1 Department of Radiology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, No.106, Zhongshan 2 Rd, Guangzhou, China.
AJR Am J Roentgenol. 2015 May;204(5):979-87. doi: 10.2214/AJR.14.12730.
The purpose of this study was to evaluate the prevalence, MDCT angiography (MDCTA) appearance, associated congenital cardiovascular abnormalities, and prognosis of anomalous origin of one pulmonary artery from the aorta (AOPA) on the basis of MDCTA.
We conducted a retrospective search of patients with AOPA from our database in a single center, consisting of 5729 patients referred for MDCTA with known or suspected congenital heart diseases from transthoracic echocardiography. The clinical information, subtypes of AOPA, associated cardiovascular anomalies, and surgical and clinical outcomes were retrospectively collected and analyzed. The MDCTA images were retrospectively processed for analysis, and the MDCTA and echocardiography images were interpreted by radiologist and cardiologist without knowledge of the actual diagnosis or surgical outcome.
AOPA was seen in 19 patients (14 males and five females; median age, 3 months; range, 4 days-21 years) showing a prevalence of 0.33%. Anomalous origin of the right pulmonary artery (AORPA, 89%), proximal origin subtype of the AOPA (89%), and ipsilateral aortic wall origin of AOPA (58%) were more commonly seen. In addition to the benefit of preoperative planning, MDCTA also supplemented echocardiography by providing accurate diagnosis of AOPA and other associated cardiovascular anomalies compared with transthoracic echocardiography (TTE). We found a total of four patients (21%) with misdiagnosis by TTE, including three patients with underdiagnosis of AOPA and one patient with misdiagnosis as transposition of the great arteries. In addition, two other patients had AOPA diagnosed, but the associated patent ductus arteriosus (PDA) was not detected. MDCTA revealed 95% association with other congenital cardiovascular anomalies, including PDA (71% of AORPA), and aortic arch anomalies (100% of anomalous origin of the left pulmonary artery, AOLPA). The types of surgery depended on the MDCTA findings, including the sub-type, origin sites of AOPA, and associated cardiovascular anomalies. Analysis of the pulmonary arterial sizes showed the McGoon ratios in these patients with a median value of 2.4 (range, 1.5-2.9). Surgical treatment performed before the age of 1 year enabled normalization of pulmonary artery pressure in 92% of patients.
AOPA had a prevalence of 0.33% among patients with congenital heart disease in our series. MDCTA was an important supplement for the diagnosis, delineating the different subtypes and origin sites of AOPA and permitting preoperative planning of AOPA in patients suspected on the basis of echocardiography of having AOPA because accurate diagnosis and early surgical treatment remain the mainstays in improving patient outcome.
本研究旨在基于多层螺旋 CT 血管造影（MDCTA）评估肺动脉异常起源于主动脉（AOPA）的发生率、MDCTA 表现、伴发先天性心血管异常及预后。
我们在单中心回顾性搜索数据库中具有 AOPA 的患者，纳入对象为经胸超声心动图检查疑诊或确诊先天性心脏病、行 MDCTA 检查的 5729 例患者。回顾性收集患者的临床资料、AOPA 亚型、伴发心血管异常及手术和临床转归，对 MDCTA 图像进行回顾性处理分析，由放射科医生和心脏病专家解读 MDCTA 和超声心动图图像，他们对实际诊断或手术结果不知情。
19 例患者（男 14 例，女 5 例；中位年龄 3 个月；范围 4 天至 21 岁）存在 AOPA，发生率为 0.33%。右肺动脉异常起源（AORPA）更常见（89%），近端起源亚型（89%），起源于主动脉壁同侧（58%）。MDCTA 不仅可术前规划，还能为经胸超声心动图（TTE）提供 AOPA 及其他伴发心血管异常的准确诊断，补充 TTE 的不足。我们发现 TTE 共误诊 4 例（21%），包括 3 例漏诊 AOPA，1 例误诊为大动脉转位。此外，还有 2 例患者诊断为 AOPA，但未发现伴发的动脉导管未闭（PDA）。MDCTA 显示 95%的患者伴发其他先天性心血管异常，包括 PDA（71%的 AORPA）和主动脉弓异常（AOLPA，100%）。手术类型取决于 MDCTA 结果，包括 AOPA 的亚型、起源部位及伴发心血管异常。分析肺动脉大小显示这些患者的 McGoon 比值中位数为 2.4（范围 1.5-2.9）。1 岁前接受手术治疗的患者中，92%的患者肺动脉压恢复正常。
在我们的研究中，先天性心脏病患者的 AOPA 发生率为 0.33%。MDCTA 是诊断的重要补充，可明确 AOPA 的不同亚型和起源部位，有助于 TTE 疑诊患者的术前规划，因为准确诊断和早期手术治疗仍是改善患者预后的关键。
