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表现为急性心肌心包炎的严重铁过载心肌病:一例报告

Severe iron overload cardiomyopathy manifested as acute myopericarditis: A case report.

作者信息

Kosum Paisit, Theerasuwipakorn Nonthikorn, Wicheantawatchai Aranna, Bunnag Napisa, Wanlapakorn Chaisiri, Tumkosit Monravee, Uaprasert Noppacharn, Satitthummanid Sudarat

机构信息

Division of Cardiovascular Medicine, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Division of Cardiovascular Medicine, Department of Medicine, Faculty of Medicine, Naresuan University, Phitsanulok, Thailand.

出版信息

Radiol Case Rep. 2023 Nov 4;19(1):290-295. doi: 10.1016/j.radcr.2023.10.020. eCollection 2024 Jan.

Abstract

Iron overload cardiomyopathy (IOC) is a condition in which iron deposition in the heart causes cardiac dysfunction. We described a 21-year-old woman who presented with acute chest pain, dyspnea, and fever. The patient had a history of transfusion-dependent thalassemia (TDT) and secondary hemochromatosis with the latest serum ferritin ranging from 8000 to 15,000. Physical examinations revealed signs of anemia and heart failure. Electrocardiography showed diffuse ST-segment elevation with reciprocal ST-segment depression in aVR and complete atrioventricular block. Cardiac markers were markedly elevated. Echocardiography demonstrated the dilated size, impaired systolic function, global wall hypokinesia, restrictive filling pattern of the left ventricle, and a small amount of pericardial effusion. Coronary angiography showed normal coronary arteries. A cardiac magnetic resonance imaging showed multifocal early and late gadolinium enhancement involving mid-wall and subepicardial areas of biventricular myocardium suggestive of diffuse myocardial injury from an inflammatory process. She was provisionally diagnosed with acute myopericarditis. Ibuprofen and loop diuretic were prescribed; however, cardiogenic shock occurred. Thus, an endomyocardial biopsy was done and revealed diffuse myocardial hemosiderin deposition without evidence of inflammatory cell infiltration. Severe IOC mimicking acute myopericarditis was considered based on an endomyocardial biopsy result. An intravenous iron chelating agent was immediately administered. Unfortunately, cardiogenic shock was refractory resulting in death. This case demonstrated a rare manifestation of IOC, which can masquerade as acute myopericarditis, and emphasized that IOC should be differentially diagnosed, particularly in patients with TDT and hemochromatosis.

摘要

铁过载心肌病(IOC)是一种心脏中铁沉积导致心脏功能障碍的病症。我们描述了一名21岁女性,她出现急性胸痛、呼吸困难和发热。该患者有输血依赖型地中海贫血(TDT)和继发性血色素沉着症病史,最新血清铁蛋白范围为8000至15000。体格检查发现贫血和心力衰竭体征。心电图显示弥漫性ST段抬高,aVR导联出现ST段压低及完全性房室传导阻滞。心脏标志物明显升高。超声心动图显示左心室扩大、收缩功能受损、整体室壁运动减弱、左心室限制性充盈模式以及少量心包积液。冠状动脉造影显示冠状动脉正常。心脏磁共振成像显示双心室心肌中层和心外膜下区域有多灶性早期和晚期钆增强,提示炎症过程导致弥漫性心肌损伤。她被初步诊断为急性心肌心包炎。给予布洛芬和袢利尿剂治疗;然而,发生了心源性休克。因此,进行了心内膜心肌活检,结果显示弥漫性心肌含铁血黄素沉积,无炎症细胞浸润证据。根据心内膜心肌活检结果,考虑为严重IOC伪装成急性心肌心包炎。立即给予静脉铁螯合剂。不幸的是,心源性休克难治,最终导致死亡。该病例展示了IOC的一种罕见表现,它可伪装成急性心肌心包炎,并强调应进行鉴别诊断IOC,尤其是在TDT和血色素沉着症患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/60d0/10652097/d22f12c3a05d/gr1.jpg

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