胎儿肺动脉瓣缺如综合征产前超声诊断分析

Prenatal ultrasonographic diagnosis of fetal absent pulmonary valve syndrome

目的总结胎儿肺动脉瓣缺如综合征产前超声图像特征。方法对2010年1月至2014年1月四川大学华西第二医院产前超声诊断并经尸解证实的5例肺动脉瓣缺如综合征胎儿超声心动图表现进行总结分析,观察胎儿心脏四腔观(4CH)、左心室流出道观(LVOT)、右心室流出道观(RVOT)、三血管气管观(3VT)各个常规切面观二维超声、彩色多普勒血流成像(CDFI)及脉冲多普勒血流流速曲线特征。结果 5例肺动脉瓣缺如综合征胎儿中3例超声心动图表现为典型的肺动脉瓣缺如综合征征象:(1)肺动脉主干呈瘤样扩张且瓣环处狭窄。(2)在心脏搏动周期内,肺动脉内见红蓝交替的往返血流信号。(3)频谱多普勒示肺动脉内呈双期双向血流流速曲线,收缩期前向血流与舒张期反向血流速度、流速曲线宽度等均相似,血流速度无明显增加。5例胎儿中2例无肺动脉瓣缺如综合征肺动脉主干瘤样扩张特征性征象,其中1例胎儿肺动脉瓣缺如伴三尖瓣闭锁,右心发育不良,肺动脉主干无瘤样扩张,反而狭窄。1例胎儿肺动脉瓣缺如伴三尖瓣下移畸形,肺动脉主干虽扩张,但无瘤样扩张。结论胎儿肺动脉瓣缺如产前超声多有特征性表现,易于诊断,对无特征性肺动脉主干瘤样扩张征象肺动脉瓣缺如综合征胎儿,重视对肺动脉瓣口彩色多普勒血流信号及血流流速曲线的观察有助于正确诊断。

Objective To summarize the value of prenatal ultrasonography in the diagnosis of fetal absent pulmonary valve syndrome （APVS）.MethodsBetween January 2010 and January 2014, ifve cases with fetal APVS diagnosed by prenatal ultrasound and conifrmed by autopsy in West China Second Hospital of Sichuan University were reviewed. Four conventional views of fetal echocardiography were used to diagnose APVS, including two-dimentional images, color Doppler lfow imaging （CDFI） and pulsed Doppler lfow spectrum.ResultsTypical signs of fetal APVS in 3 of 5 cases were found： no pulmonary valve was found in the main pulmonary artery, and pulmonary annulus was relatively smaller compared with a tumor-like expansion in pulmonary trunk; “to and fro” sign in the pulmonary trunk were observed steadily by CDFI and “to and fro” spectrum were detected by pulsed Doppler. Another 2 of 5 cases of fetal APVS have no pulmonary expansion, but “to and fro” sign could be detected by color and spectral Doppler, including 1 case with tricuspid atresia and 1 case with Ebstein’s anomaly.ConclusionsPrenatal ultrasonography has important diagnostic value for Fetal APVS. Fetal APVS on prenatal ultrasound has many characteristics and is easy to diagnose. Color and spectral doppler can detect and diagnose fetal APVS effectively.