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 in young competitive athletes: clinicopathologic correlations in 22 cases

PURPOSE: To investigate the pathologic substrates of sudden death in young competitive athletes. PATIENTS AND METHODS: Twenty-two cases of sudden death in young competitive athletes occurring in the Veneto region (northern Italy) in the period January 1979 to December 1989 were studied by postmortem examination. The athletes included 19 males and three females, ranging in age from 11 to 35 years (mean, 23 years). RESULTS: In 18 cases, sudden death occurred during (16 cases) or immediately after (two cases) a competitive sport activity. In 10 subjects, sudden death was apparently the first sign of disease. Postmortem examination disclosed that this fatality was due to arrhythmic cardiac arrest in 17 cases; among these, right ventricular cardiomyopathy, also known as "right ventricular dysplasia," was the most frequently encountered cardiovascular disease (six cases), followed by atherosclerotic coronary artery disease (four cases), conduction system pathology (three cases), anomalous origin of right coronary artery from the wrong aortic sinus (two cases), and mitral valve prolapse (two cases). In two athletes, the abrupt lethal complication was "mechanical" and consisted of pulmonary embolism and rupture of the aorta; in three athletes, death was due to a cerebral cause. All athletes with right ventricular cardiomyopathy died during effort, and most had a history of palpitations and/or syncope. Whenever available, electrocardiographic (ECG) tracings showed inverted T waves in precordial leads and/or left bundle branch block ventricular arrhythmias. CONCLUSIONS: Clinicopathologic correlations indicate that in the Veneto region of Italy, right ventricular cardiomyopathy is not so rare among the cardiovascular diseases associated with the risk of arrhythmic cardiac arrest, and seems to account for the majority of cases of sudden death in young athletes; this disorder can be suspected during life on the basis of prodromal symptoms and ECG signs.     

Sudden unexpected death from cardiovascular disease in children. A cooperative international study

The results of an international cooperative study of sudden unexpected death from cardiovascular disease in children are reported. Sudden natural death was defined as death occurring instantaneously or within 24 hours of the onset of acute symptoms or signs in ambulatory, nonhospitalized children aged 1 to 21 years. Twenty institutions representing 10 countries contributed a total of 254 acceptable case reports. In 186 cases the child had had no cardiac surgery. Four heart conditions accounted for 53 percent of these “medical” deaths: congenital aortic stenosis (18 percent), Eisenmenger's syndrome (15 percent), cyanotic congenital heart disease with pulmonary stenosis or atresia (10 percent) and hypertrophic obstructive cardiomyopathy (9 percent). A total of 33 types of cardiovascular disease were reported. Sixty-eight chlldren died months or years after cardiac surgery. In 28 of these 68 patients death appeared to be causally related to the preceding surgery rather than to the underlying cardiovascular disease. All but 2 of the 28 probably died of an arrhythmia, usually without warning.The period between the onset of acute signs and symptoms (terminal episode) was less than an hour (usually instantaneous) in 73 percent of all 254 patients. In 95 percent of patients in the “medical” group heart disease was known or suspected, but a majority of these patients were asymptomatic. Warning syncope occurred in only 16 percent. A majority of the patients (58 percent) were inactive at the time of the terminal episode (Standing, sitting, lying or sleeping). Only 10 percent were engaged in active sports.This large study appears to establish better than previous data the relative frequency of sudden unexpected death among children with heart disease in Western Europe and Central and North America. Surgical and medical preventive measures, and the indications for engaging in sports, are discussed.     

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.     

Clinical characteristics of unexpected death among young enlisted military personnel: results of a three-decade retrospective surveillance

STUDY OBJECTIVE: To explore the causes of sudden and unexpected death in a young and healthy military population, to examine the various patterns of diseases associated with these tragic events, and to investigate the factors found to be associated with this grave outcome. DESIGN: We retrospectively investigated 151 cases of Israeli military personnel who died unexpectedly over a 30-year period. We collected all the available medical records, autopsy results, and investigation committee reports. RESULTS: A total of 151 cases of sudden and unexpected death occurred among enlisted military personnel during the period. Cardiac disorders caused 47% of deaths, followed by neurologic causes (17%) and pulmonary causes (11%). In 30 events (20%), the cause of death remained undetermined. Symptoms (eg, syncope, chest pain, palpitations, and others) occurred prior to death in 52 cases (34%). Asthma was the most common risk factor in our study population having been previously recognized in 10 cases (6.7%). Eight of the 13 subjects with asthma died following an acute asthmatic attack. CONCLUSION: Cardiac events are the leading cause of unexpected death in young healthy people. The frequency of subjects with asthma was found to be higher than that in the general age-adjusted population.     

