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.