先天性肺动脉瓣缺如的超声心动图诊断

Echocardiographic diagnosis of congenital absent pulmonary valve

目的探讨超声心动图对先天性肺动脉瓣缺如(absent pulmonary valve,APV)的诊断价值。方法选取82例经CTA及心外科手术诊断为APV患者临床超声声像图资料,分析其表现特征。结果APV声像图主要表现为肺动脉瓣叶缺如或发育不良、瓣环狭窄、总干及分支瘤样扩张、瓣口重度反流。本组合并法洛四联症68例(82.9%),合并其他畸形14例(17.1%)。82例患者中动脉导管缺失73例(89.0%),合并动脉导管未闭9例(11.0%);室间隔缺损78例(95.1%),室间隔完整4例(4.9%)。82例患者中,超声诊断符合74例(90.2%);超声漏、误诊8例(9.8%),其中7例漏诊,1例误诊为肺动脉瓣上赘生物形成。结论超声心动图技术可比较准确地诊断APV,但容易漏误诊,需要与单纯肺动脉瓣狭窄及肺动脉瓣上赘生物形成鉴别。

Objective To review the imaging characteristics and to evaluate the diagnostic value of Doppler echocardiography for absent pulmonary valve(APV).Methods Doppler echocardiography was performed and these images were compared with CTA and operative findings in 82 children with APV.Results APV was diagnosed in the presence of a ridge of rudimentary pulmonary valvular tissue at the level of the pulmonary annulus and the aneurysmal dilation of the pulmonary arteries with restrictive anulus with to-and-fro blood flow on color and pulsed-wave Doppler echocardiography.The associated malformations included Tetralogy of Fallot 68 cases,14 cases with other cardiovascular anomalies.9 of 89 cases with patent ductusarteriosus,4 cases with intact ventricular septum.74 of 82 cases(90.2%)were diagnosed correctly,8 cases(9.8%)were miss-diagnosed,APV was found by CTA or during operation in 7 cases,1 case was misdiagnosed as vegetation on the pulmonic valve.Conclusion APV could be diagnosed accurately by Doppler echocardiography,it is prone to be miss-diagnosed and should be distinguished from isolated pulmonary valve stenosis and vegetation on the pulmonic valve.