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地中海贫血患者中心肌铁磁共振成像评估与左心室收缩和舒张功能的关系

Relationship of magnetic resonance imaging estimation of myocardial iron to left ventricular systolic and diastolic function in thalassemia.

作者信息

Leonardi Benedetta, Margossian Renee, Colan Steven D, Powell Andrew J

机构信息

Department of Cardiology, Children's Hospital Boston, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA.

出版信息

JACC Cardiovasc Imaging. 2008 Sep;1(5):572-8. doi: 10.1016/j.jcmg.2008.04.005.

Abstract

OBJECTIVES

We sought to evaluate whether echocardiographic diastolic function indices correlate with myocardial iron and systolic function in patients with transfusion-dependent thalassemia (TDT) who are at risk for cardiomyopathy.

BACKGROUND

In thalassemia syndromes, there is an important clinical need to risk stratify patients for the development of iron-overload cardiomyopathy so that chelation therapy can be adjusted and cardiac morbidity averted. This purpose may be served by measuring the magnetic resonance imaging (MRI)-derived parameter T2*, which varies inversely with tissue iron concentration but has limited availability. As diastolic dysfunction may precede systolic dysfunction, we sought to directly compare more readily available echocardiographic indices of diastolic function to myocardial T2* and ejection fraction (EF).

METHODS

We identified 47 paired echocardiography and MRI examinations in 24 patients with TDT. Echocardiographic measurements of transmitral flow velocities (E, A), tissue Doppler velocities (E'), and left ventricular volume and EF were compared with MRI measurements of myocardial T2*, ventricular volume, and EF.

RESULTS

All patients had a restrictive filling pattern (E/A >or=1.5 and deceleration time <140 ms) and normal relaxation. There was no significant correlation between E/E' or the Tei index versus EF. Although E/A and E' had statistically significant correlations with EF, the relationships were weak with all correlation coefficients <0.52. The parameters E/A, E', E/E', and the Tei index did not significantly correlate with myocardial iron concentration as assessed by MRI T2*. Increased myocardial iron as measured by T2* was strongly associated with lower left ventricular EF, with a T2* <9 ms having a sensitivity of 100% and specificity of 89% for MRI EF <50%.

CONCLUSIONS

In patients with TDT, echocardiographic diastolic function parameters correlated poorly with EF and myocardial T2* and were thus not well-suited for risk stratification. Myocardial T2* had a strong relationship with EF and appears to be a promising approach for predicting the development of heart failure and for iron chelator dose adjustment.

摘要

目的

我们试图评估超声心动图舒张功能指标是否与有心肌病风险的输血依赖型地中海贫血(TDT)患者的心肌铁含量及收缩功能相关。

背景

在地中海贫血综合征中,临床上迫切需要对有铁过载性心肌病发生风险的患者进行风险分层,以便调整螯合疗法并避免心脏疾病。通过测量磁共振成像(MRI)得出的参数T2可以实现这一目的,该参数与组织铁浓度呈负相关,但可用性有限。由于舒张功能障碍可能先于收缩功能障碍出现,我们试图直接比较更易获得的超声心动图舒张功能指标与心肌T2及射血分数(EF)。

方法

我们确定了24例TDT患者的47组配对超声心动图和MRI检查。将二尖瓣血流速度(E、A)、组织多普勒速度(E')以及左心室容积和EF的超声心动图测量值与心肌T2*、心室容积和EF的MRI测量值进行比较。

结果

所有患者均表现为限制性充盈模式(E/A≥1.5且减速时间<140毫秒)且舒张功能正常。E/E'或Tei指数与EF之间无显著相关性。虽然E/A和E'与EF有统计学意义上的相关性,但关系较弱,所有相关系数均<0.52。E/A、E'、E/E'和Tei指数与MRI T2评估的心肌铁浓度无显著相关性。通过T2测量的心肌铁含量增加与较低的左心室EF密切相关,对于MRI EF<50%,T2*<9毫秒的敏感性为100%,特异性为89%。

结论

在TDT患者中,超声心动图舒张功能参数与EF和心肌T2的相关性较差,因此不太适合用于风险分层。心肌T2与EF关系密切,似乎是预测心力衰竭发生及调整铁螯合剂剂量的一种有前景的方法。

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