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铁过载性心肌病:病理生理学、诊断和治疗。

Iron-overload cardiomyopathy: pathophysiology, diagnosis, and treatment.

机构信息

Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Alberta, Canada.

出版信息

J Card Fail. 2010 Nov;16(11):888-900. doi: 10.1016/j.cardfail.2010.05.009. Epub 2010 Jul 3.

DOI:10.1016/j.cardfail.2010.05.009
PMID:21055653
Abstract

BACKGROUND

The prevalence of primary (hereditary) hemochromatosis and secondary iron overload (hemosiderosis) is reaching epidemic levels worldwide. Iron-overload leads to excessive iron deposition in a wide variety of tissues, including the heart and endocrine tissues.

METHODS AND RESULTS

Iron-overload cardiomyopathy is the primary determinant of survival in patients with secondary iron overload, while also being a leading cause of morbidity and mortality in patients with primary hemochromatosis. Iron-induced cardiovascular injury also occurs in acute iron toxicosis (iron poisoning), myocardial ischemia-reperfusion injury, cardiomyopathy associated with Friedreich ataxia, and vascular dysfunction. The mainstay therapies for iron overload associated with primary hemochromatosis and secondary iron overload is phlebotomy and iron chelation therapy, respectively. L-type Ca(2+) channels provide a high-capacity pathway for ferrous (Fe(2+)) uptake into cardiomyocytes in iron-overload conditions; calcium channel blockers may represent a new therapeutic tool to reduce the toxic effects of excess iron.

CONCLUSIONS

Iron-overload cardiomyopathy is a an important and potentially reversible cause of heart failure at an international scale and involves diastolic dysfunction, increased susceptibility to arrhythmias and a late-stage dilated cardiomyopathy. The early diagnosis of iron-overload cardiomyopathy is critical since the cardiac dysfunction is reversible if effective therapy is introduced before the onset of overt heart failure.

摘要

背景

原发性(遗传性)血色素沉着症和继发性铁过载(血色素沉积症)的患病率正在全球范围内达到流行水平。铁过载导致铁在包括心脏和内分泌组织在内的多种组织中过度沉积。

方法和结果

铁过载性心肌病是继发性铁过载患者生存的主要决定因素,也是原发性血色素沉着症患者发病率和死亡率的主要原因。铁诱导的心血管损伤也发生在急性铁中毒(铁中毒)、心肌缺血再灌注损伤、弗里德里希共济失调相关心肌病和血管功能障碍中。原发性血色素沉着症和继发性铁过载相关铁过载的主要治疗方法分别是放血和铁螯合疗法。在铁过载条件下,L 型钙 (Ca 2+) 通道为亚铁 (Fe 2+) 进入心肌细胞提供了一种高容量途径;钙通道阻滞剂可能代表一种新的治疗工具,可减少过量铁的毒性作用。

结论

铁过载性心肌病是国际范围内心力衰竭的一个重要且潜在可逆转的原因,涉及舒张功能障碍、心律失常易感性增加和晚期扩张型心肌病。铁过载性心肌病的早期诊断至关重要,因为如果在明显心力衰竭发生之前引入有效治疗,心脏功能障碍是可逆的。

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