Donazzan Luca, Crepaz Robert, Stuefer Josef, Stellin Giovanni
Department of Cardiology, San Maurizio Regional Hospital, Bolzano, Italy.
Department of Cardiology, San Maurizio Regional Hospital, Bolzano, Italy
World J Pediatr Congenit Heart Surg. 2014 Oct;5(4):546-53. doi: 10.1177/2150135114551028.
Systemic hypertension (HT) is a major long-term complication even after successful repair of aortic coarctation (CoA), and many factors are involved in this pathophysiology.
To investigate the role of abnormalities in the aortic arch shape, central aortic flow dynamics, and distensibility in developing HT after successful repair of CoA.
We selected a group of 26 normotensive patients (mean age 16.9±7.3 years, range 9-32 years) with anatomically successful repair of CoA among 140 patients regularly followed after repair of CoA and analyzed their last clinical and echocardiographic data. Bicycle exercise test and ambulatory blood pressure monitoring (ABPM) were also obtained. Mean age at surgical repair was 3.2±3.9 years (range 10 days-15 years); 12 patients underwent surgical correction during the first year of life. Repair of CoA was performed by end-to-end anastomosis (TT) in 23 patients (extended TT in 6 patients with arch hypoplasia), patch aortoplasty in 2, and subcalvian flap aortoplasty in 1. The postsurgical follow-up was 13.8±7.2 years (range 3.5-29.4 years). The shape of the aortic arch was defined by magnetic resonance imaging (MRI) on this global geometry (normal-gothic-crenel), ratio of the height-transverse diameter (A/T), percentage of residual stenosis, and growth index of the transverse arch segments. Flow mapping by phase-contrast imaging in the ascending and descending aorta was performed in order to measure the systolic waveforms and central aortic distensibility. Twenty normal age-matched patients submitted to the same MRI protocol were used as controls.
Six patients were found to have a gothic and 20 a normal aortic arch shape. Patients with gothic aortic arch shape had an increased A/T ratio (0.80±0.07 vs 0.58±0.05, P<.001), a greater loss of systolic wave amplitude across the aortic arch (43%±2% vs 34%±5%, P<.001), and a lower distensibility of the ascending aorta compared to those with normal shape (4.87±1.06 mm Hg(-1)×10(-3) vs 7.20±1.73 mm Hg(-1)×10(-3); P=.005) and controls (4.87±1.06 mm Hg(-1)×10(-3) vs 8.57±1.71 mm Hg(-1)×10(-3); P<.001). The maximal systolic blood pressure (SBP) on exercise as well as SBP, diastolic blood pressure, and percentage of >135 mm Hg on ABPM were higher in the gothic than in the normal arch group. There was a correlation between nocturnal SBP, 24 hours pulse pressure on ABPM in the whole group, and different MRI variables (A/T, distensibility of the ascending aorta, and percentage of loss of systolic wave amplitude).
In the long-term follow-up after CoA repair, a gothic arch shape is associated with a decreased ascending aorta distensibility with an increased loss of systolic wave amplitude across the aortic arch. These findings explain at least in part the association between this abnormal arch geometry and late HT at rest and on exercise, left ventricular hypertrophy, and adverse cardiovascular outcome in this group of young adults with successful repair of CoA.
即使主动脉缩窄(CoA)修复成功,系统性高血压(HT)仍是主要的长期并发症,这种病理生理过程涉及多种因素。
探讨主动脉弓形态异常、主动脉中心血流动力学及扩张性在CoA成功修复后发生HT过程中的作用。
我们从140例CoA修复术后定期随访的患者中选取了26例血压正常的患者(平均年龄16.9±7.3岁,范围9 - 32岁),这些患者的CoA解剖修复成功,并分析了他们最后的临床和超声心动图数据。还进行了自行车运动试验和动态血压监测(ABPM)。手术修复时的平均年龄为3.2±3.9岁(范围10天至15岁);12例患者在出生后第一年内接受了手术矫正。23例患者采用端端吻合术(TT)修复CoA(6例主动脉弓发育不全患者采用扩大TT),2例采用补片主动脉成形术,1例采用锁骨下皮瓣主动脉成形术。术后随访时间为13.8±7.2年(范围3.5 - 29.4年)。通过磁共振成像(MRI)根据整体几何形状(正常 - 哥特式 - 城垛式)、高度与横径之比(A/T)、残余狭窄百分比以及横弓段生长指数来定义主动脉弓的形态。在升主动脉和降主动脉进行相位对比成像血流映射,以测量收缩期波形和主动脉中心扩张性。选取20例年龄匹配的正常患者,按照相同的MRI方案进行检查作为对照。
发现6例患者的主动脉弓呈哥特式,20例呈正常形态。主动脉弓呈哥特式的患者A/T比值升高(0.80±0.07对0.58±0.05,P <.001),整个主动脉弓收缩期波幅损失更大(43%±2%对34%±5%,P <.001),与正常形态患者相比,升主动脉扩张性更低(4.87±1.06 mmHg⁻¹×10⁻³对7.20±1.73 mmHg⁻¹×10⁻³;P = 0.005),与对照组相比也更低(4.87±1.06 mmHg⁻¹×10⁻³对8.57±1.71 mmHg⁻¹×10⁻³;P <.001)。运动时的最大收缩压(SBP)以及ABPM监测的SBP、舒张压和收缩压>135 mmHg的百分比,哥特式主动脉弓组均高于正常主动脉弓组。夜间SBP、全组ABPM的24小时脉压与不同的MRI变量(A/T、升主动脉扩张性和收缩期波幅损失百分比)之间存在相关性。
在CoA修复后的长期随访中,哥特式主动脉弓形态与升主动脉扩张性降低以及整个主动脉弓收缩期波幅损失增加有关。这些发现至少部分解释了这一异常弓部几何形状与该组CoA成功修复的年轻成年人静息和运动时晚期HT、左心室肥厚及不良心血管结局之间的关联。