asian populations. The interpretation of genetic variants in the context of SCD is complicated and we recommend its analysis and reporting by qualified pathologists.

Editorial introductions

Editorial introductions

HCN4 Gene Variations in Sudden Unexplained Nocturnal Death Syndrome in the Southern Han Chinese Population.

Sudden unexplained nocturnal death syndrome (SUNDS) is widely considered to be related to hereditary fatal arrhythmias. Hyperpolarization-activated cyclic nucleotide-gated channel 4 (HCN4) channels are widely distributed in sinus myocytes and play a profound role in generating pacemaker electro-activity in cardiomyocytes. In the present study, the potential correlation between HCN4 gene variations and the occurrence of SUNDS was investigated. Genomic DNA was extracted from blood samples of both 119 unrelated SUNDS patients and 184 healthy individuals and screened for candidate HCN4 gene variants. One missense heterozygous variant c.1578C>T (Ala195Val) and four synonymous heterozygous variants c.1552C>T, c.2833C>T, c.3823C>T, and c.4189C>A were discovered in the SUNDS cases. The missense variant c.1578C>T (Ala195Val) was absent in 163 recruited controls and 105 persons of the Southern Han Chinese population, had in-silico prediction indications as damaging, and was reported prevalent in sudden infant death, and is thus likely to be involved in SUNDS.

Insights into sudden cardiac death: exploring the potential relevance of non-diagnostic autopsy findings.

Unexplained sudden cardiac death (SCD) may be attributable to cardiogenetic disease. Presence or absence of autopsy anomalies detected following premature sudden death direct appropriate clinical evaluation of at-risk relatives towards inherited cardiomyopathies or primary arrhythmia syndromes, respectively. We investigated the relevance of non-diagnostic pathological abnormalities of indeterminate causality (uncertain) such as myocardial hypertrophy, fibrosis, or inflammatory infiltrates to SCD. At-risk relatives of unexplained SCD cases aged 1-64 years without prior cardiac disease (n = 98) with either normal and negative (40%, true sudden arrhythmic death syndrome; SADS) or isolated non-diagnostic (60%, uncertain sudden unexplained death; SUD) cardiac histological autopsy findings at a central forensic pathology unit were referred to the regional unexplained SCD clinic for clinical cardiac phenotyping. Uncertain SUD were older than true SADS cases (31.8 years vs. 21.1 years, P < 0.001). A cardiogenetic diagnosis was established in 24 families (24.5%) following investigation of 346 referred relatives. The proportions of uncertain SUD and true SADS explained by familial cardiogenetic diagnoses were similar (20% vs. 31%, P = 0.34, respectively), with primary arrhythmia syndromes predominating. Unexplained SCD cases were more likely than matched non-cardiac premature death controls to demonstrate at least one uncertain autopsy finding (P < 0.001). Primary arrhythmia syndromes predominate as familial cardiogenetic diagnoses amongst both uncertain SUD and true SADS cases. Non-diagnostic or uncertain histological findings associate with SUD, though cannot be attributed a causative status. At-risk relatives of uncertain SUD cases should be evaluated for phenotypic evidence of both ion channel disorders and cardiomyopathies.

Sudden Unexplained Nocturnal Death Syndrome: Epidemiological and Morphological Characteristics in Thai Autopsy Cases.

With limited knowledge on epidemiological and morphological characteristics of sudden unexplained nocturnal death syndrome (SUNDS), this study was aimed to identify such data in Thai SUNDS autopsy cases. All the cases were men and nondrug abusers aged 20-49years old. Most cases were originated in the Northeastern region of Thailand. Half of them were found dead from midnight to 6 a.m., with a peak time at 2 a.m. The death rate was relatively higher in May and June. Most SUNDS cases were blue-collar workers (93.2%) and nonsmokers (60%), with a normal BMI (72.1%). Approximately one-fifth of the cases had detectable blood alcohol concentrations. Symptoms before death were respiratory difficulty, seizures, and urinary incontinence. Their mean heart weight was 329.8±35.1g. Their lungs (88.6%) had some degree of congestion. Acute pancreatitis was not found in these SUNDS cases, and approximately half (40.9%) of the cases had their gastric content <100mL.

Seven years follow-up of early repolarisation patterns in French elite special forces.

The early repolarization pattern (ERP) may be a marker of increased risk for sudden cardiac death (SCD). Influence of ethnicity on the ERP has not been extensively studied. The aim of this study was to evaluate the epidemiology of ERP in a male multiethnic population. ECG analysis was performed among consecutive recruits from the French Foreign Legion. ERP was characterized by a J-point elevation ≥0.1mV in two continuous inferior-lateral leads, and high amplitude early repolarization (HAER-potentially malignant pattern) by an elevation ≥0.2mV. Ethnical affiliation and level of physical activity were recorded. A total of 2508 healthy men (24±5years old) from 105 different native countries were divided into three ethnic groups: 1689 Whites, 388 Afro-Caribbean, and 431 Asians. ERP was found in 489 recruits (19%), 14% in Whites, 33% in Afro-Caribbeans, and 27% in Asians without any difference according to age and physical activity. Sub-Saharan Africans or Caribbeans had the highest rate of ERP (30%), and Hispanics the lowest (8%). People from occidental countries, Middle East, Central Asia or India had a rate of 12%-18%, East and South-Asia 20%-25%. Madagascar was an exception with only 16% of ERP. HAER (2.9%) was more frequent among Asian recruits. After 5±2years of follow up, one SCD occurred in the ERP group (p=0.042). This study reports a large multiethnic analysis of ERP. HAER was more frequent in recruits from East and South-East Asia where sudden unexplained nocturnal death syndrome is endemic.