Sudden death in the young

BACKGROUND: Sudden death is rare in young people, but it has a disproportionate impact on the community. OBJECTIVES: The purpose of this study was to determine the causes of sudden, natural death in persons aged 5-35 years. METHODS: All autopsies conducted at a forensic medicine facility in the years 1995-2004 (inclusive) were reviewed. This facility serves more than 2.5 million people in the eastern part of Sydney, Australia. Data collected included subject age, height, weight, gender, circumstances of death, and pathologic findings at autopsy. Deaths caused by trauma, accidental causes, drowning, and drug toxicity were excluded from analysis. RESULTS: There were 427 nontraumatic, sudden deaths in the 10-year period (70.7% male). Cardiac causes accounted for 56.4%, noncardiac causes 39.3%, and undetermined cause 4.3%. The most common cardiac cause of sudden death was presumed arrhythmia in those with no or minimal structural heart disease (29.0%). Other cardiac causes were acute myocardial infarction (24.5%), myocarditis (11.6%), hypertrophic cardiomyopathy (5.8%), aortic dissection (5.4%), and dilated cardiomyopathy (5.4%). More than two thirds of deaths caused by acute myocardial infarction occurred in the age group from 30-35 years. Sudden cardiac death occurred during physical activity in 10.8% of cases. Sudden cardiac death was reported in a first-degree relative in 4.5% of decedents. The most common noncardiac causes of sudden death were epilepsy (23.8%), intracerebral hemorrhage (23.8%), asthma (16.1%), and pulmonary embolism (12.5%). CONCLUSION: Presumed cardiac arrhythmia is the most common cause of sudden, natural death in the young. There was no reported history of sudden death among the relatives of most decedents.     

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.     

A survey of sudden death among school children in Osaka Prefecture

For 2 years, from January 1985 to December 1986, we carried out a thorough investigation of sudden death or apparent sudden death in a population between 5 and 19 years of age in Osaka Prefecture. Sudden death (unexpected death caused by intrinsic factors within 24 h after the onset of symptoms) occurred in 62 boys and 30 girls, with an annual sudden death rate of 3.0 per 100,000 for boys and 1.5 per 100,000 for girls. Of these 92 deaths, 64 occurred among school children (elementary or high school students), and it was these that were subjected to further analyses. Of the school children deaths, 29.7% seemed to be apparent sudden cardiac death (based on circumstances immediately before death, although there was no definite evidence for sudden cardiac death), and 29.7% were sudden death clearly attributable to underlying heart diseases (hence, sudden cardiac death in the usual meaning of this term accounted for about 60% of the 64 sudden deaths). In addition, 17.2% of the 64 cases were sudden death from bronchial asthma. Analysis of seasonal variation in the incidence of sudden death showed that apparent sudden cardiac death frequently occurred in summer, while sudden death clearly attributable to underlying cardiac diseases often took place in winter, sudden death from bronchial asthma occurred chiefly in summer and fall. Of the total number of sudden deaths, death occurred during exercise in 29.7%, and during sleep in 18.8% of individuals. The percentage of sudden deaths occurring during school hours was as low as 23.4% (15/64). Analysis of the physical condition of the 64 sudden death cases immediately before death disclosed the presence of respiratory infection in 10.9%, fatigue in 21.9%, lack of sleep in 4.7% and other conditions in 4.7%. Thus, the percentage of sudden deaths which occurred under ordinary physical conditions was 57.8% (37/64), of which 14 deaths (21.9% of the total) were associated with exercise.     

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.     

Non-atherosclerotic coronary artery disease and sudden death in the young.

