分析先天性肺动脉异常起源的超声心动图特征及诊断价值。

收集北京军区总医院2008年1月至2014年12月经CT或手术证实的先天性肺动脉异常起源患儿32例为研究对象。回顾性分析患儿超声心动图特征。

单侧肺动脉缺如7例（左6/右1）、右肺动脉起源于主动脉3例、肺动脉吊带22例。超声明确诊断28例，提示但不确定1例，肺动脉吊带漏诊2例，左肺动脉缺如误诊1例；诊断符合率90.6%（29/32）。超声心动图共同特征为肺动脉主干及一侧分支正常显示，另一分支不能正常显示。特异超声心动图特征：单侧肺动脉缺如，于多切面观察均不能显示另一侧肺动脉分支；单侧肺动脉起源异常，于升主动脉可显示异常血管分支且合并重度肺动脉高压；肺动脉吊带，于右肺动脉远端可显示异常起源的左肺动脉。

先天性肺动脉异常起源具有特殊的超声心动图特征，经胸超声心动图检查能够早期、准确的做出诊断，多切面观察异常肺动脉分支的位置是鉴别诊断的关键环节。

To analyze the echocardiographic features of anomalous origin of the pulmonary artery.

Echocardiographic data of 32 patients with anomalous origin of the pulmonary artery proved by CT or surgical operation in Beijing Military General Hospital from January 2008 to December 2014 were analyzed retrospectively.

Of them, 7 cases were unilateral absence of pulmonary artery (UAPA), 3 cases were anomalous origin of pulmonary artery (AOPA), and 22 cases were pulmonary artery sling (PAS). Among 32 cases, 2 PAS were missed, 1 UAPA case was misdiagnosed and 1 case was merely suspected by transthoracic echocardiography (TTE). The accuracy rate was 90.6% (29/28). The common echocardiographic feature was that the pulmonary trunk and one branch can be displayed normally. The specific echocardiographic features were as follows. In UAPA cases, there was always only one branch of pulmonary artery can be shown. In AOPA cases, an abnormal artery branch can be found at ascending aorta area, and associated with severe pulmonary hypertension. In PAS cases, the left pulmonary artery branch was derived from the distal right pulmonary artery, which could be displayed at high-level pulmonary artery artery long axis plane.

Anomalous origin of the pulmonary artery had special echocardiographic features. Therefore TTE was able to diagnose the disease early and accurately.