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定期输血对非输血依赖型地中海贫血(NTDT)患儿的心脏保护作用:一项队列研究。

Cardio-protective effect of regular transfusion in children with non-transfusion dependent thalassemia (NTDT): A cohort study.

机构信息

Sultan Qaboos University Hospital.

Faculty of Medicine, Alexandria University.

出版信息

Acta Biomed. 2022 May 11;93(2):e2022022. doi: 10.23750/abm.v93i2.10736.

Abstract

BACKGROUND AND AIM OF THE WORK

Cardiac complications occur in patients with non-transfusion dependent thalassemia (NTDT). The study aimed to evaluate transfusion effect on systolic and diastolic cardiac function in young NTDT patients.  Methods:  Study design: Cohort study. Seventeen regularly-transfused patients with NTDT (12.5±5.3 years; group 1) and 15 none/minimally transfused patients (13.2±4.8 years; group 2) were followed up for 5 years and compared as regards their clinical parameters, echocardiographic and Tissue-Doppler-Imaging.

RESULTS

Group 2 patients had significantly higher peak late-diastolic velocity of the left-ventricular-inflow Doppler (Am). Mitral-valve A-wave duration/pulmonary-veins, A-wave duration-ratio and pulmonary-vein S/D velocities-ratio were larger in group 2 as well (p = < 0.01). The diameters of right and left outflow-tract were larger with a higher cardiac-index in patients of group 2. Systolic-function was similar in the 2 studied groups.

CONCLUSION

Diastolic function assessment revealed indicators of an abnormal relaxation of left-ventricle in non-transfused patients, which suggests a diastolic dysfunction. An increase in the diameter of the outflow-tract is likely attributed to high cardiac-output status in non-transfused NTDT patients as they have a higher cardiac index. Early start of regular transfusion for NTDT patients might prevent serious long-term cardiac complications.

摘要

背景与目的

非输血依赖型地中海贫血(NTDT)患者可发生心脏并发症。本研究旨在评估输血对年轻 NTDT 患者收缩和舒张心脏功能的影响。

方法

研究设计:队列研究。17 例定期输血的 NTDT 患者(12.5±5.3 岁;第 1 组)和 15 例非输血或最小输血患者(13.2±4.8 岁;第 2 组)接受了 5 年的随访,并对其临床参数、超声心动图和组织多普勒成像进行了比较。

结果

第 2 组患者的左心室流入多普勒(Am)峰值晚期舒张速度显著更高。二尖瓣 A 波持续时间/肺静脉、A 波持续时间比和肺静脉 S/D 速度比在第 2 组也更大(p < 0.01)。第 2 组患者的右和左流出道直径较大,心指数较高。两组患者的收缩功能相似。

结论

舒张功能评估显示非输血患者左心室舒张功能异常的指标,提示舒张功能障碍。非输血 NTDT 患者流出道直径增加可能归因于高心输出量状态,因为他们的心指数较高。早期开始对 NTDT 患者进行常规输血可能预防严重的长期心脏并发症。

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