肺血管发育不良性法洛四联症的分期治疗

目的:探讨肺血管发育不良性法洛四联症的治疗策略。方法:2007年11月至2013年12月,29例TOF因肺血管发育不良行分期手术,男性12例,女性17例。一期手术中位年龄15个月;体质量(10.7±5.0)kg;二期手术中位年龄36个月,平均体质量(14.7±5.1)kg。结果:全组无手术死亡。根治病例一期手术和二期手术平均间隔(21.8±9.4)个月。一期手术共计29例,结扎侧枝2例共5支,侧枝融合1例,McGoon比值由(0.78±0.13)增至(1.50±0.20)。两次手术间行肺动脉瓣球囊扩张5例。根治术后发生胸腔积液6例,灌注肺3例,肺不张2例,低心排出量综合征(低心排)与二次开胸止血各1例,均顺利康复出院。结论:肺血管发育不良性TOF分期手术安全有效,一期术后应根据肺血管发育情况适时行二期手术。

Staged surgical treatment of tetralogy of Fallot with severe pulmonary artery hypoplasia

Objective： To summarize the strategies of staged surgical treatment of Tetralogy of Fallot（ TOF） with severe pulmonary artery hypoplasia. Methods： 29 TOF with severe pulmonary artery hypoplasia underwent staged surgical treatment from November 2007 to December 2013. There were 12 male and 17 female. The median age at the first stage operation was 15 months; and weighted（ 10. 7 ± 5. 0） kg. The median age and the mean body weight of the second operation were 36 months and（ 14. 7 ± 5. 1） kg. Twenty-six patients received complete surgical correction eventually,and two patients received twice systemic-pulmonary shunts respectively. Only one did not underwent the second stage operation yet. Results： There was no hospital death. The interval between the first stage operation and the second stage was（ 21. 8 ± 9. 4） months. The McGoon index before the first stage operation was（ 0. 78 ± 0. 13）,and the index before the second operation was（ 1.50 ±0.20）. There were five cases received interventional pulmonary valve expansion,all of whom received complete correction eventually. The increase of McGoon of the five children was significant higher than that of the other patients who underwent complete surgical correction eventually（ 0. 936 ± 0. 155） vs.（ 0. 671 ± 0. 225）（ P =0.0206）. Conclusion： The staged treatment of TOF with severe pulmonary artery hypoplaisa is safe and effective. Regular and timely following-up is essential for evaluating the growth of pulmonary artery.