RIGHT VENTRICULAR CARDIOMYOPATHY AND SUDDEN-DEATH IN YOUNG-PEOPLE
RIGHT VENTRICULAR CARDIOMYOPATHY AND SUDDEN-DEATH IN YOUNG-PEOPLE
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DOI：
10.1056/nejm198801213180301
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发表时间：
1988-01-21
影响因子：
158.5
通讯作者：
PENNELLI, N
中科院分区：
文献类型：
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作者：
THIENE, G;NAVA, A;PENNELLI, N
从1979年到1986年，我们对意大利东北部韦内托地区60名35岁以下突然死亡的人进行了尸检研究。出乎意料的是，我们发现12例受试者，男7例，女5例，年龄从13岁到30岁不等，具有右心室心肌病的形态学特征。在受试者死亡前，未诊断或怀疑该疾病。在5例病例中，猝死是疾病的第一个迹象;其余7例受试者有心悸、晕厥发作或两者兼而有之的病史，其中5例曾在心电图检查中记录到室性心律失常。其中10名受试者在用力时死亡。尸检时，受试者的心脏重量正常或中度增加。两种主要的组织学模式被确定。sbd。脂肪瘤转化或纤维脂肪瘤转化的右心室基本上是幸免。偶尔观察到心肌变性和坏死的体征，伴或不伴炎性浸润。这些发现表明，右心室心肌病，其原因仍然是未知的，可能比以前认为的更常见。至少在意大利的这个地区，它可能代表了年轻人猝死的一个重要原因。
From 1979 to 1986, we conducted postmortem studies of 60 persons under 35 years of age who had died suddenly in the Veneto Region of northeastern Italy. Unexpectedly, we found that 12 subjects.sbd.7 males and 5 females ranging in age from 13 to 30 years.sbd.had morphologic features of right ventricular cardiomyopathy. This disorder had not been diagnosed or suspected before the subjects died. In five cases, sudden death was the first sign of disease; the remaining seven subjects had a history of palpitations, syncopal episodes, or both, and in five of those seven, ventricular arrhythmias had previously been recorded on electrocardiographic examination. Ten of the subjects had died during exertion. At autopsy, the subjects'' heart weights were normal or moderately increased. Two main histologic patterns were identified.sbd.a lipomatous transformation or a fibrolipomatous transformation of the right ventricular was substantially spared. Signs of myocardial degeneration and necrosis, with or without inflammatory infiltrates, were occasionally observed. These findings indicate that right ventricular cardiomyopathy, the cause of which is still unknown, may be more frequent than previously thought. At least in this area of Italy, it may represent an important cause of sudden death among young people.