超声心动图对儿童Shone综合征术前诊断及术后评价的临床价值及漏诊分析  

The clinical value of echocardiography in the preoperative diagnosis and postoperative evaluation of children with Shone's syndrome and the analysis of misdiagnosis

作　　者：莫莹[1] 王强[1] 李刚[1] 王霄芳[1] 丁文虹[1] MO Ying;WANG Qiang;LI Gang;WANG Xiaofang;DING Wenhong(Pediatric Cardiac Center,Beijing Anzhen Hospital,Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所小儿心脏科,100029

出　　处：《心肺血管病杂志》2024年第9期972-976,983,共6页Journal of Cardiovascular and Pulmonary Diseases

基　　金：北京市自然科学基金(7222044)。

摘　　要：目的:探讨超声心动图对Shone综合征的术前诊断、术后随访及手术效果评价的临床价值,并分析、总结漏诊原因以提高对此畸形的认识及诊断率。方法:对2014年1个月至2023年1个月间,我院儿童心脏中心收治并经手术证实的63例Shone综合征患者的超声心动图及临床资料进行回顾性分析,对比超声诊断及术中所见畸形情况。分别于术前、术后1个月、1年行超声心动图检查,测量二尖瓣、左心室流出道和主动脉弓降部峰值压差并进行统计学对比。结果:超声心动图诊断左心室流出道畸形漏诊率低于左心室流入道畸形,且瓣内环漏诊率最高,主动脉缩窄、发育不良、主动脉瓣狭窄无漏诊,余畸形漏诊率低于20%。对比患儿术后二尖瓣、左心室流出道和主动脉弓降部峰值压差显示术后较术前均明显减低,差异均有统计学意义(P <0.01)。术后1个月和1年左心各水平压差差异无统计学意义(P>0.05)。结论:超声心动图可早期准确诊断Shone综合征及心内合并畸形,并为进一步外科手术治疗及术后随访提供可靠依据,具有重要临床价值。Objective:To investigate the clinical value of echocardiography in the preoperative diagnosis,postoperative follow-up and evaluation of surgical effect of Shone's syndrome,and to summarize the reasons of misdiagnosis to improve the understanding and diagnosis rate of this malformation.Methods:From January 2014 to January 2023,the echocardiography and clinical data of 63 patients with Shone's syndrome and confirmed by surgery were retrospectively analyzed,and the abnormalities diagnosed by ultrasound and observed during surgery were compared.Echocardiography was performed before and 1 month and 1 year after surgery,and the peak pressure difference between mitral valve,left ventricular outflow tract and descending aortic arch was measured and statistically compared.Results:The missed diagnosis rate of left ventricular outflow tract malformation by echocardiography was lower than that of left ventricular inflow tract malformation,and the missed rate of intra-valvular ring was the highest,There was no missed diagnosis of coarctation,dysplasia,and aortic stenosis,and the missed diagnosis rate of residual malformations was less than 20%.The postoperative pressure was significantly lower than that before operation,and the differences were statistically significant(all P<0.01).There was no significant difference at 1 month and 1 year after surgery(P>0.05).Conclusion:Echocardiography can provide early and accurate diagnosis of Shone's syndrome,and provide a reliable basis for further surgical treatment and postoperative follow-up,which has important clinical value.

关 键 词：先天性心脏病 超声心动图 Shone综合征 

分 类 号：R54[医药卫生—心血管疾病]