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Electrocardiographic Presentation Cardiac Arrhythmias and Their Management in β‐Thalassemia Major Patients

机译:β-地中海贫血重症患者的心电图表现心脏心律不齐及其管理

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摘要

Beta‐thalassemia major (β‐ ) is a genetic hemoglobin disorder characterized by an absent synthesis of globin chains that are essential for hemoglobin formation, causing chronic hemolytic anemia. Clinical management of thalassemia major consists in regular long‐life red blood cell transfusions and iron chelation therapy to remove iron introduced in excess with transfusions. Iron deposition in combination with inflammatory and immunogenic factors is involved in the pathophysiology of cardiac dysfunction in these patients. Heart failure and arrhythmias, caused by myocardial siderosis, are the most important life‐limiting complications of iron overload in beta‐thalassemia patients. Cardiac complications are responsible for 71% of global death in the beta‐thalassemia major patients. The aim of this review was to describe the most frequent electrocardiographic abnormalities and arrhythmias observed in β‐ patients, analyzing their prognostic impact and current treatment strategies.
机译:严重的β地中海贫血(β-)是一种遗传性血红蛋白疾病,其特征是缺乏对血红蛋白形成必不可少的球蛋白链的合成,从而导致慢性溶血性贫血。重型地中海贫血的临床管理包括定期进行长寿命红细胞输注和铁螯合疗法,以消除输血时过量引入的铁。这些患者中铁沉积与炎症和免疫原性因子的结合参与了心脏功能障碍的病理生理。 β-地中海贫血患者中由铁质超负荷引起的心力衰竭和心律不齐是最重要的限制生命的并发症。严重的β型地中海贫血患者中,心脏并发症占全球死亡的71%。这篇综述的目的是描述在β-患者中观察到的最常见的心电图异常和心律不齐,分析其预后影响和当前的治疗策略。

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