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血管环:临床表现、影像学检查策略、治疗及预后

Vascular rings: presentation, imaging strategies, treatment, and outcome.

作者信息

Kir Mustafa, Saylam Gul Sagin, Karadas Ulas, Yilmaz Nuh, Çakmakçi Handan, Uzuner Nevin, Güzeloğlu Mehmet, Ugurlu Baran, Oto Öztekin

机构信息

Department of Pediatric Cardiology, Faculty of Medicine, Dokuz Eylul University, Inciralti, Izmir 35340, Turkey.

出版信息

Pediatr Cardiol. 2012 Apr;33(4):607-17. doi: 10.1007/s00246-012-0187-x.

Abstract

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell’s diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors’ diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.

摘要

本研究旨在评估血管环的临床表现、影像学检查方法的有效性及手术治疗情况。分析了一家三级转诊中心10年间44例患者(32例为前瞻性纳入,12例为回顾性分析)的数据。这些患者包括25例左位主动脉弓并右锁骨下动脉异常的患者、13例右位主动脉弓并起源于Kommerell憩室的左锁骨下动脉的患者、1例右位主动脉弓并左锁骨下动脉异常的患者、3例双主动脉弓的患者以及2例肺动脉吊带的患者。25例患者有呼吸系统症状,6例有吞咽困难。5例患者出现非典型症状,如反射性呼吸暂停、发绀、晕厥发作及运动诱发的喘息。41%的患者检测出合并先天性心脏缺陷。食管钡餐造影的诊断率为95.23%,超声心动图为54.54%,计算机断层扫描为66.66%。97.43%的患者通过磁共振成像(MRI)、90.5%的患者通过血管造影能够正确识别解剖结构。在接受手术的30例患者中,平均随访25±33.5个月,80%的患者症状完全缓解。对于有非典型症状的婴儿,血管环不应被忽视。作者选择的诊断方法是MRI,因为在描绘异常血管结构、气管和食管之间的关系方面,它优于血管造影。

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