β -地中海贫血的心力衰竭

D. Kremastinos
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引用次数: 26

摘要

尽管已取得进展,但心力衰竭仍然是β-地中海贫血患者死亡的主要原因。心肌铁沉积本身不影响左心室舒张,但直接导致左心室心肌受限,肺动脉压明显升高。这导致主要是右侧心力衰竭的症状和体征,这通常在老年人和严重含铁血黄素人群中观察到。左心室收缩功能障碍和衰竭,发生在年轻,较少含铁血黄素的人群,似乎是多因素的病因。在扩张型心肌病的背景下,除铁负荷外,免疫遗传危险因素还可触发左侧心力衰竭的发生机制。
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Heart Failure in β‐Thalassemia
Heart failure remains the main cause of death in β-thalassemia despite the progress that has been made. Myocardial iron deposition alone does not affect left ventricular relaxation but directly causes left ventricular myocardial restriction with considerably elevated pulmonary pressure. This leads to symptoms and signs of predominantly right-sided heart failure, which is usually observed in elderly and severely hemosiderotic populations. Left ventricular systolic dysfunction and failure, which occurs in younger, less hemosiderotic populations, seems to be multifactorial in etiology. Apart from iron loading, immunogenetic risk factors trigger the mechanisms of left-sided heart failure development in the context of dilated-type cardiomyopathy.
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