Xu Q, Sun H X, Xie J S, Wang J L, Pei Q Y, Zhang X H
Department of Obstetrics and Gynecology, Peking University People's Hospital, Beijing 100044, China.
Zhonghua Fu Chan Ke Za Zhi. 2018 Mar 25;53(3):149-154. doi: 10.3760/cma.j.issn.0529-567X.2018.03.002.
To explore the clinical characteristics of interrupted of the inferior vena cava with azygous continuation and the prognosis. Retrospective analysis of 21 fetuses diagnosed with interrupted inferior vena cava with azygous continuation among 28 567 pregnant women who underwent routine ultrasound scan. The clinical data, ultrasonographic features, genetic information and prognosis were collected. Interrupted of the inferior vena cava with azygous continuation occurred in 21(0.07%, 21/28 567) of 28 567 patients. Three fetuses (14%, 3/21) complicated with heart and extracardiac malformations, including endocardiac cushion defect, single atrium and single ventricle, double superior vena cava, dextrocardia, asplenia syndrome, visceral heterotaxy, duodenal atresia; six fetuses (29%, 6/21) were associated with cardiac anomalies, such as hypoplastic left heart syndrome, double outlet right ventricle, pulmonary stenosis, ventricular septal defect, persistent left superior vena cava, endocardiac cushion defect and transposition of the great arteries; six cases (29%, 6/21) were only combined with extracardiac malformations, includingasplenia syndrome, visceral heterotaxy, duodenal atresia. Three fetuses (14%, 3/21) were nonorganic abnormalities included thickening of the right ventricle wall, fetal bradycardia, pericardial effusion, hydrops abdominis, increased peak systolic velocity/end diastolic velocity and single umbilical artery. Three fetuses (14%, 3/21) were isolated interrupted inferior vena cava with azygous continuation, but without other anomalies and 2 of them had normal fetal karyotype. Five cases (24%, 5/21) were successfully vaginal delivery, 1 case (5%, 1/21) had cesarean section. After 12-40 months follow-up, we didn't obeserve obviously abnormality, nor any chromosomal abnormality. Ten patients (48%, 10/21) opted for termination of the pregnancy and the autopsies were not done. Five cases (24%, 5/21) were lost to follow up. Interrupted inferior vena cava with azygous continuation are associated with cardiovascular and extracardiac anomalies, cardiac malformation and visceral heterotaxy are the most common anomalies. Visceral heterotaxy should be considered and fetal karyotype should be suggested. In the cases of isolated interrupted inferior vena cava with azygous continuation and normal karyotype, the outcome is favorable.
探讨下腔静脉中断并奇静脉延续的临床特征及预后。回顾性分析28567例行常规超声检查的孕妇中诊断为下腔静脉中断并奇静脉延续的21例胎儿。收集其临床资料、超声特征、基因信息及预后情况。28567例患者中，21例（0.07%，21/28567）发生下腔静脉中断并奇静脉延续。3例胎儿（14%，3/21）合并心脏及心外畸形，包括心内膜垫缺损、单心房、单心室、双上腔静脉、右位心、无脾综合征、内脏异位、十二指肠闭锁；6例胎儿（29%，6/21）合并心脏异常，如左心发育不全综合征、右心室双出口、肺动脉狭窄、室间隔缺损、永存左上腔静脉、心内膜垫缺损及大动脉转位；6例（29%，6/21）仅合并心外畸形，包括无脾综合征、内脏异位、十二指肠闭锁。3例胎儿（14%，3/21）为非器质性异常，包括右心室壁增厚、胎儿心动过缓、心包积液、腹腔积液、收缩期峰值流速/舒张末期流速增加及单脐动脉。3例胎儿（14%，3/21）为孤立性下腔静脉中断并奇静脉延续，无其他异常，其中2例胎儿染色体核型正常。5例（24%，5/21）成功经阴道分娩，1例（5%，1/21）行剖宫产。随访12 - 40个月后，未观察到明显异常，也无染色体异常。10例患者（48%，10/21）选择终止妊娠，未进行尸检。5例（24%，5/21）失访。下腔静脉中断并奇静脉延续与心血管及心外畸形相关，心脏畸形和内脏异位是最常见的畸形。应考虑内脏异位并建议进行胎儿染色体核型检查。对于孤立性下腔静脉中断并奇静脉延续且染色体核型正常的病例，预后良好。
