单中心完全性肺静脉异位引流外科手术患者产前诊断情况及早期效果分析:来自22年间626例患者的分析

Current situation and effect of prenatal diagnosis for total anomalous pulmonary venous connection:a single analysis of 626 patients in 22 years

目的 分析完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的产前诊断现状,并探讨其对治疗效果的影响。方法 通过双向队列连续收集1999年2月到2020年12月22年间在广东省人民医院单中心行外科矫治术的626例TAPVC患者术前、术后的临床资料,比较产前诊断和产后诊断患者的临床状况以及手术预后情况。结果 626例TAPVC患者中,通过胎儿心脏超声诊断为心脏异常的共23例,产前诊断为3.67%,其中12例产前明确为TAPVC,产前诊断分型与产后符合率为83.33%。总体手术死亡率为4.80%。产前诊断有胎儿先天性心脏病(先心病)的患者中有9例以孕母转运(39.13%)的方式收住本院。通过倾向性评分匹配法,校正了术前梗阻和手术年龄后,与产后诊断组比较,产前诊断组选择具有儿科心脏专科的医院出生的患儿比例较高(60.87%vs. 8.70%,P<0.001),而且术后病死率明显降低(8.70%vs. 43.48%,P=0.007),差异有统计学意义。结论 TAPVC产前诊断率仍然较低,但产前诊断分型符合率较高。本中心TAPVC总体手术死亡率较低。产前诊断可促使先心病胎儿进入产前产后一体化诊疗模式,有利于选择孕母转诊方式,选择具有儿科心脏专科的医院分娩,降低患儿的术后住院病死率。

Objectives To investigate the situation of prenatal diagnosis for total anomalous pulmonary venous connection(TAPVC),and explore the effect of prenatal diagnosis by comparing the clinical data of patients with and without prenatal diagnosis.Methods TAPVC in Guangdong Provincial People′s Hospital were enrolled in this study during 22 years. Diagnostic and clinical information were extracted from the medical record. Differences of clinical data between the two groups with or without prenatal diagnosis were compared.Results Of the 626 cases with TAPVC,23(3.67%)was diagnosed with congenital heart disease(CHD)by fetal echocardiography. Among them,12 patients was diagnosed prenatally with TAPVC.Among the 12 cases,the consistency rate of prenatal and postnatal diagnosis of the subtype was 83.33%. The overall operative mortality was 4.80%. Among the 23 cases with prenatal diagnosis,9 cases(39.13%)whose mothers transferred to our hospital during pregnancy,gave birth to the baby and had them operated during hospital stay. After adjusting for preoperative obstruction and surgery age by propensity score matching(PSM),compared to the patients confirmed after birth,patients with prenatal diagnosis were more likely to be born in a hospital with CHD treatment expertise(60.87%vs. 8.70%,P<0.001),and had lower postoperative mortality(8.70% vs. 43.48%,P=0.007).Conclusions The prenatal diagnosis rate of TAPVC is still very low. However,the consistency rate of the TAPVC subtype is favorably high. The postoperative mortality for TAPVC is very low in our institute. Prenatal diagnosis can lead pregnant women and fetuses into the integrated pattern of prenatal diagnosis and postnatal treatment of CHD,which can help the family to choose the optimized transferred pattern,and reduce the postoperative mortality.