Diagnosis and management of iron-induced heart disease in Cooley's anemia
Diagnosis and management of iron-induced heart disease in Cooley's anemia
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DOI：
10.1111/j.1749-6632.1998.tb10481.x
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发表时间：
1998-01-01
期刊：
影响因子：
--
通讯作者：
Manno, CS
中科院分区：
文献类型：
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作者：
Jessup, M;Manno, CS
患有纯合子β地中海贫血的患者长期输血，如果不进行严格的螯合，则有心功能障碍的风险。现有数据表明，即使在最佳螯合的患者中，心脏病理学也是继发于铁沉积、纤维化、肥大和慢性贫血的结构效应的异常。心肌心包炎的证据也可能被发现。心脏功能通常只受到轻微的影响，主要是超声心动图或核扫描常规检测不到的舒张异常。在螯合不良的患者中，发生严重的心力衰竭，很容易预测，但总是致命的，尽管用利尿剂，血管扩张剂，正性肌力药，局部抗心律失常药治疗。基于ACE抑制剂在其他形式的心肌病中成功预防心力衰竭，我们建议进行多中心试验，以探索稳定铁诱导心脏病风险患者心功能的方法。当缺血性心脏病的可能性可能复合心功能不全时，需要对达到30岁和40岁的患者的铁沉积、舒张功能不全和异常激素调节的长期不良影响进行定量。
Patients with homozygous beta-thalassemia are chronically transfused and, if not assiduously chelated, are at risk for cardiac dysfunction. Available data suggest that even in optimally chelated patients, cardiac pathology is abnormal secondary to iron deposition, fibrosis, hypertrophy, and the structural effects of chronic anemia. Evidence of myopericarditis may also be found. Cardiac performance is usually only subtly affected, primarily with diastolic abnormalities not routinely detected on echocardiograms or nuclear scan. In poorly chelated patients, severe heart failure occurs and is easily predictable but invariably fatal, despite treatment with diuretics, vasodilators, inotropes, areal antiarrhythmics. Based on successful prevention of heart failure with ACE inhibitors in other forms of cardiomyopathy, we suggest multicenter trials to explore methods to stabilize cardiac function in patients at risk for iron-induced heart disease. Long-term adverse effects of iron deposition, diastolic dysfunction, and abnormal hormone regulation need to be quantitated in patients reaching their third and fourth decades when the potential for ischemic cardiac disease could compound cardiac dysfunction.