CMR scarring in a patient with hypertrophic cardiomyopathy correlates well with histological findings of fibrosis

CMR scarring in a patient with hypertrophic cardiomyopathy correlates well with histological findings of fibrosis
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DOI:
10.1093/eurheartj/ehi518
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发表时间:
2005-11-01
影响因子:
39.3
通讯作者:
Borggrefe, M
Borggrefe, M
中科院分区:
医学1区
文献类型:
--
作者:
Papavassiliu, T;Schnabel, P;Borggrefe, M

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1例38岁女性肥厚型心肌病并充血性心力衰竭患者接受晚期Gd增强心血管磁共振检查。Cine CMR(面板A)显示左心室大小正常(左心室舒张末内径45 mm),左心室收缩功能中度降低(射血分数40%)。最大壁厚16 mm,双侧心房严重扩张。在应用0.2 mmol/kg体重的Gd-DTPA 15分钟后,使用T1加权倒置恢复序列,左室前间隔、下间隔和前壁(B组)显示广泛的心肌强化区域。在住院期间,患者出现恶性室性心律失常,并在CMR扫描后30天接受了紧急心脏移植。移植的心脏进行了组织学检查,以确定纤维化和紊乱的程度。对移植的心脏进行的肉眼检查显示有大量的疤痕。显微镜下可见肌原纤维错乱和疤痕。这些表现可作为肥厚型心肌病的诊断依据。Gd强化区域与肉眼可见的苍白心肌区域(Panel C)和纤维化区(Panel D)有很好的相关性。面板A使用Cine CMR获得体内舒张期长轴电影的增强前图像。B组。增强后图像显示活体心肌高度强化。大面积心肌中晚期Gd强化的前壁(箭头)。C组:移植心脏相应大体标本,肉眼可见清晰的心肌中部苍白心肌,与B组晚期Gd强化的分布相似。
A 38-year-old female patient with hypertrophic cardiomyopathy and congestive heart failure underwent late gadolinium enhancement cardiovascular magnetic resonance (CMR). Cine CMR (Panel A) revealed normal dimensions of the left ventricle (left ventricular end-diastolic diameter 45mm) with moderately reduced left ventricular systolic function (ejection fraction 40%). The maximum wall thickness was 16 mm and there was severe dilation of both atria. Using a T1-weighted inversionrecovery sequence 15 min after the application of 0.2 mmol/kg body weight gadolinium-DTPA, the anteroseptal, inferoseptal, and the anterior walls (Panel B) of the left ventricle showed extensive regions of myocardial enhancement. During her hospital course, the patient developed malignant ventricular arrhythmias and underwent urgent heart transplantation 30 days after the CMR scan. The explanted heart was examined histologically for the extent of fibrosis and disarray. The macroscopic examination of the explanted heart showed massive scarring. Microscopy showed areas of myofibril disarray and scars. These findings were diagnostic of HCM. Regions of gadolinium enhancement correlate well with regions of macroscopic pale myocardium (Panel C) and regions of fibrosis (Panel D). Panel A. Pre-contrast diastolic long-axis cine image in vivo obtained with cine CMR. Panel B. Post-contrast image demonstrating myocardial hyperenhancement in vivo. There is a large area of mid-myocardial late gadolinium enhancement of the anterior wall (arrows). Panel C. Corresponding gross specimen from the explanted heart with well-defined regions of macroscopic mid-myocardial pale myocardium similar to the distribution of late gadolinium enhancement in Panel B.