OBJECTIVE--To assess prevalence and type of non-atherosclerotic coronary artery disease in young people (< or = 35 years) who died suddenly. DESIGN--A necropsy study of 150 consecutive cases of sudden death (that is, within 6 h of the onset of symptoms). RESULTS--Death was attributed to coronary artery disease in 48 cases: in 16 (33%) of them the disease was non-atherosclerotic. Twelve subjects (eight males and four females, age range 2-35 years, mean 24.2) had congenital anomalies: a deep intramyocardial course in six, origin from the wrong sinus in three, and ostial obstructions in three. Sudden death was the first manifestation of disease in six cases. The other six had a history of palpitation or syncope or both. An electrocardiogram was available in five cases and showed ventricular arrhythmias in four; none had angina pectoris. Stress testing was available in two cases: neither showed any effort-dependent ST-T abnormalities. In six cases sudden death was related to physical exercise. Acquired non-atherosclerotic coronary artery disease was found in four cases: spontaneous coronary dissection in three previously symptom free patients and Kawasaki coronary arteritis in one child who had had acute myocardial infarction. CONCLUSION--One third of the cases of fatal coronary artery disease were non-atherosclerotic with coronary artery anomalies being the most frequent form. Coronary artery anomalies should be suspected in young patients who have symptoms of ventricular arrhythmias without any overt signs and symptoms of ischaemia.     

Sudden death in the general population in Okinawa: incidence and causes of death

Sudden unexpected death is generally considered to be caused by acute myocardial infarction and/or arrhythmia. To document the incidence and causes of sudden death in Japan, where the incidence of myocardial infarction is low, the present study examined death certificates, hospital records, the forensic medical records, and the police records of residents of the southern part of Okinawa island who died at the age of 20-74 years during a 3-year period from January 1, 1992 to December 31, 1994. Sudden death was defined as death within 24 h from the onset of unexpected symptoms. The study documented 126 (87 men and 39 women) sudden deaths. The crude incidence rate was 0.37/1,000 person per year (0.51 in men and 0.23 in women). According to the death certificates, 78 cases died of heart diseases. However, the cause of death could be determined by examination of all available records in only 64 cases: myocardial infarction in 10, non-ischemic heart diseases in 13, and stroke in 23 cases. Even when the analysis was limited to the cases who died within 1 h from the onset of symptoms, heart disease was the cause of death in only 22% of the cases while the cause of death could not be determined in 53% of the cases. Only 13% of those diagnosed as heart diseases on the death certificate were verified. The agreement rate between the diagnosis reached by the re-evaluation of the records and that on the death certificate was 82% for stroke and 33% for other diseases. In Okinawa, Japan, the frequencies of heart disease and stroke as the cause of sudden death may be similar. Except for stroke, the diagnosis appearing on the death certificate has substantial inaccuracy.     

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.     

“Malignant” hypertrophic cardiomyopathy: Identification of a subgroup of families with unusually frequent premature death

Eight families were identified in which premature cardiac death due to hypertrophic cardiomyopathy occurred with unusual frequency. A total of 69 first degree relatives in the eight families were studied; 41 relatives had evidence of hypertrophic cardiomyopathy and 31 (75 percent) died of their heart disease. Eighteen of these 31 patients were less than 25 years of age at the time of death. Death was sudden and unexpected in 23 of the 31 patients; in 15 of these 23 patients sudden death was the initial manifestation of cardiac disease. The remaining eight patients (seven were from two families) died after a chronic cardiac illness characterized by congestive heart failure, atrial fibrillation or thromboembolic events.

Hence, premature cardiac death occurs frequently in certain families with hypertrophic cardiomyopathy. Such deaths are usually sudden, often occur in previously asymptomatic subjects and are common in children and young adults. These findings suggest that some families may manifest an unusually virulent expression of hypertrophic cardiomyopathy. Although this study cannot establish the precise prevalence with which “malignant” hypertrophic cardiomyopathy occurs, such families appear to be uncommon.     

