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目的探讨终末期致心律不齐性右室型心肌病(ARVC)组织学特点和 MRI 特征。方法9例患者接受心脏移植,移植后离体心脏行组织病理学检查。7例移植前行 MR 扫描。结果病理学检查显示所有心脏双室受累,右心室腔显著扩张7例,大致正常2例。右心室壁均显示严重透壁性肌肉丧失,其中3例几乎完全被脂肪组织替代,6例几乎完全被纤维脂肪组织替代。左心室中重度扩张8例、轻度扩张1例。左心室游离壁受累者7例,室间隔和左心室游离壁同时受累者2例。5例以脂肪细胞浸润为主伴小灶性纤维化;4例以弥漫性纤维化为主,伴灶性脂肪细胞浸润。7例 MRI 显示左心室射血分数平均(21.66±7.05)%,左心室轻度扩张3例,中度扩张2例,高度扩张2例。右心室腔明显扩大、壁薄者5例,其中3例可见线状高信号脂肪浸润;其余2例右心室形态、大小及信号均无明显异常,仅突出地表现为左心室受累。2例显示心外膜下脂肪信号浸润,选择性累及左心室心尖和侧、后壁;3例左心室节段性变薄伴运动功能丧失分别累及室间隔、心尖和侧后壁;4例左心室游离壁变薄,厚度不足5/mm。5例心肌灌注延迟显像均表现为不同程度的增强,左心室侧后壁强化者4例,其中透壁性和心外膜下各2例;室间隔肌壁间强化者2例;左心室心尖部强化者2例,灶性和透壁性各1例。4例患者右心室壁亦可见透壁性增强,其中累及右心室游离壁者2例,累及右心室心尖和后壁各1例。结论 ARVC 合并左心室受累是该组患者的特点,MRI 不仅能够准确地反映 ARVC 继发性的心室扩张及室壁运动的节段性变化,而且能检出心室壁的脂肪浸润以及纤维化等,因此能够在一定程度上反映生理状态下心脏组织学特征。
Objective To investigate the histological features and MRI features of end-stage arrhythmia-induced right ventricular cardiomyopathy (ARVC). Methods Nine patients underwent heart transplantation. Histopathological examination was performed on the isolated heart after transplantation. Seven patients underwent MR scan before transplantation. Results Pathological examination showed that all hearts involved in double-chamber, right ventricular cavity was significantly expanded in 7 cases, roughly normal in 2 cases. Right ventricular wall showed severe transmural muscular loss, of which 3 cases were almost completely replaced by adipose tissue and 6 cases were almost completely replaced by fibrous adipose tissue. Severe left ventricular dilatation in 8 cases, 1 case of mild dilatation. Left ventricular free wall involvement in 7 cases, both ventricular septal and left ventricular free wall involvement in 2 cases. 5 cases of infiltration of fat cells associated with focal fibrosis; 4 cases of diffuse fibrosis, with focal fat cell infiltration. Seven cases of MRI showed left ventricular ejection fraction average (21.66 ± 7.05)%, mild left ventricular dilatation in 3 cases, moderate expansion in 2 cases and high expansion in 2 cases. Right ventricular cavity was significantly enlarged, thin wall in 5 cases, of which 3 cases showed linear hyperintense fat infiltration; the other two cases of right ventricular shape, size and signal were no abnormalities, only prominent for the left ventricular involvement. 2 cases showed subepicardial fat signal infiltration, selectively involving the left ventricular apex and the posterior wall; 3 cases of left ventricular segmental thinning with loss of motor function involved ventricular septum, apical and posterior wall; 4 cases of left Ventricular free wall thinning, the thickness of less than 5 / mm. 5 cases of delayed myocardial perfusion imaging showed varying degrees of enhancement, left ventricular enhanced posterior wall in 4 cases, including transmural and subepicinal in 2 cases; interventricular septum enhancement in 2 cases; left ventricular Apex intensification in 2 cases, 1 case of focal and transmural. 4 cases of right ventricular wall can also be seen transmural enhancement, which involved the right ventricular free wall in 2 cases, involving the right apex of the apex and the posterior wall in 1 case. Conclusion ARVC combined with left ventricular involvement is the characteristic of this group of patients. MRI can not only accurately reflect the secondary changes of ventricular dilatation and wall motion of ARVC, but also detect the infiltration of fat and fibrosis of ventricular wall, So to some extent to reflect the physiological state of cardiac histology.