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[诊断为血管环的儿科患者的诊断特征及病死情况]

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

作者信息

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

机构信息

Departamento de Cardiología Pediátrica, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

Departamento de Tomografía Cardíaca, Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

出版信息

Arch Cardiol Mex. 2025 Mar 11;95(3):197-205. doi: 10.24875/ACM.24000150.

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.

摘要

目的

分析诊断为血管环的儿科患者的临床特征和自然病史。

方法

我们回顾性研究了2014年1月至2022年12月期间110例儿科患者的记录,涉及以下变量:年龄、临床表现、相关心脏病变、诊断方法和手术方式。

结果

总共60例(55%)为女性,50例(45%)为男性。平均诊断年龄为47个月。发现的血管环类型有异常锁骨下动脉(82.8%)、主动脉弓环绕(8.1%)、双主动脉弓(6.3%)和左肺动脉吊带(1.8%)。90%的病例伴有其他先天性心脏病,主要是法洛四联症、肺动脉瓣闭锁和室间隔缺损。45%的病例出现结构受压,77%的患者无症状。21%的患者接受了手术治疗,其中35%出现术后并发症:败血症(26%)、纵隔炎(4.3%)和心源性休克(4.3%)。术后死亡率为17%。在接受观察等待治疗的有症状患者的自然病史中,100%的患者症状得到缓解。

结论

在本系列研究中,最常见的血管环类型是异常锁骨下动脉。由于其症状缺乏特异性,主要为呼吸系统症状,诊断较晚。大多数轻度受累症状的病例会随着受影响结构的生长及其松弛而缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4e67/12324841/2e5577604ed0/10971AMEX253-ACM-95-197-g001.jpg

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