主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的影像学诊断

The imaging examination of aorto-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch

目的:探讨多种影像技术对主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的诊断价值。方法:2005—2013年武汉亚洲心脏病医院先后收治7例主-肺动脉间隔缺损及右肺动脉起源于升主动脉合并主动脉弓离断的患者,对其超声心动图及CT图像、导管检查结果进行回顾性分析。结果:7例患者均行超声心动图及CT检查,诊断为Ⅱ型主-肺动脉间隔缺损(Ho分型法),均合并右侧肺动脉起源于升主动脉及A型主动脉弓离断(Celoria与Patton分型法)。4例患者行导管检查,3例考虑为阻力型肺动脉高压,放弃手术治疗,余4例均行一期手术矫治。结论:超声心动图结合CT检查是诊断本病的可靠手段。手术指征的判断需结合心导管检查、心血管造影。肺动脉压力及阻力是影响手术时机和预后的关键因素。

Objective： To evaluate the diagnostic value of the imaging examination of aorto-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch. Methods： Reviewed clinical data from medical records for 7 patients who had been treated in Wuhan Asia Heart Hospital between 2005 and 2013. Results： By UCG and CT, all cases were diagnosed as type A interruption of the aortic arch（according to celoria and patton ＇s classification）, type Ⅱaorto-pulmonary septal defect（according to Ho classification） and aortic origin of the right pulmonary artery. By cardiac catheterization, 3 of 4 patients were considered as severe pulmonary hypertention and gave up the operation, other 4 patients were treated as radical surgery. Conclusions： The combination of UCG and CT is a reliabe technique in the diagnosis of aorto-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch. The decision for surgery should be based on the combined information from cardiac catheterization and cardiovascular angiography. Pulmonary artery pressure and resistance are the most important factors that have great effects on the duration of surgery and the outcome.