Fong L V, Anderson R H, Siewers R D, Trento A, Park S C
Wessex Cardiothoracic Centre, Southampton General Hospital.
Br Heart J. 1989 Nov;62(5):389-95. doi: 10.1136/hrt.62.5.389.
Six patients with anomalous origin of one pulmonary artery from the ascending aorta were reviewed. Four had anomalous origin of the right pulmonary artery and two had anomalous origin of the left pulmonary artery from the ascending aorta. Two of these six patients had tetralogy of Fallot. Two patients died in the first month of life. No changes in the pulmonary vasculature were seen at necropsy. Corrective surgery was attempted in two patients with associated tetralogy of Fallot when they were two years old but both died. At necropsy there was severe pulmonary vascular disease in the lung supplied by the anomalous pulmonary artery but no pulmonary vascular hypertensive changes in the lung supplied by the pulmonary artery from the right ventricle. Two recent patients underwent successful anastomosis of the anomalous pulmonary artery to the main pulmonary artery at three months and one month and three weeks of age respectively. Intraoperative lung biopsy in the latter patient showed early changes in both lungs. Both echocardiography and cardiac catheterisation were used in the diagnoses. Systemic or suprasystemic pressures were found in the pulmonary artery arising from the right ventricle as well as the anomalous pulmonary artery in the three patients without tetralogy of Fallot. Anomalous origin of a pulmonary artery from the ascending aorta is a distinct entity and differs from other aorto-pulmonary arterial connections. Early surgical intervention is recommended in all patients (including those patients with associated tetralogy of Fallot) because of the risk of rapid development of irreversible pulmonary vascular disease.
回顾了6例肺动脉起源于升主动脉异常的患者。其中4例右肺动脉起源异常，2例左肺动脉起源于升主动脉异常。这6例患者中有2例患有法洛四联症。2例患者在出生后第一个月死亡。尸检未见肺血管系统有改变。2例合并法洛四联症的患者在2岁时尝试进行矫正手术，但均死亡。尸检发现，异常肺动脉供血的肺脏有严重的肺血管疾病，但右心室肺动脉供血的肺脏未见肺血管高压改变。最近有2例患者分别在3个月、1个月零3周龄时成功地将异常肺动脉与主肺动脉进行了吻合。后一例患者术中肺活检显示两肺均有早期改变。诊断过程中使用了超声心动图和心导管检查。在3例不合并法洛四联症的患者中，右心室起源的肺动脉以及异常肺动脉中均发现了体循环或超体循环压力。肺动脉起源于升主动脉异常是一种独特的疾病，与其他主动脉-肺动脉连接不同。由于存在不可逆肺血管疾病快速发展的风险，建议对所有患者（包括合并法洛四联症的患者）尽早进行手术干预。
