室间隔完整型肺动脉闭锁的彩色多普勒超声诊断

目的 探讨室间隔完整型肺动脉闭锁（PAIVS）的超声影像特点.方法 回顾分析7例PAIVS的超声心动图表现,并与外科手术结果进行比较.结果 PAIVS的超声心动图特征明显：（1）二维超声心动图胸骨旁左心室长轴切面显示室间隔回声完整.（2）心尖四腔切面显示2个心房正位,室间隔回声完整.（3）胸骨旁大动脉短轴切面显示2条大动脉位置关系正常,肺动脉瓣无开放运动,肺动脉瓣无血流信号通过.手术证实7例PAIVS中肺血由未闭的动脉导管供应者6例;主肺动脉及分支发育不良,仅有体肺动脉侧支供应肺血者1例.心房水平右向左分流者7例：其中5例为卵圆孔未闭,2例为房间隔缺损.合并畸形包括三尖瓣闭锁,三尖瓣发育不良伴狭窄、房间隔膨凸瘤等.结论 PAIVS患儿肺血来源多样化,肺动脉发育程度不一,超声心动图特征明显,对PAIVS有特异性诊断价值.

The Diagnosis of Pulmonary Atresia with Intact Ventricular Septum by Colour Doppler

Objective To investigate the characteristics of the images of pulmonary atresia with intact ventricular septum （PAIVS）by color doppler echocardiography.Methods Retrospective analysis was made on 7 PAIVS cases of ultrasonic cardiogram,and the result of surgery was compared.Results The characteristics of ultrasonic image in PAIVS were obvious：（1） Two-dimensional echocardiography （2DE） parastermal left ventricle long axis view l showed intact echo of interventricular septum.（2） Apical four chamber view of 2DE showed atrial situs solitus,intact echo of interventricular septum.（3） In parasternal short axis view of 2DE,the two great arteries showed normal positionwith no opening on pulmonary artery valve,and no flow through pulmonary valve.Surgery confirmed that 6 patients of 7 PAIVS had lung blood supply through the arterial duct; only one with dysplastic main pulmonary artery and branch dysplasia,had the lung blood supply is through the major aortopulmonary collateral arteries （MAPCA）.7 cases with bypass flow from right to left in the atrium level,5 of which with acleistocardia and 2 with atrial septal defect.Associated cardiac anomalies include tricuspid atresia,Three tricuspid valve dysplasia with narrow,and interatrial septum bulging tumor,etc.Conclusions The patients with PAIVS had diversified sources of pulnonary blood flow,and the levels of growth of pulmonary arteries varied with obvious echocardiographic characteristics,which was of diagnosis value in PAIVS.