产前超声诊断肺动脉瓣缺如综合征

Prenatal ultrasound diagnosis of absent pulmonary valves syndrome

目的 探讨产前超声诊断肺动脉瓣缺如综合征的临床价值及其两种不同类型的病理改变.方法 分析超声诊断的8例肺动脉瓣缺如综合征胎儿的心脏超声表现,按节段分析法判断心房、心室及大血管的连接关系,测量房室大小、大血管内径、房室瓣口及半月瓣口的血流速度,并计算心胸面积比值.结果 ①8例均为左位心,心房正位,心室右袢;②8例均表现为典型的肺动脉瓣缺如征象：主肺动脉及左、右肺动脉呈瘤样扩张,肺动脉内见往返的五彩镶嵌的血流信号,频谱多普勒显示肺动脉狭窄与反流并存的频谱;③本组病例表现为两种类型：其中6例合并室间隔缺损、主动脉骑跨及动脉导管缺如（法洛四联征型）,其余2例无室间隔缺损,均可显示动脉导管;④5例胎儿除心脏畸形外,尚合并心外畸形.结论 肺动脉瓣缺如综合征的超声表现十分典型,产前诊断容易识别.由于存在两种不同的类型,应进一步观察胎儿室间隔及动脉导管的状况,使诊断更加完善.

Objective To assess the value of prenatal ultrasonography in diagnosing fetal absent pulmonary valve syndrome（APVS）. Methods This was a retrospective study of 8 fetuses with absent pulmonary valve syndrome. Fetal echocardiography and segmental analysis were used to determine the atrial situs,atrioventricular connections and ventriculoarterial relationships. Cardiac chamber dimensions,Doppler studies of the atrioventricular valve and semilunar value were recorded, and cardiothoracic ratio were calculated. Results Levocardia,normal situs and atrioventricular connections were present in all cases. All cases showed typical signs：severe dilatation of the pulmonary trunk and it＇s branches, and concurrent occurrence of pulmonary valve stenosis and regurgitation. In 6/8 there was an associated ventricular septal defect,overriding aorta and absent arterial duct（Fallot-like APVS）,while in 2/8 there was an assoiciated intact ventricular septal and patent arterial duct. Extracardiac malformations were detected in 5 cases.Conclusions APVS can be accurately diagnosed by prenatal ultrasonography but we should pay more attention to the ventricular septal and arterial duct,due to the existence of two variants.