EARLY DETECTION OF IRON CARDIOMYOPATHY IN THALESSEMIA

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可在其他CRISP条目中表示。所列机构为 中心，但不一定是研究者所在的机构。 本研究的目的是确定心脏功能、运动能力和心脏节律与心脏和肝脏铁负荷之间的关系。80例不同阶段铁过载的患者将接受全面的临床心脏病学评价（ECG、超声心动图、负荷运动和霍尔特监测）和MRI研究（以量化肝脏和心肌铁以及心脏功能）。这些研究可能导致1）地中海贫血患者的心律和性能的规范性数据，2）确定心脏铁含量高且静息功能正常的地中海贫血患者中运动和心律异常的患病率，以及3）确定肝脏铁含量、铁蛋白、心脏T2* 和心脏功能之间的关系。这些研究将为地中海贫血患者心脏铁负荷对心脏功能的纵向研究提供必要的基础。这项研究的结果可能会导致改善，早期识别临床前铁心肌病。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this study is to determine the relationship between cardiac function, exercise capacity, and cardiac rhythm to cardiac and liver iron loading. Eighty patients with various stages of iron overload will have a full, clinical cardiology evaluation (ECG, Echo, stress exercise and Holter monitoring) and a research MRI study (to quantify liver and myocardial iron and cardiac function). These studies may lead to 1) normative data for cardiac rhythm and performance for thalassemia patients, 2) determination of the prevalence of exercise and cardiac rhythm abnormalities in thalassemia patients with high cardiac iron and normal resting function, and 3) determination of the relationships between liver iron content, ferritin, cardiac T2* and cardiac function. These studies will provide the necessary groundwork for longitudinal studies of cardiac iron load on cardiac performance in thalassemia patients. The results of this study may lead to improved, early recognition of pre-clinical iron cardiomyopathy.