先天性原发隔异位型肺静脉异位引流的超声心动图诊断

Echocardioimagedata diagnosis of anomalous pulmonary venous connection caused by congenital malposition of the septum primum

目的分析原发隔异位(MSP)型肺静脉异位引流(APVC)的超声心动图诊断特点,以期为临床手术提供更多的信息。方法回顾性收集2015年1月至2022年4月在浙江大学医学院附属儿童医院经心脏外科手术或CTA证实的21例MSP型APVC患儿的临床资料及超声心动图表现,分析纳入患儿的一般资料、合并畸形、超声心动图特征、误诊情况及外科手术和随访情况。结果21例MSP型APVC患儿中完全性肺静脉异位引流(TAPVC)8例、部分性肺静脉异位引流(PAPVC)13例;1例合并多脾综合征。超声心动图表现为肺静脉均开口于解剖左心房的后壁,继发隔发育不良或缺失,原发隔不同程度的偏移导致部分或全部的肺静脉血液引流至解剖右心房。超声首诊正确诊断18例(18/21,85.7%),误诊3例,分别为:1例MSP型TAPVC误诊为三房心合并PAPVC,1例MSP型PAPVC误诊为TAPVC合并继发孔房间隔缺损(ASD),1例MSP型PAPVC误诊为继发孔ASD。20例患儿经心脏外科手术治疗,术后随访4~69个月(平均34个月),所有患儿均预后良好。结论超声心动图能正确诊断MSP型APVC及其合并的心内畸形,有助于手术方式的合理选择,提高手术治疗成功率。

Objective To analyze the echocardioimagedata features of anomalous pulmonary venous connection(APVC)caused by malposition of the septum primum(MSP)in order to provide more information for clinical surgical treatment of this disease.Methods The clinical data and echocardioimagedata manifestations of 21 patients with APVC caused by MSP confirmed by cardiac surgery or computed tomography angiography at the Children's Hospital of Zhejiang University School of Medicine between January 2015 and April 2022 were retrospectively collected,and the general information,comorbidities,echocardioimagedata features,misdiagnosis,surgical procedures,and follow-up data of the included children were analyzed.Results Among the 21 patients included,8 had total anomalous and 13 had partial APVC directly to the right atrium;1 case was complicated with polysplenia syndrome.The absent of the superior limbic band of the septum secundum was revealed by echocardiography.The four pulmonary veins were connected with the posterior wall of the anatomical left atrium,which became totally or partially incorporated into the right atrium because of leftward displacement of the septum primum.Eighteen cases(18/21,85.7%)were correctly diagnosed by echocardiography.Three cases were misdiagnosed as cor triatriatum combined with partial APVC,total APVC combined with ostium secundum atrial septal defects(ASD),and ostium secundum ASD,respectively.Twenty patients were treated by cardiac surgery.The follow-up period ranged from 4~69 months(mean,34 months)after the operation,and all patients were alive and doing well.Conclusion Echocardiography can correctly diagnose APVC caused by MSP and other intracardiac malformations,which is helpful for the rational selection of surgical methods and the improvement of the success rate of surgical treatment.