Fan Qiang, Ling Yunfei, Wang Yue, An Qi
Department of Cardiovascular surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
J Card Surg. 2021 Feb;36(2):696-697. doi: 10.1111/jocs.15136. Epub 2020 Dec 6.
Aortopulmonary window (APW) is a rare but serious congenital cardiac malformation, most patients with APW will die from congestive heart failure within one year after birth. In fact, patients with large APW is rarely seen in childhood or adult life. However, we report an older child with a large anomalous "window" on the ascending aorta, with discontinuous pulmonary arteries, and the left pulmonary artery (LPA) arising via a left-sided arterial duct in the presence of a right aortic arch. Preoperative diagnosis made by echocardiography and chest computerized tomography revealed anatomical futures clearly. Cardiac catheterization indicated that the pulmonary resistances indices were 2.92 Wood unit/m in LPA and 3.35 Wood unit/m in RPA, was 3.26. This patient underwent surgical correction at the age of 10 and successfully survived.
主肺动脉窗（APW）是一种罕见但严重的先天性心脏畸形，大多数APW患者会在出生后一年内死于充血性心力衰竭。事实上，患有大型APW的患者在儿童期或成年期很少见。然而，我们报告了一名大龄儿童，其升主动脉上有一个巨大的异常“窗口”，肺动脉不连续，在右位主动脉弓存在的情况下，左肺动脉（LPA）通过左侧动脉导管发出。术前经超声心动图和胸部计算机断层扫描做出的诊断清楚地显示了解剖特征。心导管检查表明，LPA的肺阻力指数为2.92伍德单位/米，RPA的肺阻力指数为3.35伍德单位/米，平均为3.26。该患者在10岁时接受了手术矫正并成功存活。
