Management of cardiac hemochromatosis
Management of cardiac hemochromatosis
复制标题
DOI：
10.5114/aoms.2017.68729
复制
发表时间：
2018-04-01
影响因子：
3.8
通讯作者：
Aronow, Wilbert S.
中科院分区：
文献类型：
--
作者：
Aronow, Wilbert S.
铁超载综合征可能是遗传性的或获得性的。患者在疾病早期可能无症状。一旦发生心力衰竭，就会迅速恶化。心脏血色素沉着症的特征是扩张型心肌病伴心室扩张、射血分数降低和短轴缩短率降低。铁的沉积可发生在整个心脏传导系统，特别是房室结。任何原因不明的心力衰竭患者应考虑心脏血色病。应使用血清铁蛋白和转铁蛋白饱和度筛查全身性铁超载。如果这些检查结果与铁过载一致，则需要进一步进行无创性和组织学确认，以确认铁过载累及的器官。心脏磁共振成像是上级其他诊断测试，因为它可以定量评估心肌铁负荷。治疗性静脉切开术是非贫血性心脏血色病患者的首选治疗方法。血清铁蛋白水平≥ 300 μ g/l的男性和血清铁蛋白水平≥ 200 μ g/l的女性应开始治疗性放血。治疗性放血包括每周抽取1单位血液（450 - 500 ml），直到血清铁蛋白水平为10 - 20 μ g/l，此后通过定期抽取血液将血清铁蛋白水平维持在50 μ g/l或更低。静脉切开术不是贫血（继发性铁超负荷紊乱）患者的治疗选择，也不是严重充血性心力衰竭患者的治疗选择。在这些患者中，治疗的选择是铁螯合疗法。
Iron-overload syndromes may be hereditary or acquired. Patients may be asymptomatic early in the disease. Once heart failure develops, there is rapid deterioration. Cardiac hemochromatosis is characterized by a dilated cardiomyopathy with dilated ventricles, reduced ejection fraction, and reduced fractional shortening. Deposition of iron may occur in the entire cardiac conduction system, especially the atrioventricular node. Cardiac hemochromatosis should be considered in any patient with unexplained heart failure. Screening for systemic iron overload with serum ferritin and transferin saturation should be performed. If these tests are consistent with iron overload, further noninvasive and histologic confirmation is indicated to confirm organ involvement with iron overload. Cardiac magnetic resonance imaging is superior to other diagnostic tests since it can quantitatively assess myocardial iron load. Therapeutic phlebotomy is the therapy of choice in nonanemic patients with cardiac hemochromatosis. Therapeutic phlebotomy should be started in men with serum ferritin levels of 300 mu g/l or more and in women with serum ferritin levels of 200 mu g/l or more. Therapeutic phlebotomy consists of removing 1 unit of blood (450 to 500 ml) weekly until the serum ferritin level is 10 to 20 mu g/l and maintenance of the serum ferritin level at 50 mu g/l or lower thereafter by periodic removal of blood. Phlebotomy is not a treatment option in patients with anemia (secondary iron-overload disorders) nor in patients with severe congestive heart failure. In these patients, the treatment of choice is iron chelation therapy.