Sudden unexpected death in persons less than 40 years of age

This study retrospectively assesses the underlying causes of sudden unexpected death and the occurrence of prodromal symptoms in 162 subjects (aged 9 to 39 years) over a 10-year period (1976 to 1985). Underlying cardiac diseases accounted for sudden death in 73% and noncardiac causes in 15% of subjects. In 12% of subjects, the causes were unidentifiable. Myocarditis (22%), hypertrophic cardiomyopathy (22%) and conduction system abnormalities (13%) were the major causes in 32 subjects aged less than 20 years. Major causes of 46 deaths in subjects 20 to 29 years were atherosclerotic coronary artery disease (24%), myocarditis (22%) and hypertrophic cardiomyopathy (13%). The largest number of deaths in 84 subjects aged greater than or equal to 30 years was attributed to coronary artery disease (58%), followed by myocarditis (11%). Among noncardiac causes of sudden death, intracranial hemorrhage was the most frequent (5%), followed by infectious disease (4%). Prodromal symptoms were reported by 54% of subjects; most frequent were chest pain (25%) in subjects aged greater than or equal to 20 years, and dizziness (16%) in those aged less than 20. Sudden death, which occurred during routine daily activity in 49% and during sleep in 23% of subjects, was related to physical exercise in 23% and emotional upset in 6%. Sudden unexpected death in the young is still an unresolved medical problem. The early recognition of prodromal symptoms could be crucial in the prevention of sudden death, specifically when exercise-related.     

Sudden death of school children in Japan

The autopsy findings of sudden deaths of school children revealed that the main causes of death were latent myocarditis and arrhythmia, followed by idiopathic cardiomyopathy. The incidence of sudden death of school children in recent years is gradually increasing and 150 cases of sudden death occurred during the 1983 school year. A high incidence of sudden death in senior high school students was found. Sudden cardiac death accounted for approximately 80% of total deaths. The incidence of sudden cardiac death revealed prominent regional differences in Japan. The average for deaths was 0.40/100,000 children/5 years and the highest prefecture revealed 0.70 and the lowest 0.07. The cause of such differences is not clear and requires further analysis.     

Lessons learnt from the autopsies of 445 cases of sudden cardiac death in adults

AIM: To determine the cause of sudden cardiac death in adults who underwent autopsy. METHODS: Four hundred and forty-five sudden cardiac deaths occurred within 1 h of the symptoms onset, and all other cardiac and noncardiac causes having been excluded from autopsy and toxicology screening, were retrospectively identified from among 902 autopsies performed in a 2-year period on the island of Crete. The presence of acute coronary thrombi and myocardial infarction was documented macroscopically and by light microscopy and histology. RESULTS: In all 445 cases, at least one coronary artery had evidence of moderate to advanced atherosclerosis. About two thirds were between 50 and 70 years. Men had a higher incidence than women, but with advancing age (>60 years) this difference was reduced. Myocardial infarction was found in 17 cases (11 acute; 6 acute and healed). Fifty-eight cases (13.0%) had coronary thrombi, mostly involving the left anterior descending and the right coronary arteries (81%); only six of these were associated with acute myocardial infarction. CONCLUSION: In our population, arrhythmia was the most common cause of sudden cardiac death, while acute coronary thrombi and acute myocardial infarction were detected only in some cases. Because of the heterogeneity in the cause of sudden cardiac deaths in adults, a detailed forensic investigation may provide important information on the cause of death and help in the development of primary and secondary prevention.     

