超声诊断复杂单心室畸形1例

2020-02-24 王豪 宋嫣 汪前洲 临床超声医学杂志

患儿女,4岁零5个月,半岁时外院诊断为复杂先天性心脏病,未进一步治疗。我院体格检查:体温36.5℃,脉搏109次/min,呼吸20次/min,血压98/62 mm Hg(1 mm Hg=0.133 kPa)。口唇青紫,杵状指及喜蹲踞现象;无咯血、晕厥史、无长期高热史;胸骨左缘第二肋间及三至四肋间均闻及3/6级收缩期杂音;发育尚正常,营养中等,神志清楚。

患儿女,4岁零5个月,半岁时外院诊断为复杂先天性心脏病,未进一步治疗。我院体格检查:体温36.5℃,脉搏109次/min,呼吸20次/min,血压98/62 mm Hg(1 mm Hg=0.133 kPa)。口唇青紫,杵状指及喜蹲踞现象;无咯血、晕厥史、无长期高热史;胸骨左缘第二肋间及三至四肋间均闻及3/6级收缩期杂音;发育尚正常,营养中等,神志清楚。
 
超声心动图检查:心脏位于左侧胸腔,心房正位,心室腔内未探及明显室间隔回声,呈单一心室结构(图1A),主腔位于后,残腔位于前,主动脉位于右前,肺动脉位于左后,均发自残腔,主动脉发育尚可,室间隔上端回声中断33 mm;CDFI:室水平可见双向分流信号,以左向右为主(图1B)。房间隔中央回声中断,宽约23 mm。三尖瓣叶呈闭锁状,代之以回声增强的组织,未探及明确的三尖瓣叶活动。



图1 四腔心切面观。A:未见室间隔回声,单心室,三尖瓣闭锁,呈长条状强回声,无启闭;LV:左室;LA:左房;RA:右房;B:CDFI示主腔为主向残腔过隔血流,二尖瓣口可见少量反流,三尖瓣未见启闭运动
 
4支肺静脉均未回流至左房,左房后方汇入大小约55.0 mm×8.6 mm共同腔经垂直静脉上行汇入右上腔静脉(图2)。主动脉弓降部未见异常,主动脉内径21 mm,主肺动脉内径14 mm,左、右肺动脉内径分别为7 mm和6 mm。收缩期右室流出道及肺动脉瓣前向血流速度加快,测得前向流速约3.8 m/s,峰值压差约58 mm Hg(图3)。


图2 胸骨上窝主动脉弓切面观。共同肺静脉(PV)异位引流汇入右上腔静脉


图3 肺动脉狭窄频谱图。右室流出道及主肺动脉内测得前向高速动脉频谱
 
右位上腔静脉及下腔静脉于正常位入右房,胸骨上窝可探及宽约10 mm的左上腔静脉汇入左房。超声提示:先天性心脏病,考虑单心室(A型),大动脉转位,三尖瓣闭锁,肺动脉狭窄,完全型肺静脉异位引流(心上型),房间隔缺损(中央型),永存左上腔静脉、细小侧支血管形成,建议进一步检查。患者后于外院行手术治疗,证实我院超声诊断结果准确。
 
讨论:
 
单心室是一种少见的先天性紫绀型心脏病,常合并其他心血管畸形,术前准确诊断对选择手术方式和评估预后均有重要意义。单心室按形态结构可分为4型:A型,左心室主腔结构为主;B型,右心室主腔结构为主;C型,室间隔缺如,左、右心室结构组成室腔;D型,不定型。该病在行超声检查时应注意:附属心室位置、功能,房室瓣数目及功能状态,大血管有无转位及主要心室或附属心室连接关系,有无右室流出道梗阻及肺动脉闭锁等。
 
本例患者属于A型,单心室合并大动脉转位、完全型肺静脉异位引流及三尖瓣闭锁等多种复杂心血管畸形,较为少见。术前超声心动图准确诊断异位肺静脉类型及是否合并其他复杂心血管疾病,对手术方案的制定至关重要。单心室合并复杂心血管畸形手术治疗方式包括:单侧双向Glenn术、双侧双向Glenn术、改良Fontan手术等,尽早行手术治疗可有效改善复杂紫绀先天性心脏病患儿的氧饱和度,降低心室容量负荷,改善其预后情况。
 
原始出处:

王豪,宋嫣,汪前洲.超声诊断复杂单心室畸形1例[J].临床超声医学杂志,2019,21(01):80.

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    2020-02-26 wushaoling
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