主动脉弓中断的外科治疗进展

Advances in surgical treatment of interrupted aortic arch

主动脉弓中断是一种少见的先天性心脏病,在前列腺素E1未能普遍应用于临床之前,其病死率很高。早期的手术治疗主要采用分期纠治,包括一期重建主动脉弓,二期纠治其他心内畸形,其病死率高且社会经济负担较重。从20世纪90年代起,随着外科技术及围术期监护、体外及麻醉技术的不断提高,主动脉弓中断合并其他相关心内畸形多采用一期纠治。主动脉弓中断的手术死亡率已明显降低,但针对其存在较高的远期再狭窄及左室流出道梗阻,外科治疗仍存在较大的挑战,文章就此对其外科治疗及相关进展以及有关远期并发症的发生进行综述。

Interrupted aortic arch （IAA） is a rare congenital heart disease with a very high mortality before the use of prostaglandins El. The early staged surgical procedures of IAA usually consisted of reconstruction of the aortic arch and pulmonary artery banding in the first stage, and repair of the other intracardiac lesions in the second stage. With the improvements of cardiac surgery, perisurgical monitoring, anesthesia, and extracorporeal circulation, one-stage repair of IAA has been well established and the surgical mortality has decreased markedly since the 1990s. However, re-stenosis and obstruction of the left ventricular outflow tract after operation are still two biggest challenges of IAA surgery. This review describes the progress in repair of IAA and its postsurgical complications.