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扩张型心肌病患儿延迟钆增强的结果

Results of Late Gadolinium Enhancement in Children Affected by Dilated Cardiomyopathy.

作者信息

Muscogiuri Giuseppe, Ciliberti Paolo, Mastrodicasa Domenico, Chinali Marcello, Rinelli Gabriele, Santangelo Teresa Pia, Napolitano Carmela, Leonardi Benedetta, Secinaro Aurelio

机构信息

Department of Imaging, Bambino Gesù - Children's Hospital IRCCS, Rome, Italy; Department of Clinical and Molecular Medicine, University of Rome "Sapienza", Rome, Italy.

Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù - Children's Hospital IRCCS , Rome , Italy.

出版信息

Front Pediatr. 2017 Feb 6;5:13. doi: 10.3389/fped.2017.00013. eCollection 2017.

Abstract

BACKGROUND

Little is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM).

MATERIALS AND METHODS

We retrospectively evaluated 15 patients (8 ± 6 years, 6 males) with diagnosis of DCM who underwent cardiac magnetic resonance since 2014. All scans were performed with a 1.5 T system (Aera, Siemens). Study protocol included cine steady-state free precession sequences, followed by administration of 0.2 mmol/kg of gadolinium-based contrast agent. Inversion recovery Turbo Flash sequences, in the same position of cine images, were acquired 10-15 min after the injection of contrast agent, in order to assess the presence of LGE. The latter was considered positive with a signal intensity >6 SD from normal myocardial tissue. Indexed end-diastolic volume (EDVi) and end-systolic volume (ESVi), and left ventricle (LV) ejection fraction (EF) were calculated by using dedicated software on off-line workstation. Global longitudinal strain and diastolic function were evaluated by echocardiography. Clinical follow-up, including death, transplant, and listing for heart transplant [major adverse cardiac events (MACE)], were evaluated. Patients were divided into two different subgroups: negative (Group A) and positive (Group B) for presence of LGE. Statistical analysis was performed by using Mann-Whitney test ( < 0.05 considered as statistically significant).

RESULTS

Seven patients (47%) showed LGE. A global diffuse subendocardial pattern was evident in all patients presenting LGE (7/7, 100%). The following main LV indexes were observed in the two subgroups. Group A: EDVi = 96 ± 33 ml, ESVi = 56 ± 29 ml, LV EF = 45 ± 10%, global longitudinal strain = -16 ± 5%, ' ratio = 10 ± 3, MACE = 1. Group B: EDVi = 130 ± 60 ml, ESVi = 89 ± 43 ml, LV EF = 31 ± 6%, global longitudinal strain = -13 ± 4%, ' ratio = 9 ± 3, MACE = 3. There was no statistically significant difference between the two groups, in terms of EDVi (: 0.2), ESVi (: 0.2), and ' ratio (0.9), whereas a significant difference of LV EF, presence of significative mitral regurgitation, and global longitudinal strain were observed (respectively, : 0.03, : 0.009, and : 0.03).

CONCLUSION

In our population of children with DCM, LGE shows a global diffuse subendocardial pattern. Presence of LGE seems to play a role in these patients determining a worst global systolic function.

摘要

背景

关于晚期钆增强(LGE)在扩张型心肌病(DCM)患儿中的临床价值,人们了解甚少。

材料与方法

我们回顾性评估了自2014年以来接受心脏磁共振检查且诊断为DCM的15例患者(年龄8±6岁,男性6例)。所有扫描均使用1.5T系统(西门子Aera)进行。研究方案包括电影稳态自由进动序列,随后静脉注射0.2mmol/kg钆基造影剂。在注射造影剂10 - 15分钟后,在与电影图像相同位置采集反转恢复快速小角度激发序列,以评估LGE的存在情况。若信号强度比正常心肌组织高>6个标准差,则认为LGE为阳性。通过离线工作站上的专用软件计算舒张末期容积指数(EDVi)、收缩末期容积指数(ESVi)和左心室(LV)射血分数(EF)。通过超声心动图评估整体纵向应变和舒张功能。评估临床随访情况,包括死亡、移植以及列入心脏移植名单[主要不良心脏事件(MACE)]。患者根据LGE的有无分为两个不同亚组:阴性(A组)和阳性(B组)。采用Mann - Whitney检验进行统计分析(P<0.05被视为具有统计学意义)。

结果

7例患者(47%)显示有LGE。所有出现LGE的患者(7/7,100%)均呈现出整体弥漫性心内膜下模式。在两个亚组中观察到以下主要的左心室指标。A组:EDVi = 96±33ml,ESVi = 56±29ml,LV EF = 45±10%,整体纵向应变=-16±5%,E/A比值 = 10±3,MACE = 1。B组:EDVi = 130±60ml,ESVi = 89±43ml,LV EF = 31±6%,整体纵向应变=-13±4%,E/A比值 = 9±3,MACE = 3。两组在EDVi(P = 0.2)、ESVi(P = 0.2)和E/A比值(P = 0.9)方面无统计学显著差异,而在LV EF、显著二尖瓣反流的存在情况和整体纵向应变方面观察到显著差异(分别为P = 0.03、P = 0.009和P = 0.03)。

结论

在我们的DCM患儿群体中,LGE呈现出整体弥漫性心内膜下模式。LGE的存在似乎在这些患者中对整体收缩功能较差起到了一定作用。

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