一期手术矫治先天性主动脉弓中断

One-stage Repair of Interrupted Aortic Arch

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。

ObjectiveTo review the clinical experience with one-stage repair of interrupted aortic arch (IAA). MethodsTen patients with IAA underwent one-stage total repair, mean surgical age was 2.7±2.4 years, five infants had type A IAA and 3 had type B, and 2 IAA had the 5th arch stenosis. Eight IAA had associated cardiac anomalies and severe pulmonary hypertension. Seven patients had direct end-side or end-end anastomosis, two had a bypass with Gore-Tex graft between approximate of IAA and descending aortic artery, and one had enlargement of the 5th arch with autopericardium patch. All of the associated cardiac defect were repaired.ResultsThere were 5 cases with early postoperative complications, only one in the last 10 years, and 3 patients died in hospital (in recent 10 years no hospital death). Pulmonary systolic pressure/systemic systolic pressure (Pp/Ps) with 5 cases was significantly decreased from 0.84±0.04 before operation to 0.28±0.03 in the early post-operation. The 7 survivors were followed up for a mean of 2.6±4.0 years, there were no late death, and 3 of them had residual pressure gradient more than 30 mmHg, but normal heart function in all. ConclusionsIt is necessary to one-stage repair of IAA with associated cardiac anomalies as early as possible, once diagnosis is established. There were good results with direct aortic arch anastomosis.