左心室不压实:未确诊的致命疾病

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摘要

左室非压实性(LVNC)是一种先天性心肌病,其特征是存在突出的小梁间凹陷,通常影响左室腔的根尖部分。这些凹陷使受影响的心肌具有独特的表型,被描述为“海绵状”。在LVNC患者中观察到毁灭性的并发症,包括在某些情况下需要心脏移植的心力衰竭,危及生命的心律失常,血栓栓塞事件,包括中风和短暂性脑缺血发作以及心源性猝死。本报告的病例为62岁女性患者,反复胸痛数年,大量阴性检查和最近的超声心动图显示左心室小梁增加,怀疑为LVNC,后经心脏磁共振(CMR)证实。
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Left ventricular non-compaction: Underdiagnosed disease with fatal outcomes
Left ventricular non compaction (LVNC) is a type of congenital cardiomyopathy characterized by the presence of prominent intertrabecular recesses that typically affect the apical portion of the left ventricular cavity. These recesses give a distinctive phenotype to the affected myocardium that has been described as “sponge-like”. Devastating complications have been observed in those patients with LVNC, including heart failure requiring heart transplantation in selected cases, life-threatening arrhythmias, thromboembolic events including stroke and transient ischemic attack and sudden cardiac death. This report follows the case of a 62-year-old female patient with recurrent chest pain for several years and extensive negative work and recent echocardiography showed increased left ventricle trabeculations, suspicious for LVNC, later confirmed by cardiac magnetic resonance (CMR).
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