The role of endomyocardial biopsy in ARVC: looking beyond histology in search of new diagnostic markers.
The role of endomyocardial biopsy in ARVC: looking beyond histology in search of new diagnostic markers.
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DOI：
10.1111/j.1540-8167.2010.01960.x
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发表时间：
2011-01
影响因子：
2.7
通讯作者：
Saffitz JE
中科院分区：
文献类型：
--
作者：
Asimaki A;Saffitz JE
心律失常性右室心肌病（ARVC）的特点是室性心动过速和心源性猝死的高发，出现在疾病的自然史早期，可能先于显著的心室重构。ARVC的典型病理表现为右心室游离壁心肌变性并被脂肪和纤维组织取代。然而，临床表现可能是高度可变的，遗传外显率通常较低，这使得明确诊断困难，特别是在疾病的早期阶段。心肌内膜活检（EMB）尚未广泛应用于ARVC的诊断，部分原因是病理改变通常在右心室游离壁的心外膜最明显，而往往忽略心内膜和室间隔。因此，ARVC的诊断性病理特征在常规的鼻中隔活检中往往看不到。通过对右心室游离壁取样或使用三维电解剖电压映射来识别受影响的区域，可以提高诊断率，但这些方法可能会增加风险，并且需要专门的设备和经验。此外，尽管心律失常和猝死的风险增加，但诊断性病理改变在早期疾病中可能不明显。这篇综述考虑了EMB在ARVC诊断中的作用，并强调了在识别疾病过程早期出现并弥漫性发生在整个心肌的潜在组织生物标志物方面的最新进展。使用这些生物标记物分析传统EMB可以提高诊断的敏感性和准确性，但该方法的广泛临床应用需要进一步验证。
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by a high incidence of ventricular tachyarrhythmias and sudden cardiac death that appear early in the natural history of the disease and may precede significant ventricular remodeling. The classical pathology of ARVC is degeneration of right ventricular free wall myocardium and its replacement by fat and fibrous tissue. The clinical presentation may be highly variable, however, and genetic penetrance is typically low which makes definitive diagnosis difficult, especially in early stages of the disease. Endomyocardial biopsy (EMB) has not been used widely in the diagnosis of ARVC, in part because pathological changes are usually most conspicuous in the epicardium of the right ventricular free wall and tend to spare the endocardium and interventricular septum. Thus, diagnostic pathological features of ARVC are often not seen in conventional septal biopsies. Diagnostic yield may be increased by sampling the RV free wall or by using 3-dimensional electroanatomic voltage mapping to identify affected areas, but these approaches can carry increased risk and require specialized equipment and experience. Moreover, diagnostic pathological changes may not be apparent in early disease despite an increased risk of arrhythmias and sudden death. This review considers the role of EMB in the diagnosis of ARVC and highlights recent advances in identifying potential tissue biomarkers that arise early in the disease process and occur diffusely throughout the myocardium. Analysis of conventional EMB using such biomarkers could improve diagnostic sensitivity and accuracy but widespread clinical application of this approach requires further validation.
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影响因子：
37.8
作者：
Sen-Chowdhry, Srijita;Syrris, Petros;McKenna, William J.
通讯作者：
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DOI：
10.1016/s0735-1097(02)01946-0
发表时间：
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影响因子：
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作者：
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通讯作者：
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影响因子：
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作者：
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通讯作者：
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