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目的:探讨单侧肺发育不良合并心血管畸形的产前超声心动图特征。方法:回顾性分析2016年4月至2019年12月经北京安贞医院产前超声心动图诊断的单侧肺发育不良合并心血管畸形(不合并其他心外畸形)胎儿的超声心动图特点，总结其超声心动图特征及鉴别要点。结果:共诊断8例胎儿，其中累及右肺7例、左肺1例，心血管畸形中5例“弯刀”综合征(SS)，2例室间隔缺损合并肺动脉闭锁(PA/VSD)(肺动脉肌性闭锁)，1例左肺动脉异常起源于升主动脉(AOLPA)。5例SS患儿均出生并手术治疗，2例PA/VSD胎儿均引产，AOLPA胎儿出生后2 h死亡。单侧肺发育不良合并心血管畸形超声特征：①心脏向肺脏发育不良侧移位；②发育不良侧肺动脉分支发育细；③双侧肺组织回声正常并且一致；④发育不良侧肺面积小；⑤右侧肺发育不良多见。结论:单侧肺发育不良合并心血管畸形首要的超声特征为心脏移位，下腔静脉的冠状和矢状切面是诊断SS的重要切面，该病预后与合并心血管畸形的严重程度密切相关。“,”Objective:To explore the prenatal echocardiographic features of cardiovascular malformations with unilateral pulmonary hypoplasia.Methods:Between April 2016 to December 2019, fetal echocardiographic characteristics were analyzed retrospectively from fetuses with prenatal diagnosis cardiovascular malformations with unilateral pulmonary hypoplasia (without other extracardial malformations) at the Pediatric Cardiovascular Center, Beijing Anzhen Hospital, the main points of its identification were summarized.Results:A total of 8 fetuses were diagnosed, including 7 cases with right pulmonary hypoplasia and 1 case with left pulmonary hypoplasia. In 8 fetuses, the cardiovascular malformation included 5 cases of scimitar syndrome, 2 cases of pulmonary atresia with ventricular septal defect (PA/VSD) (pulmonary artery muscular atresia) and 1 case of anomalous origin of left pulmonary artery from the aorta (AOLPA). All cases with scimitar syndrome were born and treated by surgery, 2 cases with PA/VSD were induced labor, and the AOLPA fetus died 2 hours after birth. The shared characteristic sonographic findings including: ①the cardiac axis was deviated and the heart moved to the dysplastic side of the lungs; ②the pulmonary artery branches on the dysplastic side were hypoplastic; ③the echo of both lung tissues was consistent and normal; ④the lung area on the hypoplasia side was small; ⑤right lung hypoplasia was more common.Conclusions:The primary sonographic characteristic of cardiovascular malformation with unilateral pulmonary hypoplasia is cardiac malposition. The coronal and sagittal views of inferior vena cava are very important for diagnosing scimitar syndrome. The prognosis is closely related to the severity of the associated vascular malformations.