探讨超声心动图对单侧肺动脉起源异常的诊断价值。

回顾性分析1999年1月至2015年5月在复旦大学附属儿科医院进行超声心动图检查，诊断或疑似诊断为单侧肺动脉起源异常（AOPA）患儿的超声心动图资料。超声心动图结果与多排螺旋CT（MSCT）和术中诊断结果比较，分析超声心动图对单侧肺动脉异常起源的诊断及随访价值。

超声心动图共诊断42例单侧肺动脉起源异常，其中确诊36例，疑似诊断6例，男性24例，女性18例，平均月龄（9.76±27.00）个月。36例确诊患儿中，4例患儿首次本院超声心动图误诊为原发性肺动脉高压，再次超声心动图检查确诊为单侧肺动脉起源异常；4例患儿外院误诊或漏诊。6例疑似诊断患儿中，通过MSCT检查，5例明确诊断为单侧肺动脉起源异常，1例为右侧肺动脉缺如。超声心动图直接征象为主肺动脉分叉结构消失，发现一侧肺动脉由主动脉发出；间接征象表现为重度肺动脉高压。诊断准确率为85.7%（36/42），近端型诊断准确率97.2%，远端型诊断准确率2%。20例患儿进行手术治疗后，超声心动图随访，吻合口平均流速为（2.36±0.62）m/s，平均压差为（23±13）mmHg（1 mmHg=0.133 kPa）。

超声心动图是术前诊断单侧肺动脉起源异常的首选有效方法，可明确诊断近端型，对远端型可提示诊断；同时也是术后评价肺动脉吻合口及肺动脉压力简便、无创的最佳方法。

To analyze the echocardiographic diagnosis value in anomalous origin of one pulmonary artery (AOPA).

The clinical, surgical findings and postoperative follow-up data of patients with an echocardiographic diagnosis of AOPA in the Childern′s Hospital of Fudan University from Jan 1999 to Sep 2014 were restrospectively reviewed. Compared with MSCT and intraoperative findings, the diagnostic and fellow-up value of echocardiography in AOPA was evaluated.

Totally, forty-two cases were diagnosed or suspected as AOPA. Thirty-six cases were diagnosed clearly while 6 cases were suspected. There were 24 males and 18 females with an average age of (9.76±27.00) month. Among 36 patients, 4 cases were misdiagnosed as primary pulmonary hypertension at first time, and definite diagnosis was made at second time by echocardiography. Among 6 suspected cases, 5 cases were confirmed by MSCT, 1 was diagnosed by MSCT as RPA absence. The direct sign of echocardiography included disappearance of pulmonary artery bifurcation structure, and one pulmonary artery arising from aorta; while the indirect sign was severe pulmonary hypertension. The diagnostic accuracy was 85.7% (36/42) totally, and 97.2% in proximal type and 2% in distal type separately. Twenty cases had operation for reconnecting of main pulmonary and the brunch. The mean velocity of the anastomotic stoma was (2.36±0.62) m/s, and the pressure gradient was (23±13) mmHg (1 mmHg=0.133 kPa).

Echocardiography is the choice of method for diagnosing AOPA preoperatively, which is noninvasive and effective. It can diagnosed proximal type clearly, and give diagnostic clues to distal type. Moreover, it is the best imaging method for patients′ post-operation follow up.