Tuzzolo Anthony, Febres-Aldana Christopher A, Poppiti Robert
From the Herbert Wertheim College of Medicine, Florida International University, Miami.
Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL.
Am J Forensic Med Pathol. 2020 Mar;41(1):42-47. doi: 10.1097/PAF.0000000000000531.
Myocardial steatosis, also known as lipomatosis cordis, is characterized by adipose tissue within the myocardium without significant fibrosis. Evidence suggests that accumulation of fat can disturb the normal electromechanical physiology of the myocardium. Herein, we discuss the case of a 60-year-old woman with a history of chronic obstructive pulmonary disease who died because of anoxic encephalopathy after a sudden cardiac arrest (SCA). An electrocardiogram showed QRS fragmentation noted as notched R in inferior leads. The autopsy revealed a very small thromboembolus in a distal subsegmental branch of the pulmonary artery, which could not explain the SCA. There was an extensive intramyocardial accumulation of adipose tissue involving the right ventricle and interventricular septum, which split the myocardium into discrete bundles. Arrhythmogenic right ventricular cardiomyopathy was ruled out based on the absence of typical fibrofatty changes. The mechanism of fat replacement was likely secondary to redistribution of visceral fat in the setting of Cushing syndrome. We propose that severe myocardial steatosis can create an anatomic substrate to facilitate the development of SCA. Myocardial steatosis should be reported to identify patients who are at risk for developing cardiovascular events secondary to extreme cardiac adiposity.
心肌脂肪变性，也称为心脏脂肪瘤病，其特征是心肌内存在脂肪组织且无明显纤维化。有证据表明，脂肪堆积会扰乱心肌的正常机电生理。在此，我们讨论一例60岁患有慢性阻塞性肺疾病的女性病例，该患者在心脏骤停（SCA）后因缺氧性脑病死亡。心电图显示下壁导联出现QRS波碎裂，表现为R波有切迹。尸检发现肺动脉远端亚段分支有一个非常小的血栓栓子，这无法解释心脏骤停。心肌内有广泛的脂肪组织堆积，累及右心室和室间隔，使心肌分裂成离散的束状。基于无典型的纤维脂肪改变，排除了致心律失常性右心室心肌病。脂肪替代的机制可能继发于库欣综合征背景下内脏脂肪的重新分布。我们提出，严重的心肌脂肪变性可形成一种解剖学基础，促进心脏骤停的发生。应报告心肌脂肪变性，以识别因极度心脏脂肪过多而有发生心血管事件风险的患者。
