Risk of Sudden Death and Outcome in Patients With Hypertrophic Cardiomyopathy With Benign Presentation and Without Risk Factors (original) (raw)
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Sudden death in hypertrophic cardiomyopathy: identification of high risk patients
Journal of The American College of Cardiology, 2000
We sought to identify patients with hypertrophic cardiomyopathy (HCM) at high risk of sudden death (SD). BACKGROUND Relatively low mortality rates in HCM make conventional analysis of multiple clinical risk markers for SD problematic. This study used a referral center registry to investigate a smaller number of generally accepted noninvasive risk markers.
Circulation, 2000
Background-Death resulting from hypertrophic cardiomyopathy (HCM), particularly when sudden, has been reported to be largely confined to young persons. These data emanated from tertiary HCM centers with highly selected referral patterns skewed toward high-risk patients. Methods and Results-The present analysis was undertaken in an international population of 744 consecutively enrolled and largely unselected patients more representative of the overall HCM spectrum. HCM-related death occurred in 86 patients (12%) over 8Ϯ7 years (meanϮSD). Three distinctive modes of death were as follows: (1) sudden and unexpected (51%; age, 45Ϯ20 years); (2) progressive heart failure (36%; age, 56Ϯ19 years); and HCM-related stroke associated with atrial fibrillation (13%; age, 73Ϯ14 years). Sudden death was most common in young patients, whereas heart failure-and stroke-related deaths occurred more frequently in midlife and beyond. However, neither sudden nor heart failure-related death showed a statistically significant, disproportionate age distribution (Pϭ0.06 and 0.5, respectively). Stroke-related deaths did occur disproportionately in older patients (Pϭ0.002). Of the 45 patients who died suddenly, most (71%) had no or mild symptoms, and 7 (16%) participated in moderate to severe physical activities at the time of death. Conclusions-HCM-related cardiovascular death occurred suddenly, or as a result of heart failure or stroke, largely during different phases of life in a prospectively assembled, regionally based, and predominantly unselected patient cohort. Although most sudden deaths occurred in adolescents and young adults, such catastrophes were not confined to patients of these ages and extended to later phases of life. This revised clinical profile suggests that generally held epidemiological tenants for HCM have been influenced considerably by skewed reporting from highly selected populations. These data are likely to importantly affect risk stratification and treatment strategies importantly for the prevention of sudden death in HCM. (Circulation. 2000;102:858-864.)
Hellenic journal of cardiology : HJC = Hellēnikē kardiologikē epitheōrēsē
The identification of high-risk patients in hypertrophic cardiomyopathy (HCM) is still a challenge. The classical clinical risk factors for sudden death have been reported by studies coming from referral HCM cohorts. So far, other studies of community-based HCM populations have not managed to identify risk factors for sudden cardiac death. The aim of the present study was to determine the clinical course of the disease in a community-based HCM population, as well as to identify the clinical factors of sudden death in such a population. Three hundred four (304) consecutive HCM patients (202 males, age 48 ± 18.5 years) from 280 different families were assessed. Referral was based on disease diagnosis, irrespective of clinical status or treatment needs. All patients were examined clinically, echocardiographically, by 24h ambulatory electrocardiographic monitoring, and by cardiopulmonary exercise testing at regular intervals, for a period of 56.4 ± 29.9 months. Most patients (n=264/304,...
Hypertrophic cardiomyopathy and sudden cardiac death
World Journal of …, 2010
Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease that affects the left ventricle. HCM can appear at any age, with the majority of the patients remaining clinically stable. When patients complain of symptoms, these include: dyspnea, dizziness, syncope and angina. HCM can lead to sudden cardiac death (SCD), mainly due to ventricular tachyarrhythmia or ventricular tachycardia. High-risk patients benefit from implantable cardioverter-defibrillators. Left ventricular outflow tract obstruction is not a rare feature in HCM, especially in symptomatic patients, and procedures that abolish that obstruction provide positive and consistent results that can improve longterm survival. HCM is the most common cause of sudden death in young competitive athletes and preparticipation screening programs have to be implemented to avoid these tragic fatalities. The structure of these programs is a matter of large debate. Worldwide registries are necessary to identify the full extent of HCMrelated SCD.
Europace, 2010
We performed a systematic literature review of recommended 'major' and 'possible' clinical risk markers for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). We searched the Medline, Embase and Cochrane databases for articles published between 1971 and 2007. We included English language reports on HCM patients containing follow-up data on the endpoint (sudden) cardiac death using survival analysis. Analysis was
Arquivos brasileiros de cardiologia, 2006
objective: To evaluate, in patients with hypertrophic cardiomyopathy and risk for SCD who underwent implantable cardioverter-defibrillator (ICD) implantation: a-the occurrence of arrhythmic events; b-the occurrence of clinical events and their correlation with arrhythmic events; c-the occurrence of ICD shock therapy and clinical and functional correlations; dclinical and functional predictors of prognosis.
Prognosis in hypertrophic cardiomyopathy observed in a large clinic population
The American Journal of Cardiology, 1993
Overall annual cardiac mortality in hypertrophic cardiomyopathy (HC) has been reported to be be tween 2 and 4%, although these numbers are primarily from retrospective studies of patients rf+ ferred to large research institutions. A clinic population of 113 patients with HC was prospectively studied to assess cardiac mortality in the overall group and in selected subgroups commonly thought to be at high risk for sudden death. The mean age at diagnosis was 37 + 16 years. During follow-up, there were 11 cardiac and 2 noncardiac deaths. The annual cardiac mortality was 1% (95% confidence interval 0.2-1.6%). Because of the small number of deaths, relative risk for cardiac death was not significantly different in the presence of young age (130 years), family history of HC and sudden death, history of syncope or previous cardiac arrest, or both, ventricular tachycardia on 24hour Holter monitoring, or septal myotomy/ myectomy for refractory symptoms and oufflow tract obstruction. It is concluded that HC has a relatively benign prognosis (1% annual cardiac mop tality) that is 2 to 4 times less than that previously reported.