改良外管道Fontan术治疗心脾综合征型复杂先天性心脏病  

Modified extracardiac conduit Fontan procedure for heterotaxia syndrome with complex congenital heart disease

作　　者：仇黎生[1] 徐志伟[1] 苏肇伉[1] 

机构地区：[1]上海第二医科大学附属新华医院上海儿童医学中心心胸外科,200127

出　　处：《中华胸心血管外科杂志》2005年第3期133-135,共3页Chinese Journal of Thoracic and Cardiovascular Surgery

摘　　要：目的　回顾总结改良外管道Fontan术治疗无脾或多脾综合征伴发的复杂型先天性心脏病(先心病)经验。方法　青紫型复杂先心病病儿11例,无脾9例(右房异构,单心室合并共同房室瓣6例,房室连接不一致的右室双出口2例,纠正性大动脉错位1例) ;多脾2例(左房异构,右室双出口合并共同房室瓣)。平均年龄(6 3±3 7)岁,平均体重(2 1 0±5 5 )kg。直接行改良外管道Fontan术3例,双向Glenn术后行二期外管道Fontan术8例。结果　术后早期发生低心排出量综合征2例,肾衰1例,室上性心动过速1例,胸腔引流(12 0ml d) >10d 2例。超声提示上腔吻合口通畅(流速0 6～0 8m s) ,下腔血流速度0 3～0 5m s ;动脉血氧饱和度9例在0 92～0 95 ,2例为0 86 ;活动能力明显改善,无早期死亡。随访中超声显示上腔静脉血流速度0 8m s,下腔静脉血流速度0 4～0 7m s ,随呼吸而变化。外管道通畅,无血栓形成,管道窗口直径0 34cm ,血流右向左分流。肺静脉回流无梗阻,心室射血分数>0 6 0。心电图未见明显心律失常。生长发育正常,活动自如,经皮氧饱和度>0 90 ,无慢性渗出、蛋白丢失肠病等并发症,无中期死亡。结论　改良外管道Fontan术适用于治疗无脾或多脾综合征型复杂先心病,术后心律失常发生率低。Objective To review and summarize the experiences of modified exlraeardiac conduit Fontan operation for heterotaxia syndrome with complex congenital heart disease. Methods There were 11 patients with cynosis complex congenital heart disease, 9 were aspleenia syndrome （fight atrium isomerism, including 6 single ventricle with cormnon atrioventricular valve, 2 doutble outlet right ventricle with atrioventricular discordance, and 1 corrected transposition of the great arteries）, and 2 were pelyspleenia （ left atrium isomerism, double outlet right ventricle with common atrioventricular valve）. The mean age was （6.3 ± 3.7）year-old, the mean body weight was （21.0 ± 5.5）kg. 3 patients underwent one-stage modified extracardiac conduit Fontan procedure, 8 patients after bi-directional Glenn operation underwent two-stage procedure. Results During early postoperative period, two patients had low cardiac output syndrome, 1 renal dysfunction; and 1 supraventriele taehyeardiac. The time of chest drainage （ 120 ml/d） was more than 10 days in 2 patients. 2D-echo showed that superior vena cava blood flow rate was 0.6 to 0.8 m/s, inferior vena eava flow rate was 0.3 to 0.4 m/s. Oxygen saturation were from 0.92 to 0.95 in room air in 9 patients, 2 patients were under 0.86. The exercise capacity was significantly improved. All patients were survived and no early death. At follow-up ranging from 6 months to 2 years, supra vena cava blood flow rate was 0.8 m/s, inferior vena eava flow rate is 0.4 to 0.7 m/s. There was no thrombus formation in the conduit. The diameter of fenestration was 0.34 cm and blond flow from right to left. No pulmonary vein drainage obstruction. Heart function was normal. No atrial arrhythemia were detected. The oxygen saturation in the room air was more than 0.90. No chronic effusion and protein-losing enteropathy, no mid-term death. Condusion Modified extracardiac conduit Fontan operation is suited for heterotaxia syndrome with complex congenital heart disease. The incidence of arrhythem

关 键 词：外管道Fontan术 手术治疗 心脾综合征 复杂先天性心脏病 心电图 心律失常 

分 类 号：R726.5[医药卫生—儿科]