QT interval shortening in families with history of sudden death at young age

AIM: To assess frequency of short QT interval among members of families with history of young age sudden death. MATERIAL: Eleven children aged 3-16 years (mean age 11.2+/-4.2 years) from families with cases of sudden death at young age (<40 years) not caused by an obvious disease according to autopsy data. None of the probands had overt heart disease, life threatening arrhythmias, long QT syndrome. METHODS: Predicted QT value was estimated according to the P. Rautaharju formula [QT(p)=656/(1+HR/100)], and values equal to 80 and 88% of QT(p) (QT(p)80 and QT(p)88) were determined. Corrected QT interval duration (QT(c)) was calculated for all these values with the use of the Bazett formula. RESULTS: Values of corrected QT(c)88 370 ms and 350 ms were considered minimal for heart rates above and below 78 bpm, respectively. QT shortening to the level of QT(p)80 at heart rates above 70 bpm corresponded to QT(c) values <330 ms. Nine of 11 children (81.1%) had QT interval equal to or less than QT(p)88. Three probands with attacks of syncope had QT equal to QT(p)80, high concentration of sudden deaths in family (up to 30% of family members), and high prevalence of shortened QT <80% among family members. CONCLUSION: Shortened QT interval is characteristic for children with family history of sudden death at young age. Registration of QT duration equal to or shorter than 88% of predicted value requires exclusion of diseases and syndromes with increased risk of sudden death.     

Sudden cardiac death in young people with apparently normal heart

OBJECTIVE: The aim of the present study was to assess the prevalence of subtle morphologic substrates, clinically unrecognizable, underlying sudden cardiac death (SCD) in young people with apparently normal heart. METHODS: In the time interval 1979--1998, 273 consecutive cases of SCD in young people (< or =35 years) which occurred in the Veneto Region of Italy were prospectively studied. Following exclusion of extracardiac causes of sudden death, the heart was examined according to a detailed morphologic protocol consisting of macroscopic and histologic examination, including study of the specialized conduction system by serial sections. RESULTS: At macroscopic examination, 197 SCD victims (72%) had an overt underlying structural heart disease such as cardiomyopathy in 56, obstructive coronary atherosclerosis in 54, valve disease in 32, non-atherosclerotic coronary artery disease in 28, aortic rupture in 13, postoperative congenital heart disease in five, and other disease in nine. The remaining 76 cases (28%) (50 males and 26 females, aged 4-35 years, mean 23+/-5 years) had a macroscopically normal heart. A total of 28 of them (37%) had experienced one or more of the following prodroma: syncope, palpitations or both in 20, ECG abnormalities in 18 and arrhythmias in ten. In 79% of them, histologic examination disclosed concealed pathologic substrates consisting of focal myocarditis in 27 cases, regional arrhythmogenic right ventricular cardiomyopathy, mostly localized to RV outflow tract, in nine, and conduction system abnormalities in 24 (leading to ventricular preexcitation in 18 and heart block in six). In 16 hearts (6%) there was no evidence of structural heart disease even after histologic study. CONCLUSION: Macroscopic heart features were normal in nearly one-third of young SCD victims. In 79% of them, however, histologic study unmasked concealed pathologic substrates such as focal myocarditis or cardiomyopathy and conduction system diseases. A total of 16 victims (6%) had no evidence of structural heart disease and the mechanism of their SCD remained unexplained.     

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.     

Sudden death in young athletes: HCM or ARVC?

Sudden non-traumatic death in young athletes is due to underlying congenital/inherited cardiac diseases in over 80% of cases. The two commonest conditions leading to sudden cardiac death in athletes below the age of 25 years are hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC).Hypertrophic cardiomyopathy is caused by mutations in genes, which code for sarcomeric contractile proteins. It can present with symptoms such as palpitation, presyncope or syncope. In a small number of cases, sudden death is the first clinical manifestation of the condition. It is well established that HCM accounts for over half of all cases sudden cardiac death in young individuals below 25 years of age. The management of HCM broadly encompasses symptom control, familial evaluation and the prevention of sudden death.Arrhythmogenic right ventricular cardiomyopathy, similarly, is a genetic disorder of the heart muscle and leads to symptoms such as palpitation and syncope and more rarely sudden death. The diagnosis of ARVC is most likely underestimated due to the lack of a single diagnostic test and subtle morphological changes in some cases. The diagnosis is based on clinical and family history and non-invasive investigations.The physiological adaptations seen in some athletes, as a response to physical training, may resemble phenotypically mild forms HCM and ARVC. Therefore, a diagnostic algorithm enabling this differentiation would be of importance especially bearing in mind the consequences of a misdiagnosis.     

