超声心动图对儿童先天性单侧肺动脉缺如的诊断价值

Ultrasound Cardiogram in Diagnosis of Pediatric Congenital Unilateral Absence of Pulmonary Artery

目的单侧肺动脉缺如是罕见的先天性肺血管发育异常,因症状不典型易漏误诊。本研究探讨超声心动图对小儿先天性单侧肺动脉缺如(UAPA)的诊断及随访价值。资料与方法回顾性分析2009年5月—2016年7月广州市妇女儿童医疗中心经手术或CT血管造影(CTA)证实的35例UAPA患儿的超声心动图图像,对超声心动图诊断正确及漏误诊的图像特征进行分析。结果 35例UAPA患儿中,右侧21例,左侧14例;单纯性16例,合并其他心内畸形19例。超声心动图首诊27例,漏诊7例,误诊1例;诊断正确率为77.1%。UAPA超声心动图显示正常的肺动脉分叉结构消失,主肺动脉延续为一侧肺动脉,向左或向右走行,形成肺动脉左支或右支。缺如侧肺内段的肺动脉通过主动脉或其分支或其他侧支血管供血。8例患儿进行单侧肺动脉重建手术治疗后1个月超声心动图显示近端吻合口平均流速为(1.22±0.17)m/s,远端平均流速为(2.17±0.56)m/s。结论超声心动图是UAPA术前诊断的有效方法,同时也是术后评价肺动脉吻合口及肺动脉压力简便、无创、有效的方法。

Purpose Unilateral absence of pulmonary artery is a rare congenital pulmonary arterydysplasia whose untypical symptom often leads to missed diagnosis or misdiagnosis. Diagnostic and follow-up values of ultrasound cardiogram to pediatric congenital unilateral absence of pulmonary artery（UAPA） are explored in this paper. Materials and Methods Ultrasound cardiogram images of 35 UAPA patients confirmed by surgery or CT angiography in Guangzhou Women and Children＇s Medical Center from May 2009 to July 2016 were analyzed retrospectively, and images characteristics of the correctly diagnosed and missed diagnosis or misdiagnosed ultrasound cardiogram were analyzed. Results Among these 35 UAPA patients, 21 cases were on the right side, 14 cases on the left side, 16 cases single UAPA and 19 cases combined with other intracardiac malformations. 27 cases were initially diagnosed by ultrasound cardiogram, 7 cases were missed diagnosed and 1 case misdiagnosed, with 77.1% diagnosis accuracy. UAPA ultrasound cardiogram showed that normal bifurcation structure of pulmonary artery disappears. Main pulmonary artery continued to be one-side pulmonary artery, running to left or right and forming left branch or right branch of pulmonary artery. Pulmonary artery of the one-side absence of pulmonary segment supplied blood through aorta or other branches or vessels on the other side. After receiving unilateral pulmonary artery reconstruction surgery, ultrasound cardiogram of 8 patients of one month indicated that average flow rate of close-end anastomosis was（1.22±0.17） m/s and far-end（2.17±0.56） m/s. Conclusion Ultrasound cardiogram is efficient for UAPA preoperative diagnosis. Meanwhile, it is convenient, noninvasive and efficient in assessing anastomosis and pressure of pulmonary artery after surgery.