A Primer on Arrhythmias in Patients with Hypertrophic Cardiomyopathy

Patients with hypertrophic cardiomyopathy are at risk of atrial and ventricular arrhythmias, yet treatment options for these patients are made almost solely by extrapolation from patients with other diseases. Heart block may be seen spontaneously but is especially prevalent following septal reduction strategies. Atrial fibrillation is the most common arrhythmia in patients with hypertrophic cardiomyopathy. The onset of atrial fibrillation often represents a turning point clinically for patients, marked by substantial functional deterioration and morbidity. Sudden cardiac death is the most common cause of death in the young patient, but still contributes to mortality in older patients. Major risk factors for sudden cardiac death include resuscitated sudden cardiac death, marked hypertrophy, syncope, and family history of sudden cardiac death due to hypertrophic cardiomyopathy. Minor risk factors for sudden cardiac death include nonsustained ventricular tachycardia, and hypotensive response to exercise. Emerging possible risk factors include atrial fibrillation, myocardial ischemia, left ventricular outflow tract obstruction, genetic mutations, left ventricular apical aneurysms, myocardial fibrosis, and end stage disease.     

Causes of sudden death during sports activities in Spain

INTRODUCTION AND OBJECTIVES: Sudden death during sports activities has a profound impact on relatives, society, and athletes. Medical screening programs usually fail to prevent sudden death. We report the characteristics of a series of sudden deaths that occurred during sports in Spain. METHODS: We reviewed cases of sudden death that occurred during sports activities from 1995 to 2001 in the registries of the Institute of Toxicology of Madrid, Spain (Ministry of Justice). RESULTS: The series included 61 cases ranging in age from 11 to 65 years (average 31.9 14.2), 59 males and 2 females. The sports most frequently involved were cycling (21), football (13), and gymnastics (5). The causes of death were atheromatous coronary disease: 25 (40.9%) (23 over 30 years); arrhythmogenic cardiomyopathy: 10 (16.3%) (7 under 30 years); hypertrophic cardiomyopathy: 4 (6.5%); idiopathic left ventricular hypertrophy: 3 (4.9%); postmyocarditis myocardial fibrosis: 2 (3.2%); dilated cardiomyopathy: 1 (1.6%); congenital anomalies in the origin of the coronary arteries: 2 (3.2%); aortic valve disease: 2 (3.2%); and others: 2 (3.2%). In 10 cases (16.3%) (all under 30), the cause of death was undetermined. In 16 cases (26.2%) there was a known pathological antecedent. The disease responsible for death had been diagnosed in only three cases. CONCLUSIONS: Arrhythmogenic cardiomyopathy and severe left ventricular hypertrophy were the most common causes of sports-related death in persons under the age of 30. In 30% the cause of death was undetermined. Atheromatous coronary disease was prevalent over the age of 30 years and associated with cycling. Medical screening programs actually in use fail to detect a significant proportion of athletes at risk for sudden death.     

Sudden Unexpected Death in Children with Heart Disease

Objective. To review a mortality database, and identify all sudden unexpected deaths in patients followed by the cardiac program. Design. Retrospective review of prospectively maintained database. Results. Over 8 years, we identified 80 sudden unexpected deaths, among which there were sufficient data in 69 (24 females). Patients died at a median age of 17.2 months (28 days?18.8 years). Forty‐six patients had 2 functional ventricles and 23 had received palliation for a single‐functional ventricle. Patients with a single ventricle died at a younger age (median 120 days; 28 days?17.2 years) and sooner after last assessment (median 27 days; 1–146 days) than patients in the biventricular group (median age 2 years; 43 days?18.8 years; median time since last assessment 49 days, 1 days?1 year) (P < .01; P = .01). Thrombosis was the most common cause (61%) of death in the single‐ventricle group. Arrhythmia or presumed arrhythmia was the most common cause (46%) of death in the biventricular group. Fifty‐one patients had undergone surgery. Six patients had primary electrophysiological disease, and 5 had cardiomyopathy. Eight deaths occurred in patients with pulmonary vascular disease. Conclusion. Our study demonstrates that sudden unexpected death occurred at a frequency of at least 10 patients per year over an 8‐year period with 55 730 patient encounters. We were able to determine a clinical cause of death in most patients. Arrhythmias (30%) and pulmonary vascular disease (13%) are important causes of sudden death. Simple aortic valve disease and hypertrophic cardiomyopathy are rare (4%) causes of sudden death in childhood. Infants and young children with surgical shunts comprise 23% of sudden unexpected deaths that occur within a month of the last evaluation. Close surveillance of these patients is warranted.     

