心肌声学造影指导经皮腔内室间隔心肌消融治疗梗阻性肥厚型心肌病1例

2019-12-06 任建丽 李兴升 朱叶锋 临床超声医学杂志

患者男,73岁,因活动后胸闷不适1个月入院。高血压病史5年,无冠状动脉粥样硬化性心脏病史。体格检查:神清,心界不大,心率68次/min,律齐,心前区可闻及Ⅲ~Ⅳ/6级收缩期喷射性杂音。家族史中无原发性心肌病患者。心电图检查:窦性心律,V1导联呈RS波形,V2-4导联T波倒置。心肌酶谱检查:α-L岩藻糖苷酶、α-羟丁酸脱氢酶、肌酸激酶稍高。

患者男,73岁,因活动后胸闷不适1个月入院。高血压病史5年,无冠状动脉粥样硬化性心脏病史。体格检查:神清,心界不大,心率68次/min,律齐,心前区可闻及Ⅲ~Ⅳ/6级收缩期喷射性杂音。家族史中无原发性心肌病患者。心电图检查:窦性心律,V1导联呈RS波形,V2-4导联T波倒置。心肌酶谱检查:α-L岩藻糖苷酶、α-羟丁酸脱氢酶、肌酸激酶稍高。
 
超声心动图检查:左房增大,左室流出道变窄,室间隔与左室壁呈非对称性增厚,室间隔基底段最厚处约20mm;CDFI示收缩期左室流出道呈蓝色为主的花色血流,频谱呈“匕首征”,峰值速度约345 cm/s,压差约48mmHg(1mmHg=0.133kPa),见图1A。超声心动图提示:非对称性梗阻性肥厚型心肌病。


图1连续多普勒测左室流出道压差。A:术前左室流出道压差为48mmHg;
 
拟行心肌声学造影指导经皮腔内室间隔心肌消融术,消毒,利多卡因局麻,穿刺左、右桡动脉及右股静脉,置入鞘管,经股静脉途径送入临时起搏器电极至右室心尖部;经左桡动脉送入5F多功能导管至左室心尖部,用于监测左室压力;再选择6FEBU3.5导管经头臂干至左冠状动脉开口处,用于检测主动脉压力和手术操作,测得初始压差约29mmHg,多巴酚丁胺(15μg·kg-1·min-1)激发后压差约127mmHg;连续多普勒示压差约163mmHg(图1B),故行梗阻性肥厚型心肌病室间隔消融指征明确。

图1B 多巴酚丁胺激发后左室流出道压差为163mmHg;
 
冠状动脉造影检查:左主干无狭窄,左前降支中段可见心肌桥,未见明显狭窄,左回旋支无狭窄。选择宝马导丝至间隔支靶血管远端,同轴整体支换型(OTW)球囊(1.5mm×15.0mm)沿导丝送入靶血管近中段,12atm(1atm=101.325kPa)加压,冠脉造影确定球囊位置及大小合适,拔出导丝,经OTW球囊中心腔注入2ml声诺维超声造影剂,超声造影示造影剂分布于肥厚的室间隔(图2),判断靶间隔支合适,然后撤出球囊,重新置入导丝,经微导管沿导丝将弹簧圈送入靶血管,造影显示位置合适,释放弹簧圈(图3A),超声心动图显示室间隔内弹簧圈位置(图3B),左室流出道压差显著降至14mmHg(图1C),撤出导丝;行冠脉造影显示靶间隔支消失,前降支血流良好,手术顺利。

图1C 术后左室流出道压差为14mmHg。

图2 超声造影示增厚的室间隔显影(箭头示)

图3 弹簧圈位置显示。A:冠状动脉造影显示弹簧圈位置;B:超声心动图显示弹簧圈位置(箭头示)。
 
讨论:
 
梗阻性肥厚型心肌病是以左室流出道梗阻为主要特征的心肌病,其主要病理生理及临床症状来自左室流出道梗阻,因此治疗的关键是降低左室流出道压差,减轻左室流出道梗阻。目前根据不同的病理特征,临床选择内科保守治疗、外科二尖瓣人工机械瓣置换术、外科左室流出道疏通术、起搏器植入术及经皮腔内室间隔心肌消融术等方法来缓解左室流出道梗阻。
 
经皮腔内室间隔心肌消融术的关键是寻找支配梗阻相关心肌的靶血管,通过向靶血管内注射无水酒精或放入弹簧圈,使靶血管闭塞,人为地造成小范围的心肌梗死,解除或减轻左室流出道的梗阻,降低左室流出道压差。因此,靶血管的准确选择成为室间隔心肌消融术的关键。因为室间隔是通过多条间隔支供血,并且间隔支相互交叠,个体差异较大,以往应用间隔支球囊试栓堵的方法判断靶血管并不可靠,而冠状动脉造影显示冠状动脉的微循环灌注比较困难,无法准确地显示间隔支所供应的心肌范围。
 
声诺维是一种超声造影剂,其具有红细胞的流变学特性,可以严格地按照血管走行分布,能清晰地勾勒相应血管床供应的心肌范围,由此可以半定量评价各间隔支血管供应的心肌范围,与冠状动脉造影结合可在术中准确地选择靶血管,保证消融部位及范围的定位准确。本病例即是应用选择性靶血管心肌声学造影结合冠状动脉造影确定靶血管,靶血管选择性心肌超声造影不仅能显示血管与心肌的匹配关系,还能显示间隔支靶血管血供的具体范围,能够定位消融部位和半定量其消融范围,在靶间隔支释放弹簧圈后使靶血管闭塞,人为地造成肥厚的室间隔心肌梗死,解除左室流出道的梗阻,本病例术后超声心动图随访显示肥厚的室间隔波幅减低,左室流出道压差恢复正常,提示该方法以最小的损伤获得最大的临床疗效。
 
原始出处:

任建丽,李兴升,朱叶锋,冉海涛,王志刚.心肌声学造影指导经皮腔内室间隔心肌消融治疗梗阻性肥厚型心肌病1例[J].临床超声医学杂志,2018(09):624+642.

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    2019-12-08 zhaojie88
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