Corrado D, Basso C, Thiene G
Department of Cardiology, University of Padua Medical School, Padua, Italy.
Cardiovasc Res. 2001 May;50(2):399-408. doi: 10.1016/s0008-6363(01)00254-1.
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.
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.
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.
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.
本研究旨在评估表面心脏正常的年轻人中心脏性猝死（SCD）潜在的、临床上无法识别的细微形态学基础的患病率。
在1979年至1998年期间，对意大利威尼托地区连续发生的273例年轻人（≤35岁）心脏性猝死病例进行了前瞻性研究。在排除心外性猝死原因后，按照详细的形态学方案对心脏进行检查，包括大体检查和组织学检查，其中组织学检查包括通过连续切片对特殊传导系统进行研究。
大体检查时，197例心脏性猝死受害者（72%）有明显的潜在结构性心脏病，如56例心肌病、54例阻塞性冠状动脉粥样硬化、32例瓣膜病、28例非动脉粥样硬化性冠状动脉疾病、13例主动脉破裂、5例术后先天性心脏病以及9例其他疾病。其余76例（28%）（50例男性和26例女性，年龄4至35岁，平均23±5岁）心脏大体正常。其中共有28例（37%）经历过以下一种或多种前驱症状：晕厥、心悸或两者皆有（20例）、心电图异常（18例）以及心律失常（10例）。在其中79%的病例中，组织学检查发现了隐匿性病理基础，包括27例局灶性心肌炎、9例区域性致心律失常性右室心肌病（主要局限于右室流出道）以及24例传导系统异常（18例导致心室预激，6例导致心脏传导阻滞）。16例心脏（6%）即使在组织学研究后也没有结构性心脏病的证据。
近三分之一的年轻心脏性猝死受害者心脏大体特征正常。然而，其中79%的病例通过组织学研究发现了隐匿性病理基础，如局灶性心肌炎或心肌病以及传导系统疾病。共有16例受害者（6%）没有结构性心脏病的证据，其心脏性猝死的机制仍无法解释。
