Abstract
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。
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