Non-atherosclerotic coronary artery disease and sudden death in the young

Objective—To assess prevalence and type of non-atherosclerotic coronary artery disease in young people (≤35 years) who died suddenly.Design—A necropsy study of 150 consecutive cases of sudden death (that is, within 6 h of the onset of symptoms).Results—Death was attributed to coronary artery disease in 48 cases: in 16 (33%) of them the disease was non-atherosclerotic. Twelve subjects (eight males and four females, age range 2–35 years, mean 24·2) had congenital anomalies: a deep intramyocardial course in six, origin from the wrong sinus in three, and ostial obstructions in three. Sudden death was the first manifestation of disease in six cases. The other six had a history of palpitation or syncope or both. An electrocardiogram was available in five cases and showed ventricular arrhythmias in four; none had angina pectoris. Stress testing was available in two cases: neither showed any effort-dependent ST-T abnormalities. In six cases sudden death was related to physical exercise. Acquired non-atherosclerotic coronary artery disease was found in four cases: spontaneous coronary dissection in three previously symptom free patients and Kawasaki coronary arteritis in one child who had had acute myocardial infarction.Conclusion—One third of the cases of fatal coronary artery disease were non-atherosclerotic with coronary artery anomalies being the most frequent form. Coronary artery anomalies should be suspected in young patients who have symptoms of ventricular arrhythmias without any overt signs and symptoms of ischaemia.     

Maximum left ventricular thickness and risk of sudden death in patients with hypertrophic cardiomyopathy

OBJECTIVES: We sought to assess the relationship between maximum left ventricular (LV) wall thickness and outcome in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND: An association between maximum LV wall thickness and risk of sudden death was suggested in HCM. This finding requires further investigation, given the important implications for risk stratification and treatment. METHODS: We analyzed the mortality and risk profile of 237 patients (age 41 +/- 17 years; 63% male) classified into five groups based on echocardiographic maximum LV thickness. RESULTS: During follow-up (12 +/- 7 years), 36 patients died of cardiovascular causes, including 16 sudden deaths. Maximum LV thickness was not associated with a risk of sudden death (p = 0.37) nor with overall cardiovascular mortality (p = 0.7). With the exception of the small subset with thickness values < or =15 mm, with a consistently benign clinical course, the distribution of sudden death and overall cardiovascular mortality was not significantly different among the other four classes, ranging from 16 to 19 mm to > or =30 mm. Among 30 patients with extreme LV thickness (> or =30 mm), only one sudden event occurred among six patients diagnosed at <18 years of age (17%) and none among 24 diagnosed at > or =18 years of age. The prevalence of nonsustained ventricular tachycardia, syncope, an abnormal blood pressure response to exercise, and atrial fibrillation was similar among the five thickness classes. CONCLUSIONS: During 12-year follow-up, we observed no association between maximum LV thickness and cardiovascular mortality in a community-based population with HCM. The degree of maximum LV wall thickness should be considered in the context of a multifactorial approach to risk stratification, rather than as an isolated risk factor. Only in those patients diagnosed at a very young age might the presence of extreme LV wall thickness represent, per se, a potential marker of risk of sudden death.     

Precursors of sudden coronary death. Factors related to the incidence of sudden death.

Precursors of sudden death were sought in men--1838 civil servants in Albany, New York, and 2282 residents of Framingham, Massachusetts--under continuous surveillance for 16 years. In men 45-74 years old there were 234 deaths attributed to coronary heart disease (CHD) of which 109 occurred within one hour of onset of symptoms. More than half of all deaths due to CHD occurred outside the hospital and about 80 per cent of these were sudden. Most were unheralded by prior symptoms of CHD. Persons at high risk of death from CHD, including sudden death, can be identified long before the terminal unexpected catastrophe. The same precursive stigmata exist in persons subject ot coronary attacks whether or not immediately fatal. The risk of sudden death in these two populations was positively correlated with high blood pressure, the electrocardiographic pattern of left ventricular enlargement, obesity, and heavy cigarette usage. Sudden death is a common and possibly incidental expression of lethal coronary heart disease. The potential candidate for sudden death cannot be confidently distinguished from the individual who succumbs more slowly of myocardial infarction. The inescapable conclusion is that the prevention of sudden death requires the prevention of coronary attacks.