Risk factor profiles of patients with sudden cardiac death and death from other cardiac causes: a report from the Coronary Artery Surgery Study (CASS)

Identification of patients at risk of sudden death is essential if optimal preventive treatment strategies are to be developed. In the Coronary Artery Surgery Study (CASS) Registry, 19,946 patients were analyzed to characterize baseline clinical, hemodynamic and angiographic features of patients dying from sudden cardiac death and to compare them with features of patients dying from other cardiac causes, of those dying from noncardiac causes and of survivors. Of the 11,843 medically treated patients, 1,621 died during a mean follow-up period of 5.0 years: death was sudden in 557 (34%), nonsudden but cardiac in 813 (50%) and noncardiac in 251 (16%). In 8,103 surgically treated patients, 824 deaths occurred during a mean follow-up period of 5.1 years: death was sudden in 204 (25%), nonsudden but cardiac in 390 (47%) and noncardiac in 230 (28%). In general, the patients (both medically and surgically treated) who died of cardiac causes, either suddenly or nonsuddenly, were similar to each other but significantly different from patients who either survived or died of noncardiac causes. Although patients with an increased risk of any type of cardiac death could be identified, there were no measures of angiographic or hemodynamic characteristics that were significantly different between patients with sudden cardiac death and those with nonsudden cardiac death. Identification of patients at high risk for sudden cardiac death will require approaches in addition to clinical, angiographic and hemodynamic assessment, such as electrophysiologic assessment or monitoring techniques to identify triggering mechanisms.     

Sudden death in patients and relatives with the syndrome of right bundle branch block, ST segment elevation in the precordial leads V(1)to V(3)and sudden death.

BACKGROUND: The syndrome with an electrocardiographic pattern of right bundle branch block, ST segment elevation in leads V(1)to V(3)and sudden death is genetically determined and caused by mutations in the cardiac sodium channel. The inheritance of the disease is autosomal dominant. Sudden death may, however, occur from a variety of causes in relatives and patients with this syndrome. PATIENTS AND METHODS: Twenty-five Flemish families with this syndrome with a total of 334 members were studied. Affected members were recognized by means of a typical electrocardiogram either occurring spontaneously or after the intravenous administration of antiarrhythmic drugs. Sudden deaths in these families were classified as related or not to the syndrome by analysis of the data at the time of the event, mode of inheritance of the disease, and data provided by survivors.Results Of the 25 families with the syndrome, 18 were symptomatic (at least one sudden death related to the syndrome) and seven were asymptomatic (no sudden deaths related to the syndrome). In total, there were 42 sudden cardiac deaths (12% incidence). Twenty-four sudden deaths were related to the syndrome and all occurred in symptomatic families. Eighteen sudden deaths (43% of total sudden deaths) were not related to the syndrome (nine cases) or were of unclear cause (nine cases). Three of them occurred in two asymptomatic families and the remaining 15 in five symptomatic families. Twenty-four of the 50 affected members (47%) suffered (aborted) sudden death and 18 of the 284 unaffected members (6%). This difference in the incidence of sudden death was statistically significant (P<0.0001). Patients with (aborted) sudden death caused by the syndrome were younger than patients with sudden death of other or unclear causes (38+/-4 years vs 59+/-3 years respectively, P=0.0003). CONCLUSIONS: In families at high risk of sudden death because of genetically determined diseases, the main cause of sudden death remains the disease. However, almost the half of sudden deaths are caused by unrelated diseases or are of unclear cause. Accurate classification of the causes of sudden death is mandatory for appropriate analysis of the causes of death when designing preventive treatments.     

