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目的 探讨先天性主动脉弓中断 (IAA)一期手术矫治的手术方法、疗效 ,总结其临床经验。 方法 对10例少见的先天性 IAA进行一期手术矫治 ,平均手术年龄 2 .7± 2 .4岁 ,其中 5例为 A型 IAA,3例为 B型 ,另 2例IAA合并残存第 5弓狭窄 ;8例患者均合并其它心血管畸形和重度肺动脉高压。一期矫治术中有 7例进行了主动脉弓直接端侧或端端吻合连接术 ,2例行 Gore- Tex管道连接重建主动脉弓 ,1例 IAA合并残存第 5弓狭窄用自身心包补片作狭窄处扩大成形术 ;8例患者于矫治 IAA的同时矫治心血管其他畸形。 结果 术后早期发生心功能不全、心律失常、肺动脉高压危象等并发症 5例 ,其中近 10年仅发生 1例。住院死亡 3例 ,近 10年连续 6例无住院死亡。术后早期 5例肺动脉收缩压 /体循环动脉收缩压 (Pp/ Ps)由术前的 0 .84± 0 .0 4显著下降至正常范围 (0 .2 8± 0 .0 3) ,1例主动脉弓部压力阶差为 30 mm Hg(1k Pa=7.5 mm Hg)。随访 7例 ,平均随访 2 .6± 4 .0年 ,均存活 ,其中有 3例主动脉弓部压力阶差≥ 30 mm Hg。心功能均正常。 结论 先天性 IAA一旦诊断明确 ,应尽早进行一期矫治术 ;主动脉弓直接吻合连接术效果较佳。
Objective To investigate the surgical method and curative effect of primary correction of congenital aortic arch interruption (IAA) and summarize its clinical experience. Methods One case of 10 rare cases of congenital IAA underwent surgery. The mean operative age was 2.7 ± 2.4 years. Among them, 5 cases were type A IAA, 3 cases were type B and 2 cases were IAA with residual fifth arch Narrow; 8 patients were combined with other cardiovascular malformations and severe pulmonary hypertension. In the first phase, 7 cases underwent aortic arch direct anastomosis or end-to-end anastomosis, 2 cases underwent Gore-Tex duct reconstruction of the aortic arch, 1 case of IAA with residual arch 5 stenosis with its own pericardial patch for stenosis 8 cases of patients with correction of IAA at the same time other cardiovascular malformations. Results Early postoperative cardiac dysfunction, arrhythmia, pulmonary hypertension risk complications such as 5 cases, of which only 10 cases occurred in 1 case. In-hospital death in 3 cases, nearly 6 consecutive cases of in-hospital deaths in recent 10 years. The pulmonary artery systolic pressure / systemic systolic pressure (Pp / Ps) decreased significantly from 0. 84 ± 0. 0 4 in preoperation to normal range (0.28 ± 0.30) and 1 case The pressure gradient is 30 mm Hg (1k Pa = 7.5 mm Hg). All the patients were followed up for 7 years and were followed up for an average of 2.6 ± 4.0 years. Three of them had aortic arch pressure gradient ≥ 30 mm Hg. Heart function are normal. Conclusions Once the diagnosis of congenital IAA is clear, an orthodontic treatment should be carried out as soon as possible. The aortic arch direct anastomosis is better.