单源化手术治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管

Unifocalization repair for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

目的 探讨肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的手术方式,评价其临床疗效.方法 2008年7月至2010年8月本院采用单源化手术治疗肺动脉闭锁（PA）合并室间隔缺损（VSD）及重要主肺动脉侧支血管（MAPCAs）患儿8例.男3例,女5例,年龄4～72个月,中位年龄24个月,体重7.5～15.5 kg,平均体重（11.2±2.4）kg.7例采取分期于术治疗,1例采取一期根治手术.结果 全组患儿均存活.随诊6～20个月,1例一期根治手术患儿痊愈;7例分期手术患儿,临床紫绀症状均好转.其中3例肺动脉明显发育,进一步行二期根治手术治愈.结论 单源化手术是治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的有效方法,分期手术可取得良好的临床疗效.

Objective To review the opertion experience and outcome of unifocalization repairs for PA with VSD and MAPCAs. Methods From Jul. 2008 to Aug. 2010, 8 patients （ male 3, female 5 ） with age ranged from 5 to 72 （median 8.2） months and body weight ranged from 4.5 to 13 （mean 8.7 ±2.8） kg were treated with unifocalization repairs. One case underwent radical operation. The other 7 cases underwent a multistaged repair approach. Results There were no deaths, all cases were being referred to the outpatient department for follow-up which lasted for 6 to 20 months, When reasonable pulmonary vascularization has been obtained, there were 3 cases were treated with complete repair with transventricular closure of the VSD and right ventricular outflow tract reconstruction. Conclusions Unifocalization as a palliative operation is a useful method for patients PA with VSD and MAPCAs. Staged repair yields a relatively low mortality rate with good functional results.