Division of Cardiology, Department of Internal Medicine, Medical University of Graz, Auenbruggerplatz 15, 8036, Graz, Austria.
Division of General Radiology, Department of Radiology, Medical University of Graz, Auenbruggerplatz 9, 8036, Graz, Austria.
ESC Heart Fail. 2020 Aug;7(4):1900-1908. doi: 10.1002/ehf2.12763. Epub 2020 May 31.
Cardiac involvement in myopathies that primarily affect the skeletal muscle is variable and may be subtle, necessitating sensitive diagnostic approaches. Here, we describe the prevalence of cardiac abnormalities in a cohort of patients with skeletal muscle disease presenting at a tertiary care neuromuscular centre.
We systematically investigated patients with skeletal myopathies and comprehensively analysed their cardiac phenotype including 24 h electrocardiogram, echocardiography with strain analyses, contrast-enhanced cardiac magnetic resonance imaging, and, if at increased risk of coronary artery disease, computed tomography coronary angiography. We prospectively screened 91 patients with diverse skeletal myopathies and enrolled 73 patients. The most pronounced cardiac involvement was present in patients with dystrophic myopathies (cardiac abnormalities in 59% of patients). We analysed myotonic dystrophies (n = 29) in more detail and found prolonged QRS (99.4 ± 15.6 vs. 91.5 ± 10.3 ms; P = 0.027) and QTc times (441.1 ± 28.1 vs. 413.0 ± 23.3 ms; P < 0.001) and increased left atrial size (27.28 ± 3.9 vs. 25.0 ± 3.2 mm/m ; P = 0.021) when compared with healthy controls. Left ventricular systolic function was reduced (ejection fraction < 55%) in 31% of myotonic dystrophies, while only 4% had an ejection fraction < 50%. Apical peak systolic longitudinal strain was slightly reduced (P = 0.023).
Screening for cardiac involvement in the skeletal muscle disease seems prudent particularly in patients with dystrophic myopathies. In the subset of myotonic dystrophy patients, QRS and QTc times as well as myocardial strain may be useful parameters. Their potential for predicting cardiac adverse events needs further evaluation.
主要影响骨骼肌的肌病可导致心脏受累，其变化程度不一且可能较为隐匿，因此需要采用敏感的诊断方法。在此，我们描述了在一家三级神经肌肉中心就诊的骨骼肌疾病患者队列中心脏异常的发生率。
我们系统地检查了患有骨骼肌病的患者，并全面分析了他们的心脏表型，包括 24 小时心电图、应变分析的超声心动图、对比增强心脏磁共振成像，如果存在冠状动脉疾病风险增加，则进行计算机断层扫描冠状动脉造影。我们前瞻性筛查了 91 名患有不同骨骼肌病的患者，并纳入了 73 名患者。在患有肌营养不良症的患者中存在最明显的心脏受累（59%的患者存在心脏异常）。我们更详细地分析了肌强直性营养不良症（n=29），发现 QRS 时间延长（99.4±15.6 比 91.5±10.3 ms；P=0.027）和 QTc 时间延长（441.1±28.1 比 413.0±23.3 ms；P<0.001），并且左心房增大（27.28±3.9 比 25.0±3.2 mm/m；P=0.021）。31%的肌强直性营养不良患者的左心室收缩功能降低（射血分数<55%），而只有 4%的患者射血分数<50%。心尖段收缩期纵向应变略有降低（P=0.023）。
在骨骼肌疾病中筛查心脏受累似乎是谨慎的，特别是在患有肌营养不良症的患者中。在肌强直性营养不良患者亚组中，QRS 和 QTc 时间以及心肌应变可能是有用的参数。需要进一步评估它们预测心脏不良事件的潜力。
