[Diagnostic characterization and morbi-mortality in pediatric patients with diagnosis of vascular ring].

José L Castro-Machuca, Andrés M Tavera-Paredes, Francisco Castillón-Castillo, Gloria Juárez-Vázquez, Alfonso Buendía-Hernández
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Abstract

Objective: To analyze the clinical characteristics and natural history of pediatric patients with a diagnosis of vascular ring.

Method: We retrospectively studied the records of 110 patients of pediatric age, from January 2014 to December 2022, with the following variables: age, clinical manifestations, associated cardiac lesions, diagnostic method and surgical approach.

Results: Of the total, 60 (55%) were female and 50 (45%) were male. Diagnosis was made, on average, at 47 months of life. The vascular rings found were aberrant subclavian artery (82.8%), circumflex aorta (8.1%), double aortic arch (6.3%) and left pulmonary artery sling (1.8%). Ninety percent of the cases were associated with other congenital heart disease, mainly tetralogy of Fallot, pulmonary valve atresia and ventricular septal defect. Forty-five percent of the cases produced compression of structures and 77% of the patients were asymptomatic. Twenty-one percent of the patients received surgical treatment of which 35% presented postoperative complications: sepsis (26%), mediastinitis (4.3%) and cardiogenic shock (4.3%). Postoperative mortality was 17%. Within the natural history of symptomatic patients who remained in expectant management, 100% resolved their symptoms.

Conclusions: In this series, the most frequent type of vascular ring was aberrant subclavian artery. Diagnosis is late due to the lack of specificity of its symptoms, being mainly respiratory. The symptoms of mild involvement resolve in most cases, associated with the growth of the affected structures and their laxity.

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[诊断为血管环的儿科患者的诊断特征和死亡率]。
目的:分析诊断为血管环的儿科患者的临床特点及自然病史。方法:回顾性分析2014年1月至2022年12月110例小儿年龄患者的临床资料,包括年龄、临床表现、相关心脏病变、诊断方法、手术入路等。结果:女性60例(55%),男性50例(45%)。确诊时间平均为47个月。血管环以锁骨下动脉异常(82.8%)、旋主动脉(8.1%)、双主动脉弓(6.3%)、左肺动脉悬吊(1.8%)为主。90%的病例合并其他先天性心脏病,主要为法洛四联症、肺动脉瓣闭锁和室间隔缺损。45%的病例产生结构压迫,77%的患者无症状。21%的患者接受手术治疗,其中35%出现术后并发症:脓毒症(26%)、纵隔炎(4.3%)和心源性休克(4.3%)。术后死亡率为17%。在有症状的患者的自然史中,仍在期待治疗,100%解决了他们的症状。结论:在本组病例中,最常见的血管环类型为异常锁骨下动脉。由于其症状缺乏特异性,诊断较晚,主要是呼吸道。在大多数情况下,轻度受累的症状会消退,与受累结构的生长及其松弛有关。
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