Working Group on Cardiovascular Magnetic Resonance, Experimental and Clinical Research Center a joint cooperation between the Charité - Universitätsmedizin Berlin, Department of Internal Medicine and Cardiology and the Max-Delbrueck Center for Molecular Medicine, and HELIOS Klinikum Berlin Buch, Department of Cardiology and Nephrology, Berlin, Germany.
DZHK (German Centre for Cardiovascular Research), Berlin, Germany.
Curr Heart Fail Rep. 2021 Aug;18(4):211-224. doi: 10.1007/s11897-021-00521-2. Epub 2021 Jul 28.
This review aims to outline the utility of cardiac magnetic resonance (CMR) in patients with different types of muscular dystrophies for the assessment of myocardial involvement, risk stratification and in guiding therapeutic decisions.
In patients suffering from muscular dystrophies (MD), even mild initial dysfunction may lead to severe heart failure over a time course of years. CMR plays an increasing role in the diagnosis and clinical care of these patients, mostly due to its unique capability to precisely characterize subclinical and progressive changes in cardiac geometry, function in order to differentiate myocardial injury it allows the identification of inflammation, focal and diffuse fibrosis as well as fatty infiltration. CMR may provide additional information in addition to the physical examination, laboratory tests, ECG, and echocardiography. Further trials are needed to investigate the potential impact of CMR on the therapeutic decision-making as well as the assessment of long-term prognosis in different forms of muscular dystrophies. In addition to the basic cardiovascular evaluation, CMR can provide a robust, non-invasive technique for the evaluation of subclinical myocardial tissue injury like fat infiltration and focal and diffuse fibrosis. Furthermore, CMR has a unique capability to detect the progression of myocardial tissue damage in patients with a preserved systolic function.
本文旨在概述心脏磁共振(CMR)在不同类型肌营养不良症患者中的应用,用于评估心肌受累、危险分层和指导治疗决策。
在肌营养不良症(MD)患者中,即使是轻微的初始功能障碍也可能导致心脏在数年内出现严重衰竭。CMR 在这些患者的诊断和临床治疗中发挥着越来越重要的作用,主要是因为它具有独特的能力,可以精确描述心脏几何形状和功能的亚临床和进行性变化,从而区分心肌损伤,它还可以识别炎症、局灶性和弥漫性纤维化以及脂肪浸润。CMR 可以提供除体格检查、实验室检查、心电图和超声心动图之外的其他信息。需要进一步的试验来研究 CMR 对不同类型肌营养不良症的治疗决策和长期预后评估的潜在影响。除了基本的心血管评估外,CMR 还可以为评估脂肪浸润和局灶性及弥漫性纤维化等亚临床心肌组织损伤提供一种强大的、非侵入性的技术。此外,CMR 还具有独特的能力来检测收缩功能正常患者心肌组织损伤的进展。