单侧肺动脉异常起源于升主动脉的产前超声心动图诊断

目的探讨单侧肺动脉异常起源于升主动脉(AOPA)的产前超声心动图特征,提高对本病产前诊断的准确率。方法回顾性分析2012年6月1日至2018年10月30日经首都医科大学附属北京安贞医院(我院)产前诊断的6例AOPA胎儿的超声心动图特点,总结该病胎儿期的超声心动图特征。结果 5例胎儿为右肺动脉异常起源于升主动脉(AORPA),其中3例为孤立型AORPA,2例合并Berry综合征; 1例为左肺动脉异常起源于升主动脉(AOLPA),同时合并先天性肺动脉瓣缺如综合征。6例胎儿均为近端型AOPA,其中2例胎儿引产(1例AOLPA,1例合并Berry综合征)。出生的4例患儿中,1例Berry综合征患儿于出生后63 d因反复肺炎、心衰死亡; 2例行AORPA根治手术,术后均恢复良好; 1例刚出生,仍在随访中。近端型AOPA胎儿超声心动图特征:①多切面显示正常的肺动脉主干分叉处"八"字型结构消失,正常的肺动脉分支内径增宽;②在三血管气管切面(3VT)及左室长轴切面二维及彩色多普勒显像(CDFI)可显示升主动脉发出一粗大血管向肺内走行,该血管发出位置靠近主动脉瓣左后或右后侧壁的升主动脉;③CDFI显示异常肺动脉分支血流孤立,与主肺动脉缺乏确切连接;④频谱多普勒显示向肺内走行的血管为肺动脉血流频谱;⑤不合并其他心内畸形时,心内结构正常。结论超声心动图可对胎儿期的近端型AOPA做出准确的诊断,3VT及左室长轴切面对诊断近端型AOPA至关重要。

Prenatal diagnosis of anomalous origin of one pulmonary artery branch from the aorta by echocardiography

Objective To improve the prenatal diagnosis accuracy of anomalous origin of one pulmonary artery branch from the aorta (AOPA) by analyzing fetal echocardiography features. Methods Fetal echocardiographic features were analyzed retrospectively in 6 cases with prenatal diagnosis of AOPA from June 2012 to October 2018, and the fetal echocardiography characteristics of AOPA were summarized. Results Among the 6 fetuses with AOPA, 5 cases were anomalous origin of right pulmonary artery from the aorta (AORPA), including 3 cases with isolated AORPA and 2 cases combined with Berry syndrome, and one case was anomalous origin of left pulmonary artery from the aorta (AOLPA) with tetralogy of Fallot (TOF) with an absent pulmonary valve. All cases had proximal form. Among the 6 fetuses, 2 cases had induced labor, including one case with TOF and one case combined with Berry syndrome. One case with Berry syndrome died of recurrent pneumonia and heart failure at 63 days after birth without surgical treatment. Other 2 cases with isolated AORPA underwent surgical repair after birth and recovered well after surgery. One case was just born and still under observation. Characteristic sonographical findings included: ① The main pulmonary artery was continuous with one pulmonary artery and there was no confluence with the other pulmonary artery. The diameter of normal pulmonary artery branch was widened. ② On the basis of three vessels and trachea view (3VT) and long axial view of left ventricle (LV), two-dimensional and color Doppler imaging (CDFI) showed a large vessel originated from the aortic arch, and the anomalous vessel arose proximally from the right or left lateral posterior aspect of the ascending aorta close to the aortic valve. ③ CDFI showed that the blood flow of the anomalous pulmonary artery branch was isolated and there was no exact connection with the main pulmonary artery. ④ Spectrum Doppler of the anomalous vessel of the lung confirmed that it was the anomalous origin of one pulmonary artery branch. ⑤ When other intracardiac abnormalities were not present, the inner diameter and proportion of the heart cavity were normal. Conclusion Proximal form of AOPA in the prenatal period can be accurately diagnosed by echocardiography. The 3VT and long axial view of LV are very important for the diagnosis of proximal form of AOPA.