一期纠治主肺动脉窗或右肺动脉起源于升主动脉

目的总结主肺动脉窗或右肺动脉起源于升主动脉的外科纠治经验。方法17例病儿中单纯主肺动脉窗5例,右肺动脉起源于升主动脉4例,主肺动脉窗伴主动脉弓中断、法洛四联症、房室间隔缺损、二尖瓣反流和气管狭窄等8例。1例无手术指征,16例行解剖纠治,平均手术年龄1．7岁;平均体重8．5 kg。结果手术无死亡。2例延迟关胸;1例再进胸止血。随访无死亡,无主动脉瓣上狭窄及肺动脉狭窄。结论主肺动脉窗及右肺动脉起源于升主动脉易早期产生肺动脉高压,一经诊断需立即手术。手术方式首选与伴发畸形一期纠治。主肺动脉窗及右肺动脉起源于升主动脉尽管多有伴发畸形,但早期手术纠治可获得较佳中、长期疗效。

Surgical repair of aortopulmonary window and aortic origin of right pulmonary artery

Objective The prupose of this study is to describe our approach to the management of aortopulmonary window and aortic origin of right pulmonary artery. Methods From Apr. 2000 to Jan. 2006, 17 patients were hospitalized. The mean age was 1.7 years and mean body weight was 8.5 kg. The associated cardiac abnormalities included ventrieular septal defect, atrial septal defeet, interrupted aortic arch, tetralogy of Fallot, mitral valve regurgitation and tracheal stenosis. Results 16 patients underwent the single stage surgical repair and all survived. 1 patient had postoperative bleeding and received re operation. During follow-up, all patients are doing well. There were no supra-valve aortic stenosis and pulmonary stenosis. Conclusion For most patients with aortopulmonary window and aortic origin of right pulmonary artery, repair should be undertake at the time of diagnosis because of the risk of puhmonary vasctdar occlusive disease, Inpatients with associated abnormalities, single stage repair of all defects are recommended. Surgical repair of aortopulmonary window and aortic origin of right pulmonary artery, even when associated with other cardiac abnormalities, carries a low risk. Early surgical treatment would achieve excellent immediate and long-term results.