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Cardiac magnetic resonance in patients with muscular dystrophies
European Journal of Preventive Cardiology ( IF 8.4 ) Pub Date : 2020-05-16 , DOI: 10.1177/2047487320923052
Chrysanthos Grigoratos 1 , Alberto Aimo 2 , Andrea Barison 1, 2 , Vincenzo Castiglione 2 , Giancarlo Todiere 1 , Giulia Ricci 3 , Gabriele Siciliano 3 , Michele Emdin 1, 2
Affiliation  

Muscular dystrophies are inherited disorders sharing similar clinical features and dystrophic changes on muscle biopsy. Duchenne muscular dystrophy is the most common inherited muscle disease of childhood, and Becker muscular dystrophy is a milder allelic variant with a slightly lower prevalence. Myotonic dystrophy is the most frequent form in adults. Cardiac magnetic resonance is the gold standard technique for the quantification of cardiac chamber volumes and function, and also enables a characterisation of myocardial tissue. Most cardiac magnetic resonance studies in the setting of muscular dystrophy were carried out at single centres, evaluated small numbers of patients and used widely heterogeneous protocols. Even more importantly, those studies analysed more or less extensively the patterns of cardiac involvement, but usually did not try to establish the added value of cardiac magnetic resonance to standard echocardiography, the evolution of cardiac disease over time and the prognostic significance of cardiac magnetic resonance findings. As a result, the large and heterogeneous amount of information on cardiac involvement in muscular dystrophies cannot easily be translated into recommendations on the optimal use of cardiac magnetic resonance. In this review, whose targets are cardiologists and neurologists who manage patients with muscular dystrophy, we try to summarise cardiac magnetic resonance findings in patients with muscular dystrophy, and the results of studies evaluating the role of cardiac magnetic resonance as a tool for diagnosis, risk stratification and follow-up. Finally, we provide some practical recommendations about the need and timing of cardiac magnetic resonance examination for the management of patients with muscular dystrophy.

中文翻译:

肌营养不良症患者的心脏磁共振

肌营养不良症是遗传性疾病,具有相似的临床特征和肌肉活检的营养不良变化。杜氏肌营养不良症是儿童时期最常见的遗传性肌肉疾病,而贝克尔肌营养不良症是一种较温和的等位基因变异,患病率略低。强直性肌营养不良是成人中最常见的形式。心脏磁共振是量化心室体积和功能的金标准技术,并且还能够表征心肌组织。大多数针对肌营养不良症的心脏磁共振研究都是在单一中心进行的,评估了少量患者并使用了广泛的异质方案。更重要的是,这些研究或多或少地广泛分析了心脏受累的模式,但通常没有试图确定心脏磁共振对标准超声心动图的附加价值、心脏病随时间的演变以及心脏磁共振的预后意义发现。因此,关于肌营养不良症心脏受累的大量且异构的信息不能轻易转化为关于心脏磁共振最佳使用的建议。在这篇综述中,我们的目标是管理肌营养不良症患者的心脏病专家和神经科医生,我们试图总结肌营养不良症患者的心脏磁共振检查结果,以及评估心脏磁共振作为诊断、风险工具的作用的研究结果。分层和跟进。最后,我们针对肌营养不良症患者进行心脏磁共振检查的必要性和时机提供了一些实用建议。
更新日期:2020-05-16
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