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病例报告:心室心肌致密化不全导致的心脏性猝死。

Case Report: Sudden cardiac death due to ventricular myocardial non-compaction.

机构信息

Department of Forensic Medicine, Taher Sfar Hospital, University of Monastir, Mahdia, 5100, Tunisia.

Department of Cardiology, Taher Sfar Hospital, University of Monastir, Mahdia, 5100, Tunisia.

出版信息

F1000Res. 2020 Aug 26;9:1045. doi: 10.12688/f1000research.24583.2. eCollection 2020.

Abstract

Ventricular non-compaction (VNC) is a rare myocardium disorder, which can be both genetic and sporadic. A poor wall compaction process or an excessive trabeculae formation may be at the genesis of myocardial hypertrabeculation with multiple recesses. It is often complicated by ventricular dysfunction, arrhythmias and cardiac embolism. Herein we report a case of a 20-year-old male patient with no particular past medical history who was followed up at the cardiology department for dyspnea. Echocardiography showed reduced ejection fraction of the left ventricle with potential hypertrabeculation in the right ventricle, confirmed by cardiac MRI. The patient was not put under medication and was later lost to follow-up. He died few months later without a clear cause explaining death. A forensic autopsy was performed that attributed death to acute ventricle arrhythmia secondary to VNC, emphasizing the major role of an early and specific treatment to avoid such a fatal outcome.

摘要

心室肌致密化不全(VNC)是一种罕见的心肌疾病,可为遗传性或散发性。心肌肥厚伴多发性深陷窦可能是由于心壁致密过程不良或小梁过度形成所致。它常伴有心室功能障碍、心律失常和心脏栓塞。在此,我们报告了一例 20 岁男性患者,无特殊既往病史,因呼吸困难在心脏病科就诊。超声心动图显示左心室射血分数降低,右心室可能存在心肌肥厚,心脏 MRI 进一步证实了这一点。该患者未接受药物治疗,随后失访。几个月后,他不明原因死亡。进行了法医尸检,死因归咎于 VNC 继发的急性心室心律失常,强调了早期和特异性治疗的重要性,以避免这种致命结局。

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