-地中海贫血患者的心肌功能指数恶化,尽管MRI显示铁负荷永久正常:反映心血管受累的简单而廉价的指标?

Umberto Barbero MD , Filomena Longo MD , Paola Destefanis MD , Carmen Maria Gaglioti MD , Roberto Pozzi MD , Antonio Piga MD, Prof
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引用次数: 12

摘要

背景反复输血引起的铁离子过载心肌病(IOC)仍然是重度地中海贫血(TM)患者死亡的主要原因。由于铁超载长期无症状,因此在出现心力衰竭临床症状之前,根据发生IOC的风险对患者进行分层是很重要的。磁共振成像(MRI) T2*可能有用,但价格昂贵,其MRI软件的可用性有限;常规超声心动图参数,虽然容易获得,保持正常,直到晚期的IOC。组织多普勒成像(TDI)为直接或通过心肌表现指数(MPI)等衍生指标探讨心脏收缩和舒张功能开辟了新的途径,在不同的心肌病中具有预后价值。方法连续选取46例无心衰临床症状的β-TM患者,分别于2011年和2015年进行超声心动图检查。采用TDI法计算左、右心室外壁MPI。所有TM患者前一年均行心脏T2* MRI检查。结果尽管超声和T2*评价的形态学数据没有明显变化,但随着时间的推移,侧室壁S波减少,MPI在随访期间恶化,肺动脉收缩压(PAPs)在2015年较2011年升高。结论尽管T2*值正常,但tdi和MPI可在早期发现TM患者心肌功能障碍。与pap一起,这些患者应定期检查。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Worsening of myocardial performance index in beta-thalassemia patients despite permanently normal iron load at MRI: A simple and cheap index reflecting cardiovascular involvement?

Background

Iron Overload Cardiomyopathy (IOC) due to repeated transfusions still represents the main cause of death in Thalassemia major (TM) patients. Because iron overload remains asymptomatic for long time, it is important to stratify the patients based on the risk of developing IOC before the appearance of clinical signs of heart failure. The magnetic resonance imaging (MRI) T2* may be useful but it is expensive and its MRI software has limited availability; conventional echocardiographic parameters, although easy availability, remain normal until advanced stages of IOC. Tissue Doppler Imaging (TDI) opened a new way to explore systolic and diastolic function directly or through derived index such as myocardial performance index (MPI) which has a prognostic value in different cardiomyopathies.

Methods

We enrolled 46 consecutive β-TM patients without clinical signs of heart failure and we tested them with echocardiography in 2011 and again in 2015. MPI of left and right ventricular lateral wall was calculated by TDI. All TM patients had a T2* MRI evaluation of the heart in the year before.

Results

Despite the absence of significant changes in morphological data at echo and at T2* evaluation, S′ waves of the lateral ventricular wall decrease while time passed, MPI worsened during the follow-up period and the derived systolic pulmonary artery systolic pressure (PAPs) increased in 2015 with respect to 2011.

Conclusion

TDI and MPI may reveal very early myocardial dysfunction in TM patients despite a normal T2* value. Together with PAPs they should be periodically checked in these patients.

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