Peters S, Davies M J, McKenna W J
University Hospital Magdeburg, Germany.
Jpn Heart J. 1996 Mar;37(2):195-202. doi: 10.1536/ihj.37.195.
The aim of the study was to classify the value of endomyocardial biopsies taken from the right ventricular septum in patients with ventricular tachyarrhythmias originating from the right ventricle. In a cohort of 62 young patients (mean age 35.6 years) 4-5 endomyocardial biopsies were taken from the right ventricular septum and myocardial atrophy, fibrosis and lipomatosis were analysed qualitatively and semi-quantitatively. Much interest was focused on the arrangement and type of fibrosis. Lipomatosis, myocardial atrophy and a finer form of fibrosis surrounding individual myocytes or groups of myocytes were judged as the typical histopathological findings of arrhythmogenic right ventricular dysplasia. The cohort of patients was subdivided clinically into idiopathic right ventricular outflow tract tachycardia (n = 50) and arrhythmogenic right ventricular dysplasia (n = 12). In the group of arrhythmogenic right ventricular dysplasia pathological findings were found in 92% with typical fibrolipomatosis in two cases, severe fibrosis ( > 40% per biopsy) in 5 cases, a finer form of fibrosis surrounding individual myocytes without lipomatosis in two cases and a slight interstitial or subendocardial fibrosis in two cases. In only one patient the endomyocardial biopsy was normal. In right ventricular outflow tract tachycardia normal endomyocardial biopsy findings predominated (80%). In two cases lipomatosis without fibrosis was a more or less normal finding, and in two other cases a slight interstitial or subendocardial fibrosis was found. Fibrosis was judged to be moderate or severe with discrete signs of inflammation in only two cases; in a long-term follow-up these two patients developed typical features of idiopathic dilated cardiomyopathy. Endomyocardial biopsies taken exclusively from the right ventricular septum were able to distinguish between right ventricular outflow tract tachycardia and arrhythmogenic right ventricular dysplasia, although the typical finding of fibrolipomatosis in cases with arrhythmogenic right ventricular dysplasia was rare. Characteristics of fibrosis are the key to the correct diagnosis provided that histopathological analysis is based on experienced qualitative criteria (type and arrangement of fibrosis). In conclusion, qualitative and semi-quantitative analyses of endomyocardial biopsies are far better than computerized quantitative methods. Biopsies from the free right ventricular wall cannot be generally recommended if a correct definition of arrhythmogenic right ventricular dysplasia in endomyocardial biopsies from the right ventricular septum is used.
本研究的目的是对右心室起源的室性心律失常患者取自右心室间隔的心内膜心肌活检的价值进行分类。在一组62例年轻患者（平均年龄35.6岁）中，从右心室间隔取4 - 5份心内膜心肌活检样本，对心肌萎缩、纤维化和脂肪浸润进行定性和半定量分析。人们对纤维化的排列和类型尤为关注。脂肪浸润、心肌萎缩以及围绕单个心肌细胞或成组心肌细胞的较细微形式的纤维化被判定为致心律失常性右心室发育不良的典型组织病理学表现。该组患者临床上分为特发性右心室流出道心动过速（n = 50）和致心律失常性右心室发育不良（n = 12）。在致心律失常性右心室发育不良组中，92%发现病理改变，其中2例有典型的纤维脂肪浸润，5例有严重纤维化（每份活检样本>40%），2例有围绕单个心肌细胞的较细微形式的纤维化但无脂肪浸润，2例有轻微的间质或心内膜下纤维化。仅1例患者的心内膜心肌活检正常。在右心室流出道心动过速组中，心内膜心肌活检正常结果占主导（80%）。2例无纤维化的脂肪浸润或多或少属于正常表现，另外2例发现有轻微的间质或心内膜下纤维化。仅2例纤维化被判定为中度或重度且有离散的炎症迹象；在长期随访中，这2例患者出现了特发性扩张型心肌病的典型特征。仅取自右心室间隔的心内膜心肌活检能够区分右心室流出道心动过速和致心律失常性右心室发育不良，尽管在致心律失常性右心室发育不良病例中典型的纤维脂肪浸润表现少见。只要组织病理学分析基于经验性的定性标准（纤维化的类型和排列），纤维化的特征就是正确诊断的关键。总之，心内膜心肌活检的定性和半定量分析远优于计算机化定量方法。如果使用右心室间隔的心内膜心肌活检对致心律失常性右心室发育不良进行正确定义，一般不推荐取自右心室游离壁的活检。
