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气管和左肺动脉狭窄指数与气管狭窄伴肺动脉吊带预后的关系。

Relationship between a Tracheal and Left Pulmonary Artery Stenosis Index and the Prognosis of Pulmonary Artery Sling with Tracheal Stenosis.

机构信息

Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.

出版信息

Pediatr Cardiol. 2021 Oct;42(7):1585-1593. doi: 10.1007/s00246-021-02643-9. Epub 2021 May 27.

Abstract

Pulmonary artery sling is a rare congenital pulmonary vascular malformation, often associated with tracheal or bronchial stenosis. Surgical treatment of pulmonary artery sling with tracheal stenosis (T) has a high risk of death and a relatively poor prognosis. This study explored the relationship between the T and left pulmonary artery stenosis (P) ratio and the effects of surgery for pulmonary artery sling with tracheal stenosis. Patients undergoing surgery for pulmonary artery sling in our center from January 2010 to December 2018 were retrospectively analyzed. Routine cardiac-enhanced computed tomography (CT) was performed preoperatively, and the P and T diameters were measured on the CT image. The T/P ratio was then calculated and analyzed. Thirty cases of pulmonary artery sling combined with tracheal stenosis were operated under cardiopulmonary bypass. The mean age at operation was 13.8 ± 13.6 months (1.2-57.1 months, Q1-Q3: 5-17 months), and the mean body weight was 8.8 ± 3.5 kg (3.8-17.3 kg, Q1-Q3: 5.8-11.5 kg). Twenty-three patients survived, and seven died, with an overall survival rate of 76.7%. Twenty-four patients underwent left pulmonary artery re-implantation, six patients underwent simultaneous left pulmonary artery re-implantation and slide tracheoplasty, and patients with intracardiac malformations (ventricular septal defect (n = 6) and atrial septal defect (n = 4)) underwent concurrent repair of the intracardiac defect. All cases had different degrees of tracheal stenosis, and the most narrowed trachea occurred with compression by the left pulmonary artery sling. The T/P ratio in the tracheoplasty group was significantly higher than that in the non-tracheoplasty group. The mortality rate in the T/P ≤ 1.15 group was significantly lower than that in the T/P > 1.15 group. Pulmonary artery sling treatment has a high risk of death. Left pulmonary artery re-implantation is an effective and safe surgical method for treating pulmonary artery sling, and slide tracheoplasty is an effective surgical method to correct tracheal stenosis. Children with T > 78.4% should receive active intervention for the tracheal stenosis, and these children have a considerable postoperative survival rate. The T/P ratio can be used to compare the relative superiority of the two compressions. A T/P ratio > 1.15 can be used as a reference index for intervention in tracheal stenosis and is a risk factor for postoperative death.

摘要

肺动脉吊带是一种罕见的先天性肺血管畸形,常伴有气管或支气管狭窄。对于伴有气管狭窄(T)的肺动脉吊带的手术治疗具有很高的死亡率和相对较差的预后。本研究探讨了 T 与左肺动脉狭窄(P)比值与肺动脉吊带合并气管狭窄手术的关系。回顾性分析 2010 年 1 月至 2018 年 12 月在我院接受肺动脉吊带手术的患者。术前常规行心脏增强 CT(CT)检查,在 CT 图像上测量 P 和 T 直径。然后计算并分析 T/P 比值。30 例肺动脉吊带合并气管狭窄患者在体外循环下手术。手术时的平均年龄为 13.8±13.6 个月(1.2-57.1 个月,Q1-Q3:5-17 个月),平均体重为 8.8±3.5kg(3.8-17.3kg,Q1-Q3:5.8-11.5kg)。23 例患者存活,7 例死亡,总存活率为 76.7%。24 例行左肺动脉再植入术,6 例行同期左肺动脉再植入和滑气管成形术,伴有心内畸形(室间隔缺损(n=6)和房间隔缺损(n=4))的患者同期修复心内缺损。所有患者均有不同程度的气管狭窄,最狭窄的气管发生在左肺动脉吊带压迫处。气管成形组 T/P 比值明显高于非气管成形组。T/P≤1.15 组死亡率明显低于 T/P>1.15 组。肺动脉吊带治疗的死亡率较高。左肺动脉再植入术是治疗肺动脉吊带的有效、安全的手术方法,滑气管成形术是纠正气管狭窄的有效手术方法。T>78.4%的患儿应积极干预气管狭窄,这些患儿术后有相当高的存活率。T/P 比值可用于比较两种压迫的相对优势。T/P>1.15 可作为气管狭窄干预的参考指标,是术后死亡的危险因素。

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