Abstract

 

OBJECTIVES

Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division.

METHODS

A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed.

RESULTS

The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up.

CONCLUSIONS

The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.

中文翻译：

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儿童血管环分裂的长期结果：多机构经验

摘要

 

目标

血管环是先天性心脏病的罕见异常，可引起呼吸道和胃肠道症状。本研究评估了血管环分裂患者的长期预后。

方法

对 2007 年 11 月至 2019 年 10 月在 3 个儿科心脏机构进行手术分割的 371 例血管环患者进行了多机构回顾性研究。

结果

完整的血管环由双主动脉弓（24.5%）、右主动脉弓和左动脉韧带（36.7%）和左主动脉弓和右动脉韧带（0.5%）组成。不完整的血管环包括肺动脉吊带（22.9%）、左主动脉弓伴右锁骨下动脉异常（15.1%）和无名动脉受压综合征（0.3%）。呼吸道症状包括喘鸣（71.4%）、喘息（49.1%）、咳嗽（31.5%），胃肠道症状包括窒息（12.4%）、吞咽困难（3.2%）和呕吐（1.9%）。只有一名患者在出院后死亡，晚期死亡率为 0.3% (1/360)。10年总生存率为96.8%。51 例患者报告了术后并发症，其中 15 例需要再次手术。10年无再手术率为95.9%。95年完成随访。4% (354/371) 的患者，平均随访时间为 4.3 ± 2.9 年（范围为 1 至 13 年）。20 名患者（5.6%）在长期随访期间出现残留症状。

结论

血管环分割的结果非常好。Kommerell 憩室 > 1.5 倍于异常的左锁骨下动脉起源是初次切除的手术指征。气管软化是再手术和残留症状的危险因素，术前纤支镜检查对评估很重要。