Lücke Christian, Gutberlet Matthias
Ltd. OA MRT Radiologie, Abteilung für Diagnostische und Interventionelle Radiologie, Herzzentrum Leipzig, Strümpellstr. 39, 04289, Leipzig, Deutschland.
Leiter Abteilung für Diagnostische und Interventionelle Radiologie, Professur für Kardiologische Bildgebung in der Radiologie der Universität Leipzig, Herzzentrum Leipzig, Strümpellstr. 39, 04289, Leipzig, Deutschland.
Radiologie (Heidelb). 2022 Nov;62(11):920-932. doi: 10.1007/s00117-022-01068-6. Epub 2022 Sep 21.
The classification of cardiomyopathies used in Germany goes back to the European Society of Cardiology (ESC) classification of 2008. The cardiomyopathies are subdivided according to the phenotype, so that magnetic resonance imaging (MRI) is able to differentiate between the various cardiomyopathies.
The strength of MRI is the ability to differentiate nonischemic cardiomyopathies from other diseases with similar morphofunctional aspects, based on the possibilities of tissue differentiation. In the case of dilated cardiomyopathy (DCM), for example, a differentiation from inflammatory DCM is possible. In the case of hypertrophic cardiomyopathy (HCM), obstructive and nonobstructive forms can be differentiated analogously to the echo but amyloidosis or Fabry disease can also be detected. Evaluation of the right ventricular function is reliable in arrhythmogenic right ventricular cardiomyopathy (ARVC). The use of MRI is also able to directly detect the characteristic fibrofatty degeneration. In the rare restrictive cardiomyopathies (RCM), MRI can track restriction and, for example by means of T1, T2 and T2* mapping, detect sphingolipid accumulation in the myocardium in the context of Fabry disease or iron overload in the context of hemochromatosis.
The quantitative methods of parametric mapping provide the possibility of treatment monitoring but the clinical relevance of this monitoring is still the subject of current research. The unclassified cardiomyopathies can present clinically with similar symptoms to ischemic or inflammatory diseases, so that in the case of myocardial infarction without obstructive coronary arteries (MINOCA) in cardiac catheterization, MRI is a decisive diagnostic tool to determine the actual underlying disease. Similarly, in new cardiomyopathies such as noncompaction cardiomyopathy, MRI can pave the way for a morphological disease definition.
德国使用的心肌病分类可追溯至2008年欧洲心脏病学会（ESC）的分类。心肌病根据表型进行细分，因此磁共振成像（MRI）能够区分各种心肌病。
MRI的优势在于能够基于组织分化的可能性，将非缺血性心肌病与形态功能方面相似的其他疾病区分开来。例如，对于扩张型心肌病（DCM），可以与炎症性DCM进行区分。对于肥厚型心肌病（HCM），梗阻性和非梗阻性形式可类似于超声心动图进行区分，同时也能检测出淀粉样变性或法布里病。在致心律失常性右室心肌病（ARVC）中，对右室功能的评估是可靠的。MRI的应用还能够直接检测到特征性的纤维脂肪变性。在罕见的限制型心肌病（RCM）中，MRI可以追踪限制情况，例如通过T1、T2和T2* 成像，在法布里病的背景下检测心肌中的鞘脂蓄积，或在血色素沉着症的背景下检测铁过载。
参数成像的定量方法为治疗监测提供了可能性，但这种监测的临床相关性仍是当前研究的主题。未分类的心肌病在临床上可能表现出与缺血性或炎症性疾病相似的症状，因此在心脏导管检查中出现无阻塞性冠状动脉的心肌梗死（MINOCA）时，MRI是确定实际潜在疾病的决定性诊断工具。同样，在诸如心肌致密化不全心肌病等新型心肌病中，MRI可为形态学疾病定义铺平道路。
