Reznik Elena V, Nguyen Thanh Luan, Semyachkina Alla N, Shkolnikova Maria A
Pirogov Russian National Research Medical University of the Ministry of Healthcare of the Russian Federation, Moscow, Russia.
Department of Moscow, "City Clinical Hospital No 31" of Healthcare, Moscow, Russia.
Clin Med Insights Cardiol. 2024 Oct 1;18:11795468241249075. doi: 10.1177/11795468241249075. eCollection 2024.
Infiltrative heart disease (InHD) is a group of diseases characterized by the deposition of abnormal substances in the heart tissue, causing diastolic, less often systolic, dysfunction of the ventricle(s). Their classification still does not exist. In 2013, the MOGE(S) classification of cardiomyopathies was published, taking into account, along with the morphological and functional characteristics of the heart, damage to other organs, the presence of genetic mutations, acquired causes (e.g., myocardial inflammation, autoimmune diseases, storage diseases, amyloidosis), etc. By analogy with it we offer the MORAL-STAGE classification for InHD. It includes ten features: morphofunctional characteristics (M), organ damage (O), risk of cardiac death (R), age of clinical presentation, age of disease-specific therapy initiation (A), localization of the infiltrative process (inside or outside the cell, L), information about the functional class heart failure and stage of infiltrative heart disease (S), treatment (T), abnormal rhythm or conduction (A), genetic or familial nature of inheritance (G), etiology of the process (E). This article summarizes the cornerstones of the MORAL-STAGE classification and its clinical relevance. In addition, new issues are discussed that can be considered in future versions of the MORAL-STAGE classification.
浸润性心脏病(InHD)是一组以异常物质沉积于心脏组织为特征的疾病,可导致心室舒张功能障碍,较少见收缩功能障碍。目前尚无其分类方法。2013年,心肌病的MOGE(S)分类法发表,该分类法除考虑心脏的形态和功能特征外,还考虑其他器官的损害、基因突变的存在、后天病因(如心肌炎症、自身免疫性疾病、贮积病、淀粉样变性等)。与之类似,我们提出了InHD的MORAL-STAGE分类法。它包括十个特征:形态功能特征(M)、器官损害(O)、心源性死亡风险(R)、临床表现年龄、疾病特异性治疗起始年龄(A)、浸润过程的定位(细胞内或细胞外,L)、心力衰竭功能分级和浸润性心脏病分期的信息(S)、治疗(T)、心律失常或传导异常(A)、遗传或家族遗传性质(G)、疾病过程的病因(E)。本文总结了MORAL-STAGE分类法的要点及其临床相关性。此外,还讨论了可在MORAL-STAGE分类法未来版本中考虑的新问题。