Asymptomatic myocardial infarction in a patient with myotonic dystrophy type 1
Asymptomatic myocardial infarction in a patient with myotonic dystrophy type 1
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1 型强直性肌营养不良患者无症状心肌梗死
DOI：
10.1016/j.jccase.2022.05.004
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发表时间：
2022
影响因子：
--
通讯作者：
Komuro Issei
中科院分区：
文献类型：
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作者：
Seki Yuka;Yamada Takanobu;Kiyosue Arihiro;Kimura Koichi;Uehara Masae;Hatano Masaru;Sasako Takayoshi;Shirota Yuichiro;Sudo Atsushi;Ishiura Hiroyuki;Toda Tatsushi;Yamauchi Toshimasa;Komuro Issei
1型肌强直性营养不良（DM1）表现出广泛的心脏表现，包括传导系统紊乱、心律失常和心肌病。由于进行性心肌损伤和纤维化，DM1患者常表现为心电图异常，有时不能与心肌缺血区分。即使在心电图显示有冠状动脉疾病的DM1病例中，冠状动脉造影和冠状动脉计算机断层扫描也经常显示冠状动脉完整。在本文中，我们报告了一例56岁的DM1患者因心电图ST段改变而入院接受进一步检查。超声心动图显示室间隔壁严重运动不足，左室血栓顶端，提示左前降支区陈旧性心肌梗死的可能性。冠状动脉计算机断层血管造影和冠状动脉造影显示严重狭窄，提示LAD近端有易损斑块，尽管病变的血流储备分数未显示功能性缺血。一种β受体阻滞剂和一种钠-葡萄糖共转运蛋白2抑制剂被引入，期望具有心脏保护作用。出院一年后，患者死于吸入性肺炎引发的感染性和心源性休克。学习目的虽然1型强直性肌营养不良患者的主要心脏表现为传导异常和心肌病，但由于其常伴有代谢异常，如胰岛素抵抗和血脂异常导致糖尿病，且无症状或非特异性表现，诊断困难，因此应考虑冠心病的可能性。
Myotonic dystrophy type 1 (DM1) displays a wide range of cardiac manifestations, including conduction system disturbances, arrhythmias, and cardiomyopathy. As a result of progressive myocardial injury and fibrosis, patients with DM1 frequently show electrocardiogram (ECG) abnormalities which sometimes cannot be differentiated from myocardial ischemia. Even in DM1 cases with ECG findings indicative of coronary artery disease, coronary angiography and coronary computed tomography often demonstrate intact coronary arteries. In this article, we report a case of a 56-year-old DM1 patient with ST segment change on ECG, who was admitted to our hospital for further examination. Echocardiography revealed severe hypokinesis in the anteroseptal wall and left ventricular thrombus in the apex, suggesting the possibility of an old myocardial infarction in the left anterior descending artery (LAD) region. Coronary computed tomography angiography and coronary angiography demonstrated a severe stenosis suggestive of vulnerable plaque in the proximal part of LAD, although fractional flow reserve of the lesion did not indicate functional ischemia. A beta-blocker and a sodium-glucose cotransporter 2 inhibitor were introduced expecting a cardioprotective effect. One year after his discharge, the patient died of septic and cardiogenic shock triggered by aspiration pneumonia.Learning objectiveAlthough the prevalent cardiac manifestations of patients with myotonic dystrophy type 1 are conduction abnormalities and cardiomyopathy, the possibility of having coronary artery disease should be considered because they often have some atherosclerotic risk factors with their tendency toward metabolic abnormalities such as diabetes mellitus due to insulin resistance and dyslipidemia and with diagnostic difficulty due to asymptomatic or non-specific manifestations.