Ann and Robert H. Lurie Children's Hospital of Chicago, 225 E. Chicago Ave., Chicago, IL, 60611, USA.
Northwestern University Feinberg School of Medicine, Chicago, IL, USA.
Pediatr Radiol. 2020 Jan;50(1):17-27. doi: 10.1007/s00247-019-04507-0. Epub 2019 Aug 31.
Interrupted aortic arch (IAA) is a rare but severe congenital abnormality often associated with bicuspid aortic valve (BAV). Complex re-interventions are often needed despite surgical advances, but the impact of aortic hemodynamics in repaired patients is unknown.
Investigate effect of IAA repairs on aortic hemodynamics, wall shear stress and flow derangements via 4-D flow MRI.
We retrospectively analyzed age- and gender-matched cohorts (IAA [n=6], BAV alone [n=6], controls [n=6]) undergoing cardiac MRI including 4-D flow. Aortic dimensions were measured from standard MR angiography. We quantified peak systolic velocities, regurgitant fractions and wall shear stress in the ascending aorta (AAo), transverse arch and descending aorta (DAo) from 4-D flow, and we graded helix/vortex flow patterns from 3-D blood flow visualization.
Children and young adults with IAA had a wide range of arch dimensions, peak systolic velocities, regurgitant fractions and flow grades. Peak transverse arch systolic velocities were higher in patients with IAA versus controls (P=0.02). Flow derangements in the AAo were found in patients with IAA (median grade=2, 5/6 patients, P=0.04) and BAV (median grade=3, 5/6 patients, P=0.03) versus controls. Flow derangements in the DAo were only seen in patients with IAA (median grade=1, 5/6 patients, P=0.04), and 5/6 people with IAA had helical flow in head and neck vessels. Wall shear stress was increased in people with IAA along the superior transverse arch and proximal DAo versus controls (P=0.02).
Complex congenital aortic arch repairs can change aortic hemodynamics. Associated cardiac defects can further alter findings. Studies are warranted to investigate clinical implications in larger cohorts.
中断的主动脉弓(IAA)是一种罕见但严重的先天性异常,常与二叶式主动脉瓣(BAV)相关。尽管手术技术有所进步,但仍经常需要进行复杂的再次介入治疗,但修复患者的主动脉血流动力学的影响尚不清楚。
通过 4D 流 MRI 研究 IAA 修复对主动脉血流动力学、壁面切应力和血流紊乱的影响。
我们回顾性分析了接受心脏 MRI 检查(包括 4D 流)的年龄和性别匹配队列(IAA [n=6]、BAV 单独 [n=6]、对照组 [n=6])。从标准 MR 血管造影中测量主动脉尺寸。我们从 4D 流中量化升主动脉(AAo)、横弓和降主动脉(DAo)的峰值收缩速度、反流分数和壁面切应力,并从 3D 血流可视化中对螺旋/涡旋血流模式进行分级。
患有 IAA 的儿童和年轻人的弓尺寸、峰值收缩速度、反流分数和血流分级范围广泛。与对照组相比,IAA 患者的横弓峰值收缩速度更高(P=0.02)。IAA(中位数分级=2,5/6 例患者,P=0.04)和 BAV(中位数分级=3,5/6 例患者,P=0.03)患者的 AAo 血流紊乱。仅在 IAA 患者中观察到 DAo 的血流紊乱(中位数分级=1,5/6 例患者,P=0.04),并且 5/6 例 IAA 患者的头颈部血管存在螺旋血流。与对照组相比,IAA 患者的上横弓和近端 DAo 处的壁面切应力增加(P=0.02)。
复杂的先天性主动脉弓修复可以改变主动脉血流动力学。相关的心脏缺陷会进一步改变发现。需要进行更大队列的研究来调查临床意义。