Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?
Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?
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DOI：
10.1007/s10554-008-9352-y
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发表时间：
2009-01
影响因子：
2.1
通讯作者：
Gottliebson, William M.
中科院分区：
文献类型：
--
作者：
Puchalski, Michael D.;Williams, Richard V.;Askovich, Bojana;Sower, C. Todd;Hor, Kan H.;Su, Jason T.;Pack, Nathan;Dibella, Edward;Gottliebson, William M.
进行性心肌病是杜氏肌营养不良症（DMD）的常见死因，可能继发于心肌纤维化。左室后基底壁和左室外侧游离壁是心肌纤维化的起始部位。本研究的目的是评估心脏磁共振成像（CMRI），利用晚期钆增强（LGE），是否可以识别纤维化的心肌的选择性领域，并评估的存在和程度的关系，左心室功能的纤维化。对Primary Children's Medical Center和辛辛那提儿童医院医学中心的心脏病学数据库进行了审查，以确定在过去2年内接受过CMRI的DMD患者。记录年龄、左室射血分数、左室质量、LGE的存在和位置。使用MASS（Medis，Inc.）来计算射血分数和质量获取LGE图像，当阳性时，在所有切片上对晚期钆增强区域进行手动和定制的计算机辅助尺寸测量。左室射血分数> 54%为心功能正常。共有74名DMD患者有完整的数据集（中位年龄13.7岁，范围7.7 - 26.4）。24例患者（32%）的LGE累及心外膜下分布的LV后基底区。累及较多的患者已扩散至下壁和左侧游离壁，并进行性透壁纤维替代。室间隔和右心室相对保留。有LGE的患者明显比无LGE的患者年龄大（平均年龄16.4岁对12.9岁，p<0.001）。LGE与BSA校正的LV质量、LV舒张末期容积、LV收缩末期容积和RV收缩末期容积呈正相关，但与LV（p<0.001）和RV（p = 0.004）的射血分数呈负相关。通过CMRI的LGE能够检测DMD患者心肌选择性区域的纤维化。不利的LV重构，伴随相应的射血分数降低，与LGE的存在相关。系列研究是必要的，以确定是否LGE之前的功能下降，如果早期的医疗管理是有用的，以防止进展，一旦LGE的记录。
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function. The cardiology databases at Primary Children's Medical Center and Cincinnati Children's Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, manual and customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%. A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7 − 26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age16.4 years vs 12.9 years, p<0.001). LGE was positively associated with BSA-adjusted LV mass, LV end-diastolic volume, LV end-systolic volume, and RV end-systolic volume but inversely correlated with ejection fraction of the LV (p<0.001) and RV (p = 0.004). LGE by CMRI is able to detect fibrosis in selective regions of myocardium in patients with DMD. Unfavorable LV remodeling, with a corresponding decreased ejection fraction, is associated with the presence of LGE. Serial studies are warranted to determine if LGE precedes a decrease in function, and if early medical management is useful in preventing progression once LGE is documented.
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