Fukushima Naoya, Maeda Jun, Yoshimura Yukihiro, Shibuya Kazuhiko, Nagamine Hiroki, Miura Masaru
Department of Cardiology, Tokyo Metropolitan Children's Medical Center, 2-8-29, Musashidai, Fuchu, Tokyo, 183-8561, Japan.
Department of Pediatrics, Hiratsuka City Hospital, Hiratsuka, Kanagawa, Japan.
Pediatr Cardiol. 2024 Aug 12. doi: 10.1007/s00246-024-03617-3.
To investigate the relationship between quantitative tracheal geometry and clinical course among various types of vascular ring and to identify factors correlating with symptom presentation. Patients with vascular ring diagnosed between April 2010 and December 2022 were included. All the patients were classified as type 1 (complete double aortic arch); type 2 (incomplete double aortic arch); type 3 (circumflex aorta); type 4 (right aortic arch and aberrant left subclavian artery with a left retroesophageal diverticulum of Kommerell); or type 5 (mirror-imaged right aortic arch with retroesophageal aortic diverticulum). Their clinical characteristics and quantitative variables on computed tomography (CT) were compared. Of the 50 patients enrolled, those with type 1 tended to have a smaller luminal tracheal diameter at the level of the ring. The median symptom-free survival time was shortest in this group (16.0 days [95% confidence interval (CI): 9.4-51.0]), followed by type 3 (138.0 days [95% CI: 0.0-851.4]). Type 1 (hazard ratio [HR]: 9.0; 95% CI: 2.3-35.0; P = 0.001), type 3 (HR: 4.2; 95% CI: 1.4-13.2; P = 0.013), and the percentage of tracheal narrowing in the anteroposterior projection (HR: 0.87; 95% CI: 0.78-0.96; P = 0.008) were significantly associated with symptom presentation in the time-dependent course. The aortic arch encircling the entire circumference in type 1 and high-pressure vasculature in front of the vertebral body in types 1 and 3 may contribute to raising the risk of symptom presentation.
研究不同类型血管环的气管定量几何结构与临床病程之间的关系,并确定与症状表现相关的因素。纳入2010年4月至2022年12月期间诊断为血管环的患者。所有患者分为1型(完全性双主动脉弓);2型(不完全性双主动脉弓);3型(回旋主动脉);4型(右主动脉弓伴左锁骨下动脉异常及Kommerell左食管后憩室);或5型(镜像右主动脉弓伴食管后主动脉憩室)。比较他们的临床特征和计算机断层扫描(CT)上的定量变量。在纳入的50例患者中,1型患者在血管环水平的气管腔直径往往较小。该组的中位无症状生存时间最短(16.0天[95%置信区间(CI):9.4 - 51.0]),其次是3型(138.0天[95%CI:0.0 - 851.4])。1型(风险比[HR]:9.0;95%CI:2.3 - 35.0;P = 0.001)、3型(HR:4.2;95%CI:1.4 - 13.2;P = 0.013)以及前后位气管狭窄百分比(HR:0.87;95%CI:0.78 - 0.96;P = 0.008)在时间依赖性病程中与症状表现显著相关。1型中环绕整个圆周的主动脉弓以及1型和3型中椎体前方的高压血管系统可能有助于增加症状出现的风险。