背景:双主动脉弓是最常见的完整血管环形式。气管和/或食道可以被完整的血管环压缩,这可能导致儿童双主动脉弓的早期呼吸和/或食管症状。准确的产前评估气管压迫情况可为围产期双主动脉弓的临床处理和婴幼儿双主动脉弓的急诊治疗提供相关信息。胎儿气管充满羊水,可以用产前超声清楚地看到。先前的研究报道了使用产前超声测量正常胎儿的气管内径,并显示胎儿气管内径与胎龄之间存在线性相关性。然而,据我们所知,很少有研究使用超声定量评估双主动脉弓胎儿的气管压迫。
目的:本研究旨在应用产前超声评估双主动脉弓胎儿血管环对气管压迫的影响,分析气管压迫与出生后临床症状的关系。
方法:回顾性分析2011年1月至2021年4月2家机构产前超声诊断为双主动脉弓的胎儿资料。前瞻性招募以正常胎儿为对照组的单胎妊娠。在具有双主动脉弓的胎儿和正常胎儿中评估了通过比较气管内径z评分与胎龄来评估的气管压迫。将具有双主动脉弓的活产婴儿分为有症状组和无症状组,以比较z评分。绘制了气管内径z评分截止值和双主动脉弓有症状婴儿的预测的接收器工作特征曲线。对观察员内部和观察员之间的协定进行了调查。
结果:共诊断出26例双主动脉弓胎儿,14例(53.8%)双主动脉弓胎儿存活。在14名活产婴儿中,7例(50.0%)有症状,而7例(50.0%)无症状。双主动脉弓组气管内径z评分明显低于正常组(-0.62±1.36vs0.00±0.78;P<.001)。有症状组的气管内径z评分明显低于无症状组(-1.42±0.92vs-0.49±0.96;P=.018)。曲线下面积为0.878(95%置信区间,0.689-1.000)。使用气管内径z评分截止值为-1.21,灵敏度为71%,特异性接近100%。观察者间和观察者内协议的组内相关系数为0.987(95%置信区间,0.980-0.992)和0.975(95%置信区间,0.955-0.987),分别。
结论:双主动脉弓患儿的临床症状与产前气管压迫有关,可以使用超声波进行产前评估。如果胎儿被诊断为双主动脉弓,气管内径的产前监测以及与z评分参考范围的比较可以提供有助于围产期临床管理的相关信息.
Double aortic arch is the most common form of complete vascular ring. The trachea and/or esophagus could be compressed by the complete vascular ring, which may lead to early respiratory and/or esophageal symptoms in children with double aortic arch. Accurate prenatal assessment of tracheal compression could provide relevant information for perinatal clinical management of double aortic arch and emergency treatment of infants with double aortic arch. The fetal trachea is filled with amniotic fluid and can be clearly visualized with prenatal ultrasound. Previous studies reported the use of prenatal ultrasound to measure the tracheal internal diameters in normal fetuses and showed a linear correlation between the fetal tracheal internal diameters and gestational age. However, to the best of our knowledge, few studies have quantitatively evaluated tracheal compression in fetuses with double aortic arch using ultrasound.
This study aimed to evaluate the tracheal compression caused by the vascular ring in fetuses with double aortic arch using prenatal ultrasound and to analyze the relationship between tracheal compression and postnatal clinical symptoms.
The data of fetuses with double aortic arch diagnosed with prenatal ultrasound at 2 institutions from January 2011 to April 2021 were retrospectively analyzed. Singleton pregnancies with normal fetuses as the control group were prospectively recruited. The tracheal compression-evaluated by comparing the tracheal internal diameter z scores against the gestational age-was assessed in fetuses with double aortic arch and in normal fetuses. The live-born infants with double aortic arch were divided into symptomatic and asymptomatic groups for the comparison of z scores. The receiver operating characteristic curve for the tracheal internal diameter z score cutoffs and prediction of symptomatic infants with double aortic arch was plotted. Intraobserver and interobserver agreements were investigated.
A total of 26 fetuses with double aortic arch were diagnosed, and 14 fetuses (53.8%) with double aortic arch were delivered alive. Among the 14 live-born infants, 7 (50.0%) were symptomatic, whereas 7 (50.0%) were asymptomatic. The tracheal internal diameter z scores were significantly lower in the double aortic arch group than in the normal groups (-0.62±1.36 vs 0.00±0.78; P<.001). The tracheal internal diameter z scores were significantly lower in the symptomatic group than in the asymptomatic group (-1.42±0.92 vs -0.49±0.96; P=.018). The area under the curve was 0.878 (95% confidence interval, 0.689-1.000). Using a tracheal internal diameter z scores cutoff of -1.21, the sensitivity was 71%, and the specificity was close to 100%. The intraclass correlation coefficients of interobserver and intraobserver agreements were 0.987 (95% confidence interval, 0.980-0.992) and 0.975 (95% confidence interval, 0.955-0.987), respectively.
The clinical symptoms in infants with double aortic arch were associated with prenatal tracheal compression, which can be prenatally evaluated using ultrasound. If fetuses are diagnosed with double aortic arch, prenatal surveillance of the tracheal internal diameters and comparison with z score reference ranges could provide pertinent information that would aid perinatal clinical management.