2017 年第 12 期 第 12 卷

产前超声心动图对胎儿主肺动脉间隔缺损的诊断价值

Value of prenatal echocardiography in diagnosis of fetal aorto-pulmonary septal defect

作者：李文秀耿斌吴江杨爽

英文作者：Li Wenxiu Geng Bin Wu Jiang Yang Shuang

单位：100029首都医科大学附属北京安贞医院小儿心脏中心北京市心肺血管疾病研究所    

英文单位：Pediatric Cardiac Center Beijing Anzhen Hospital Capital Medical University Beijing Institute of Heart Lung and Blood Vessel Diseases Beijing 100029 China

关键词：主肺动脉间隔缺损；胎儿超声心动图；Berry综合征；产前诊断

英文关键词：Aorto-pulmonaryseptaldefect；Fetalechocardiography；Berrysyndrome；Prenataldiagnosis

• 摘要：

• 目的    探讨主肺动脉间隔缺损（APSD）胎儿的产前超声心动图特点，以提高对本病的产前诊断准确率。方法    回顾性分析2014年1月至2016年12月在首都医科大学附属北京安贞医院小儿心脏中心经产前超声心动图及手术证实的8例APSD的产前超声心动图检查结果，总结超声心动图特征。结果    8例APSD胎儿中，Ⅱ型5例，Ⅲ型3例,合并Berry综合征2例，合并室间隔缺损1例，合并主动脉弓离断2例,合并右位主动脉弓2例，所有病例均不合并其他心外畸形。8例胎儿中，引产１例（Berry综合征），出生的7例中，6例行外科手术治疗，术后均恢复良好，1例Berry综合征患儿出生后第63天因反复肺炎、心力衰竭死亡，该例患儿未行手术治疗。APSD胎儿超声心动图特征为：①主动脉与肺动脉双流出道冠状切面、主肺动脉间隔短轴切面、三血管及三血管气管切面可显示肺动脉与升主动脉之间间隔回声缺失；②肺动脉与主动脉在肺动脉分叉及气管前融合；③升主动脉内径增宽；④除外Berry综合征，多合并动脉导管缺如；⑤彩色多普勒显示肺动脉与主动脉之间双向分流，无动脉导管向主动脉分流。结论    产前超声心动图可对APSD做出准确诊断，本病预后与手术时间及合并的心内畸形相关

• Objective    To explore prenatal echocardiographic features of aorto-pulmonary septal defect(APSD). Methods    Prenatal echocardiography images of 8 cases of fetal APSD confirmed by echocardiographic and operative results in Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University from January 2014 to December 2016 were retrospectively reviewed. Echocardiographic features of APSD were analyzed. Results    In 8 fetuses with APSD, there were 5 cases of type Ⅱ and 3 cases of type Ⅲ; 2 cases were complicated with Berry syndrome; 1 case had ventricular septal defect; 2 cases had interruption of aortic arch; 2 cases had right aortic arch; no extracardiac abnormality was observed. One case with Berry syndrome had induced abortion; 6 infants had surgical treatments after birth and recovered well; the other case with Berry syndrome died of recurrent pneumonia and heart failure 63 d after birth without surgical treatments. Characteristics of APSD fetal echocardiogram： ①Coronal view of double outflow tract of aorta and pulmonary artery, short-axis view of aorto-pulmonary septum, three-vessel view, three vessels and trachea view showed a communication between pulmonary artery trunk and ascending aorta. ②The communication between pulmonary artery and aorta was located before pulmonary artery bifurcation. ③The diameter of ascending aorta was wide. ④Except Berry syndrome, absence of ductus arteriosus was commonly seen. ⑤Color Doppler could show a bidirectional shunt between pulmonary artery trunk and ascending aorta and there was no shunt between ductus arteriosus and ascending aorta. Conclusion    Prenatal echocardiography can provide accurate information for diagnosis of fetal APSD, the prognosis of which depends on operation timing and nature of associated cardiac anomalies.