【摘要】 目的　总结手术治疗肺血减少型复杂先天性心脏病的外科经验。方法　对 112例复杂紫绀型先天性心脏病伴有肺动脉狭窄 (PS)病人手术的临床资料分析。年龄 2～ 2 3岁 ,平均 9.5 7± 4.84岁 ,体重 :8.1～ 5 4kg ,平均 2 2 .7± 11.36kg。复杂心内畸形 :法洛四联症 (TOF) 81例 ,右室双出口 (DORV ) 14例 ,大动脉转位 (TGA) 11例 ,房室管畸形(AVCD) 4例 ,单心室 (SV)及Ebstein畸形各 1例。PS包括 :1.漏斗部狭窄 93例 ,其中伴肺动脉瓣狭窄 47例 ,瓣、环及干狭窄 38例 ,左肺动脉口狭窄及缺如各 1例 ;2 .单纯肺动脉瓣狭窄 5例 ;3.肺动脉瓣、环、干狭窄 14例 ,伴左右肺动脉细小2例。手术方法 :TOF根治术 81例 ,DORV根治术 13例 ,TGA心内畸形纠治术 7例 ,AVCD纠治术 3例 ,双向Glenn手术 2例 ,Rastelli手术、心外管道全腔静脉 -肺动脉接术、Senning手术、改良Fontan手术、Ebstein畸形纠治术及探查术各1例。合并PS手术 :瓣交界切开扩张瓣环 5 8例 ,流出道 (RVOT)直接缝合 4例 ,RVOT加宽心包补片 5 2例 ,跨环心包补片 48例 ,同种主动脉带瓣外管道右室 -肺动脉连接 1例 ,结果　全组手术死亡率 6 .3%(7/112例 ) ,其中TOF死亡率为4.9%(4/81例 )。死亡原因 :低心排综合征 3例 ,RH(-)血型创面广泛渗血、血源缺乏 ,脑气栓 ,?更多还原

【Abstract】 Objective To evaluate the surgical experience for the treatment of complex congenital heart anomalies with pulmonary stenoses(PS).Methods In 112 patients with complex congenital heart diseases associated PS were surgically treated.Cardiac anomalies included tetralogy of Fallot (TOF) in 81 cases,double outlet right ventricle(DORV) in 14,transposition of the great arteries (TGA) in 11,atrioventricular canal defects(AVCD) in 4,single ventricle in 1,Ebstein’s malformation in 1.The PS included obstruction of right ventricular outflow tract in 93 cases,with (pulmonary valve stenoses in 47,valve、ring and trunk stenoses in 38,left pulmonary branch stenoses in 1 and atresia in 1);pure pulmonary stenoses in 5;pure pulmonary valve、ring and trunk stenoses in 14.The repair procedures were performed for the radical operation of TOF in 81 patients ,DORV in 13;for the corrective operation of TGA in 7,AVCD in 3;Rastell in 1 and othe surgical procedure were underwent Glenn in 2、 Extracardiac conduit total cavopulmonary connection 、Senning 、Fontan 、Ebstein and surgical explorative procedure in 1,respectively.The surgical treatment of the PS with intracardiac anomalies included 52 right ventricular outflow tract reconstructions by a patch of autologus pericardium,48 by a transannular patch of autologus pericardium,1 by aortic homografts valved conduit.Results There were 7 operative deaths with a mortality rate of 6.3%.The postoperative complications occurred in 15 patients recovered uneventfully.There were 2 late deaths.The remaining patients remain well.Conclusion Depending upon hypoplasia of pulmonary vessels and left ventricle with degree of complex intracardiac anomalies,the surgical procedures included in radical operation、corrective operation and palliative operation can be achieved with excellent the clinical outcome.更多还原