Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia
Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia
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DOI：
10.1161/01.cir.0000108396.65446.21
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发表时间：
2003-12-16
期刊：
影响因子：
37.8
通讯作者：
Bouvagnet, P
中科院分区：
文献类型：
--
作者：
Tabib, A;Loire, R;Bouvagnet, P
背景-猝死是致心律失常性右室心肌病/发育不良(ARVC/D)的可能后果。然而，ARVC/D在意外心脏性猝死(USCD)中的患病率仍然不确定，死亡情况以及与ARVC/D相关的大体和显微表现，特别是希氏束异常。方法和结果-我们回顾了1980年1月至1999年1月司法当局要求的在2000名居民地区进行的14000例法医尸检。记录年龄、性别和死亡情况。对心脏进行了宏观和微观检查。ARVC/D组连续发生USCD 200例(10.4%)，其中男性108例，女性92例，平均年龄分别为32.5岁和34.5岁。近三分之一的死亡发生在生命的第四个十年期间。死亡情况多种多样，但75.6%发生在日常生活事件中(63.1%发生在家里，6.6%发生在街头，6.1%发生在工作场所)，仅7例发生在体育活动中(3.5%)。19例(9.5%)发生在围手术期。右室脂肪渗出是孤立的(20%)，或与纤维化(74.5%)和淋巴细胞(5.5%)相关。共有14.5%的病例有心肌肥厚，通过心脏重量和/或左室壁厚度的增加来评估。在大多数病例中，His束及其分支的异常要么是由于脂肪组织的渗透(8.1%)，要么是由于纤维化(54.3%)，或者两者兼而有之(5.6%)。死亡最常发生在久坐活动期间。他的异常和左心室肥厚可能与ARVC/D有关。
Background - Sudden death is a possible consequence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Prevalence of ARVC/D in unexpected sudden cardiac death (USCD), however, remains imprecise, as do circumstances of death and ARVC/D-associated gross and microscopic findings, especially His bundle anomalies.Methods and Results - We reviewed 14 000 forensic autopsies required by judicial authorities from January 1980 to January 1999 in a 2 000 000-resident area. Age, gender, and circumstances of death were recorded. Hearts were examined macroscopically and microscopically. In this series, the ARVC/D group accounted for 200 consecutive cases (10.4%) of USCD, including 108 males and 92 females ( average age 32.5 and 34.5 years, respectively). Nearly one third of deaths occurred during the fourth decade of life. Circumstances of death were various, but 75.6% occurred during everyday life events ( at home, 63.1%; in the street, 6.6%; or at work, 6.1%); only 7 cases (3.5%) occurred during sports activity. Nineteen cases (9.5%) happened during the perioperative period. Adipose infiltration of the right ventricle was either isolated (20%) or associated with fibrosis (74.5%) and lymphocytes (5.5%). A total of 14.5% of cases had cardiac hypertrophy, assessed by an increase in heart weight and/or left ventricular wall thickness. In most cases, the His bundle and its branches were abnormal either because of infiltration of adipose tissue (8.1%), fibrosis (54.3%), or both (5.6%).Conclusions - In ARVC/D, both sexes are equally affected, and there is a peak of risk during the fourth decade. Death most frequently occurs during sedentary activity. His abnormalities and left ventricular hypertrophy may be associated with ARVC/D.