Muscogiuri Giuseppe, Ciliberti Paolo, Mastrodicasa Domenico, Chinali Marcello, Rinelli Gabriele, Santangelo Teresa Pia, Napolitano Carmela, Leonardi Benedetta, Secinaro Aurelio
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.
Front Pediatr. 2017 Feb 6;5:13. doi: 10.3389/fped.2017.00013. eCollection 2017.
Little is known about the clinical value of late gadolinium enhancement (LGE), in children affected by dilated cardiomyopathy (DCM).
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).
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).
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.
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。
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)。
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)。
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。