EARLY DETECTION OF IRON CARDIOMYOPATHY IN THALASSEMIA

这个子项目是利用由NIH/NCRR资助的中心拨款提供的资源的许多研究子项目之一。子项目和调查员(PI)可能从另一个NIH来源获得了主要资金，因此可能会出现在其他CRISE条目中。列出的机构是针对中心的，而不一定是针对调查员的机构。本研究的目的是确定心脏功能、运动能力和心率与心脏和肝脏铁负荷的关系。80名不同阶段铁负荷过高的患者将接受全面的临床心脏病学评估(心电图、回声、应激运动和Holter监测)和研究性核磁共振研究(量化肝脏和心肌铁和心脏功能)。这些研究可能导致：1)地中海贫血患者心脏节律和表现的标准化数据，2)心脏铁含量高、静息功能正常的地中海贫血患者运动和心率异常的发生率，以及3)肝铁含量、铁蛋白、心脏T2*和心功能之间的关系的确定。这些研究将为地中海贫血患者心脏铁负荷对心脏功能影响的纵向研究提供必要的基础。这项研究的结果可能有助于改善对临床前铁性心肌病的早期认识。

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.