肺动脉瓣缺如综合征的外科治疗

目的:探讨肺动脉瓣缺如综合征(APVS)的外科治疗经验。方法:1994年12月至2006年11月共治疗APVS患儿5例,其中男性4例,女性1例。5例均伴法洛四联症,其中1例伴左肺动脉起源异常,1例伴左肺动脉狭窄,发育不良。全组患儿均在中低温体外循环下行根治手术。其中4例患儿肺动脉瓣区置带瓣补片,1例采用牛心包直接补片。结果:4例存活,1例死亡,术后平均随访(13.8±5.76)个月。超声心动图检查示肺动脉瓣少量返流,右心室至肺动脉平均流速及压差较术前明显下降(P<0.01)。结论:APVS的外科治疗应选用带瓣补片重建右心室流出道。肺动脉严重扩张的患儿应行肺动脉成形术,以解除气道压迫症状。

Surgical treatment of absent pulmonary valve syndrome

Objective： To discuss the surgical treatment technique of absent pulmonary valve syndrome （APVS） .Method： From December 1994 to November 2006, our center had performed 5 cases of APVS correction. Those 5 patients were all associated with Tetralogy of Fallot. Among them, one patient was associated with aortic origin of the left pulmonary artery, another one was with left pulmonary artery hypogenesis. Result： Four children were survived. The follow-up period is from half year to 2 years. The pulmonary valve functioned well with mild regurgitation on echocardiography. The velocity and pressure between the right ventricle and pulmonary artery were significantly decreased, P 〈 0.01. Conclusion： It is crucial to reconstruct the right ventricle-pulmonary with single valve tissue. The technique of plication for dilating pulmonary artery may improve outcome in patients with airway compression.