Timing of sudden death in patients with heart failure

Objectives. The purpose of this study was to determine the timing of sudden death in patients with advanced heart failure.Background. The frequency of sudden cardiac death and myocardial infarction is greatest in the morning hours, suggesting that physiologic processes associated with morning activities may trigger these events. In patients with advanced heart failure, a variety of mechanisms may cause sudden death, and the frequency of their occurrence may differ from that in other patient groups, perhaps altering the timing of sudden death in heart failure.Methods. Deaths among 566 consecutive patients followed up after treatment for advanced heart failure were prospectively categorized as sudden death, death due to heart failure or noncardiac death. For 72 sudden deaths the time of death was determined from witnesses to the event and from death certificates.Results. Sudden death occurred 2.5 times more frequently between 6:01 amand 12 noon than in the three other 6-h intervals, with 46% of deaths occurring during this period (p < 0.005). The morning peak occurred both in patients with coronary artery disease and in those with nonischemic causes of heart failure.Conclusions. Despite a variety of potential mechanisms of sudden death and underlying causes of heart disease in patients with heart failure, the 24-h distribution of sudden death in these patients is similar to that observed in other patient groups. Morning surges in sympathetic nervous system activity may promote a variety of sudden death mechanisms, including ischemic and nonischemic arrhythmias.     

Sudden death and obstructive hypertrophic cardiomyopathy

Sudden death is the most common cause of death in obstructive hypertrophic cardiomyopathies. Ventricular arrhythmias appear to be involved most frequently. The typical clinical picture is that of a young, asymptomatic male subject with a family history of sudden death and obstructive hypertrophic cardiomyopathy. The electrocardiogram at rest, the left ventricular pressure gradient, and the width of the septum are not good predictive criteria. Holter monitoring with the discovery of a run of ventricular tachycardia is the only method which identifies a subgroup at high risk for sudden death. Treatment with beta blockers does not protect patients with obstructive hypertrophic cardiomyopathy from sudden death and only combined therapy with an antiarrhythmic agent can control ventricular rhythm disturbances and decrease the number of sudden deaths in this condition.     

The role of molecular autopsy in unexplained sudden cardiac death

PURPOSE OF REVIEW: Sudden cardiac death (SCD) is one of the most common causes of death, with many attributable to cardiac/coronary abnormalities evident at autopsy. A significant number of SCDs, however, particularly in young people, remain unexplained following a medico-legal investigation, including autopsy, and are referred to as autopsy-negative sudden unexplained death (SUD). Due to molecular advances, however, a cardiac channel molecular autopsy may potentially provide a pathogenic basis for SUD and establish cause and manner of death. RECENT FINDINGS: Over the past decade, five population-based investigations of sudden death in young people elucidated the frequency of and causes responsible for these tragic events. The most inclusive epidemiologic study concluded that nearly 30% of SCDs in young people are autopsy-negative (i.e. SUD) and most likely secondary to cardiac channelopathies. Case reports on the post-mortem molecular diagnosis of cardiac channelopathies through the use of a molecular autopsy have been presented. Recently, a molecular autopsy series of SUD identified pathogenic mutations in long QT syndrome and catecholaminergic polymorphic ventricular tachycardia-associated genes in over one-third of cases. Similar post-mortem cardiac channel genetic testing in a large population-based cohort of sudden infant death syndrome has elucidated mutations in 5-10% of cases. SUMMARY: With autopsy-negative SUD accounting for a significant number of sudden deaths in young people, a new role for the medical examiner is emerging. An accurate diagnosis, derived from a molecular autopsy, will guide the appropriate initiation of pre-emptive strategies in hopes of preventing future tragedies among those left behind.     

