14例单侧肺动脉异常起源于升主动脉胎儿的产前诊断及生后随访  

Prenatal diagnosis and pregnancy outcome of anomalous origin of one pulmonary artery branch from the aorta:analysis of 14 cases

作　　者：梁小碧 李珊 王红英 张蕊 李健茹 Xiaobi Liang;Shan Li;Hongying Wang;Rui Zhang;Jianru Li(Heart Center,Guangzhou Women and Children's Medical Center,Guangzhou 510623,China;Department of Ultrasonography,Guangzhou Women and Children's Medical Center,Guangzhou 510623,China)

机构地区：[1]广州市妇女儿童医疗中心心脏中心,广州510623 [2]广州市妇女儿童医疗中心超声科,广州510623

出　　处：《中华围产医学杂志》2023年第4期325-330,共6页Chinese Journal of Perinatal Medicine

基　　金：广州市科技计划项目(202102010315)。

摘　　要：目的分析单侧肺动脉异常起源于升主动脉(anomalous origin of one pulmonary artery branch from the aorta,AOPA)胎儿的产前诊断、分型及预后情况。方法回顾性选择2016年6月至2022年8月在广州市妇女儿童医疗中心产前诊断AOPA病例14例,对其产前及生后超声心动图特点、生后诊断、手术治疗及随访等情况进行描述性分析。结果14例AOPA胎儿中,7例为近端型AOPA(其中3例为孤立性AOPA,3例合并Berry综合征,1例合并主动脉弓离断、主肺动脉间隔缺损和室间隔缺损),7例为孤立性远端型AOPA。7例近端型AOPA胎儿中,2例引产终止妊娠,5例产前与生后诊断相符,活产儿均行手术治疗,术后进行了密切的随访,预后良好。7例远端型AOPA胎儿中,1例引产终止妊娠,6例活产儿中有2例最初诊断为AOPA,随访后因动脉导管变细或关闭更正诊断为单侧肺动脉缺如(unilateral absence of pulmonary artery,UAPA),另4例生后直接诊断为UAPA;活产儿均行手术治疗,术后密切随访,预后良好。结论产前应尽量准确诊断AOPA并分型,建议远端型AOPA生后诊断为UAPA,并按UAPA处理,两者在生后应适时行手术治疗,预后良好。Objective To summarize the prenatal diagnosis features,classification and pregnancy outcome of anomalous origin of one pulmonary artery branch from the aorta(AOPA).Methods This study involved 14 cases who were prenatally diagnosed with AOPA in Guangzhou Women and Children's Medical Center between June 2016 and August 2022.Prenatal and postnatal echocardiographic features,postpartum diagnosis,surgical treatment and pregnancy outcome in these cases were summarized and analyzed by descriptive analysis.Results Out of the 14 fetuses,there were seven fetuses with proximal-type AOPA(including three isolated AOPA,three Berry syndrome and one with interruption of the aortic arch,aorticopulmonary septal defect and ventricular septal defect)and another seven with isolated distal-type of AOPA.Among the seven cases of proximal-type AOPA,two were terminated and five were born alive.The postpartum diagnosis was consistent with the prenatal diagnosis in the five babies who later underwent surgical treatment with good outcomes.Among the seven cases of distal-type AOPA,one was terminated;two were initially diagnosed as AOPA in the neonatal period but then as unilateral absence of pulmonary artery(UAPA)due to tapering or closure of the ductus arteriosus during follow-up;the other four were confirmed with UAPA after delivery.All of the six neonates underwent surgical treatment with good outcomes.Conclusions Prenatal diagnosis and classification of AOPA should be as accurate as possible.It is recommended that the distal-type of AOPA could be diagnosed as UAPA after delivery and treated according to UAPA.Both kinds of patients should be treated with surgery timely after delivery to ensure a good prognosis.

关 键 词：肺动脉 主动脉 血管畸形 产前诊断 产后期 随访研究 

分 类 号：R714.5[医药卫生—妇产科学]