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地中海贫血患者反复输血时,心肌收缩力学与血清铁蛋白水平作为预后指标的关系。

Relation of myocardial systolic mechanics to serum ferritin level as a prognosticator in thalassemia patients undergoing repeated transfusion.

作者信息

Chen Ming-Ren, Ko Ho-Shun, Chao Tze-Fan, Liu Hsi-Che, Kuo Jen-Yuan, Bulwer Bernard E, Yeh Hung-I, Hung Chung-Lieh

机构信息

Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan; Mackay Junior College of Medicine Nursing and Management, Taipei, Taiwan; Mackay Medical College, Taipei, Taiwan.

出版信息

Echocardiography. 2015 Jan;32(1):79-88. doi: 10.1111/echo.12590. Epub 2014 Mar 27.

Abstract

INTRODUCTION

Myocardial iron overload leading to congestive heart failure (HF) or arrhythmias is a recognized complication in thalassemia patients undergoing chronic blood transfusion. The relationship between myocardial iron load, subclinical systolic dysfunction, and clinical events remains less well known.

MATERIALS AND METHODS

We studied a total of 77 subjects, comprising 37 thalassemia patients (mean age: 24.2 ± 5.5 years) with a history of repeated blood transfusions, in addition to 40 age- and gender-matched controls (mean age: 24 ± 4.5 years). Serum ferritin levels were checked in all subjects, as well as semiautomated quantification of left ventricular (LV) longitudinal, circumferential, and radial deformations assessed by two-dimensional (2D) speckle tracking analysis.

RESULTS

Compared with the control group, thalassemia patients showed significantly larger LV mass index and lower myocardial deformations (P < 0.05), but left ventricular ejection fraction (LVEF) did not differ significantly (P = 0.233). There was a significant linear correlation between serum ferritin level and longitudinal (r = 0.44, P = 0.0078) and radial strain (r = -0.46, P = 0.0051), with optimal cutoff provided to be -15.48%, -21.31%, and 26.67% for longitudinal, circumferential, and radial strain in predicting clinical events, respectively. During a median of 802.5 days follow-up, 11 clinical composites (HF incidence, ventricular tachyarrhythmia, and death) occurred (N = 9 subjects); subjects with composites had significantly reduced longitudinal and radial strain compared to those without (both P < 0.05). After adjusting for age, sex, serum ferritin level, and LV mass index, a worsening of longitudinal strain remained as an independent predictor of clinical events and death (HR: 6.05, P = 0.033).

CONCLUSION

Subclinical systolic dysfunction appears more likely in thalassemia subjects with a history of repeated blood transfusions, which further correlated with serum ferritin levels. In addition, worsening LV myocardial deformation parameters may play an independent role in predicting clinical outcomes beyond traditional measures in this population.

摘要

引言

心肌铁过载导致充血性心力衰竭(HF)或心律失常是接受慢性输血的地中海贫血患者公认的并发症。心肌铁负荷、亚临床收缩功能障碍与临床事件之间的关系仍鲜为人知。

材料与方法

我们共研究了77名受试者,包括37名有反复输血史的地中海贫血患者(平均年龄:24.2±5.5岁),以及40名年龄和性别匹配的对照组(平均年龄:24±4.5岁)。检测了所有受试者的血清铁蛋白水平,并通过二维(2D)斑点追踪分析对左心室(LV)纵向、圆周和径向变形进行半自动定量。

结果

与对照组相比,地中海贫血患者的左心室质量指数显著更大,心肌变形更小(P<0.05),但左心室射血分数(LVEF)无显著差异(P=0.233)。血清铁蛋白水平与纵向(r=0.44,P=0.0078)和径向应变(r=-0.46,P=0.0051)之间存在显著线性相关性,纵向、圆周和径向应变预测临床事件的最佳截断值分别为-15.48%、-21.31%和26.67%。在中位802.5天的随访期间,发生了11例临床复合事件(HF发生率、室性快速心律失常和死亡)(N=9名受试者);与未发生复合事件的受试者相比,发生复合事件的受试者纵向和径向应变显著降低(均P<0.05)。在调整年龄、性别、血清铁蛋白水平和左心室质量指数后,纵向应变恶化仍然是临床事件和死亡的独立预测因素(HR:6.05,P=0.033)。

结论

有反复输血史的地中海贫血患者更易出现亚临床收缩功能障碍,这与血清铁蛋白水平进一步相关。此外,左心室心肌变形参数恶化在预测该人群的临床结局方面可能比传统指标发挥更独立的作用。

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