Causes of sudden death in competitive athletes

Cardiovascular diseases responsible for sudden unexpected death in highly conditioned athletes are largely related to the age of the patient. In most young competitive athletes (less than 35 years of age) sudden death is due to congenital cardiovascular disease. Hypertrophic cardiomyopathy appears to be the most common cause of such deaths, accounting for about half of the sudden deaths in young athletes. Other cardiovascular abnormalities that appear to be less frequent but important causes of sudden death in young athletes include congenital coronary artery anomalies, ruptured aorta (due to cystic medial necrosis), idiopathic left ventricular hypertrophy and coronary artery atherosclerosis. Diseases that appear to be very uncommon causes of sudden death include myocarditis, mitral valve prolapse, aortic valve stenosis and sarcoidosis. Cardiovascular disease in young athletes is usually unsuspected during life, and most athletes who die suddenly have experienced no cardiac symptoms. In only about 25% of those competitive athletes who die suddenly is underlying cardiovascular disease detected or suspected before participation and rarely is the correct clinical diagnosis made. In contrast, in older athletes (greater than or equal to 35 years of age) sudden death is usually due to coronary artery disease, and rarely results from congenital heart disease.     

Sudden death associated with anomalous coronary origin and obstructive coronary disease in the young

Sudden cardiac death in a young patient is a catastrophic occurrence. Anomalous coronary origin (ACO) is a significant cause of sudden cardiac death among individuals under the age of 35 years. We sought to define the premortem clinical and postmortem histopathologic findings in victims of sudden cardiac death resulting from either ACO or obstructive atherosclerotic coronary artery disease (CAD) among U.S. military recruits (ages 17-35 years). The autopsy records of all sudden cardiac deaths occurring among recruits during their basic military training period from 1977 through 2001 were reviewed. Twenty-one deaths were associated with ACO and 10 with CAD. Recruits with ACO were more likely to have prodromal symptoms of exertional syncope and/or chest pain (48% vs. 0%, P = 0.011). All sudden cardiac deaths resulting from ACO involved a left main coronary artery takeoff from the right coronary sinus with a course between the aorta and the right ventricular outflow tract and an otherwise normal distribution of the major epicardial coronary arteries. Myocardial fibrosis was seen equally in those with both CAD and ACO (30% vs. 20%, P = 0.66), but the finding of necrosis tended to be more common among recruits with CAD (50% vs. 15%, P = 0.08). In conclusion, review of autopsy data of sudden cardiac deaths among U.S. military recruits reveals myocardial fibrosis or necrosis occurred in 70% of cases with CAD and 35% of cases with ACO. Sudden cardiac deaths resulting from ACO were more likely to be associated with premortem exertional chest discomfort and/or syncope compared with deaths resulting from CAD.     

心源性晕厥或猝死的原因分析

分析 32例在入院时或入院后至少发生 1次或 1次以上心源性晕厥或猝死患者的原因及其发作时与发作前、后的常规 12导联心电图或持续心电监视心电图。结果 :引起心源性晕厥或猝死的基本原因可分为以下几种类型 :①冠心病急性或陈旧性心肌梗死 ;②长QT(U)综合征 ;③Brugada综合征 ;④扩张型和肥厚型心肌病 ;⑤特发性巨大异常J波 ;⑥其他原因。上述各种心源性晕厥或猝死患者有各自不同的心电学特征。结论 :心源性晕厥或猝死是由不同原因、不同心电学特征所致的非单一独立的临床实体     

Sudden Cardiac Death in Dilated Cardiomyopathy – Therapeutic Options

BACKGROUND: Despite routine use of angiotensin-converting enzyme (ACE) inhibitors, beta-blockers and spironolactone in patients with heart failure due to dilated cardiomyopathy (DCM), these patients still have a considerable annual mortality rate of 5-10%. Sudden unexpected death accounts for up to 50% of all deaths and is most often due to rapid ventricular tachycardia or ventricular fibrillation and less often due to bradyarrhythmias or asystole. THERAPEUTIC OPTIONS: The use of beta-blockers in patients with heart failure has been shown to improve overall mortality considerably. This survival benefit has been demonstrated for bisoprolol, metoprolol and carvedilol. Therefore, one of these three beta-blocking agents should be administered routinely starting with low doses in all patients with New York Heart Association (NYHA) class II or III heart failure in addition to ACE inhibitors, unless there is a contraindication to beta-blocker use. In addition, NYHA class IV heart failure patients have been shown to benefit from carvedilol therapy, if tolerated. The conflicting results of GESICA and CHF-STAT studies do not support a strategy of "prophylactic" amiodarone therapy in patients with DCM in order to prevent sudden cardiac death. Despite growing evidence that implantable cardioverter defibrillator (ICD) therapy results in improved overall survival py preventing sudden cardiac death in patients at high risk for serious arrhythmic events, arrhythmia risk stratification with regard to prophylactic ICD implantation remains highly controversial in patients with DCM. CONCLUSION: This review describes potential arrhythmia mechanisms in DCM and summarizes the results of antiarrhythmic drug trials and of prophylactic ICD trials in patients with heart failure as well as our knowledge concerning arrhythmia risk stratification in patients with DCM.     

