超声心动图诊断先天性单侧肺动脉缺如的价值

Evaluation of echocardiography in diagnosing unilateral absence of pulmonary artery

目的探讨彩色多普勒超声心动图对诊断先天性单侧肺动脉缺如(UAPA)的临床应用价值。方法选取2008至2012年经手术或增强CT证实的7例单侧肺动脉缺如(左侧5例,右侧2例)患儿为研究对象,回顾分析其超声心动图特征。结果先天性单侧肺动脉缺如患儿7例,2例单发,5例合并其他心血管畸形。超声首诊5例(5/7),漏诊1例,误诊1例,超声首诊时将左肺动脉缺如误诊肺动脉吊带。先天性单侧肺动脉缺如超声心动图表现为正常的肺动脉分叉结构消失,而是向远端直接延续为一侧肺动脉,向左或向右走行,形成肺动脉左支或右支。结论先天性单侧肺动脉缺如较罕见,临床表现无特异性,容易漏诊误诊。患者行常规超声心动图检查时应注意肺动脉分叉及左右分支的发育情况,若有异常应多切面仔细扫查,对提高该病诊断率具有重要的临床应用价值。

Objective To explore the value of echocardiography in diagnosing unilateral absence of pulmonary artery（ UAPA ）. Methods A retrospective analysis was done for 7 patients with UAPA （5 were left pulmonary artery absent and 2 were right pulmonary artery absent） diagnosed from 2008 to 2012. We reviewed the etiological and echocardiographic characteristics of this disease. Results There were 7 children with UAPA in the study. Five of them had additional congenital cardiac malformations. A total of 5 UAPA patients （5/7） were first and correctly diagnosed by echocardiography. Meanwhile, 1 patient was missed and 1 case was misdiagnosed. One misdiagnosed case was first diagnosed as pulmonary artery sling and then was diagnosed correctly as UAPA by another experienced doctor. The result was proved by surgical operation. The U APA was characterized as complete absence of normal pulmonary artery bifurcation, and main pulmonary artery extending into only one artery branch （left or right）. Conclusions UAPA is a rare anomaly and the disease has non-specific presentation and easy to make incorreet and missed diagnosis. Echocardiography is an effective method in the diagnosis of UAPA. Attention should be paid on pulmonary artery bifurcation and left and right pulmonary artery when performing routine ultrasound examination. Multiple sections should be performed to achieve the correct diagnosis when abnormal findings were present.