Hypertrophic cardiomyopathy: Management issues in the new millennium

Hypertrophic cardiomyopathy is an inherited cardiac disorder. Sudden cardiac death frequently occurs in otherwise healthy individuals, and accounts for nearly 35% of all sudden deaths within this age group. Although symptoms occur commonly, they often go unreported. Despite this, a degree of functional limitation is often seen on objective assessment. Management of hypertrophic cardiomyopathy is aimed at relieving symptoms, identifying and treating those individuals at increased risk of sudden death, and screening family members.     

Evaluation and outcome of patients with syncope

We studied 433 patients with syncope to derive insights into the diagnostic evaluation and outcome of patients with this common problem. This study shows that the etiology of syncope was not found in approximately 41% of patients. When a cause of syncope was determined, it was most frequently established on the basis of initial history, physical examination and an electrocardiogram (EKG). Furthermore, many of the other entities (e.g., aortic stenosis, subclavian steal) were suggested by findings on the history and physical examinations that required directed diagnostic testing. Initial EKG was abnormal in 50% of patients but led to a cause of syncope infrequently (less than 7%). Prolonged electrocardiographic monitoring, which has assumed a central role in the evaluation of syncope, led to a specific cause in only 22% of patients. Other tests were less often helpful in assigning a cause of syncope. At 5 years, the mortality of 50.5% in patients with a cardiac cause of syncope was significantly higher than the 30% mortality in patients with a noncardiac cause or 24.1% in patients with an unknown cause. At 5 years, a mortality of 50.5% in patients with a cardiac cause of syncope was noted. There were 54 actual deaths in this group as compared to 10.7 expected deaths based on 1980-86 mortality data from Allegheny County, PA (standardized mortality ratio = 5.02). At 5 years, a 33.1% incidence of sudden death was noted in patients with cardiac cause of syncope, as compared with 4.9% in patients with a noncardiac cause and 8.5% in patients with an unknown cause. Mortality and sudden death remained significant for the first 3 years after which the survival curves were parallel. A cardiac cause of syncope was an independent predictor of sudden death and mortality. Recurrences were common but were not associated with an increased risk of mortality or sudden death. Major vascular events were also more frequent in patients with cardiac causes of syncope. The results of this study will be helpful in designing future studies to evaluate the usefulness of newer diagnostic techniques. Furthermore, short- and long-term outcome data will be useful in planning intervention strategies in these patients.     

Sudden death and vigorous exercise--a study of 60 deaths associated with squash.

The circumstances surrounding 60 sudden deaths (59 men, one woman) associated with squash playing are described. The mean age (SD) of those who died was 46 (10.3) years (range 22-66 years). Necropsy reports were available in 51. The certified cause of death was coronary artery disease in 51 cases, valvar heart disease in four, cardiac arrhythmia in two cases, and hypertrophic cardiomyopathy in one case. There were only two deaths from non-cardiac causes. Forty five of those who died had reported prodromal symptoms, the most common of which was chest pain, and 22 were known to have had at least one medical condition related to the cardiovascular system during life, the most common of which was systemic hypertension (14 subjects). Those dying from coronary artery disease had a high frequency of risk factors. Some of these deaths might have been prevented by appropriate counselling of players after prospective medical screening, which would have detected most of the patients with overt cardiovascular disease and some of those with subclinical coronary artery disease.