摘要:
目的 总结年龄大于6个月的伴室间隔缺损合并重度肺动脉高压完全性大动脉转位患儿诊断性治疗-根治性手术策略的应用经验及术后效果,探讨手术指征.方法 2010年1月至2011年10月手术治疗17例伴室间隔缺损合并重度肺动脉高压完全性大动脉转位患儿,男13例,女4例.中位年龄1.2岁,其中0.5岁~<1.0岁6例,≥1.0岁~<3.0岁3例,≥3.0岁8例.合并动脉导管未闭6例,房间隔缺损5例,二尖瓣关闭不全2例,肺动脉瓣轻度狭窄2例.术前均行超声心动图检查,冠状动脉CT检查11例,右心导管检查3例.全组均行诊断性治疗2~4周,静吸复合麻醉低温体外循环下行大动脉调转术(ASO),术后残留肺动脉高压者继续予肺动脉高压靶向药物治疗.结果 全组无手术死亡.术前经诊断性治疗动脉氧饱和度提高10% ~21%,肺动脉平均压下降10 ~20mmHg(1.33 ~2.67kPa).随访6~32个月,平均11.2个月.随访期间1例死于食物中毒致急性腹泻、电解质紊乱和心律失常,余患儿至最终随访日均生存.术后6例(35.29%)残余肺动脉高压,年龄均≥3岁,肺动脉高压靶向药物治疗6 ~20个月后,肺动脉压力明显下降.结论 大于6个月的伴室间隔缺损肺动脉高压完全性大动脉转位患儿经诊断性治疗后可以选择性实施根治性手术(ASO),效果良好.%Objective To analyze and summarize the applicative experience and operative effective of the diaguostictreatment-repair strategy in the transpossion of great arteries(TGA) infants with ventricular septal defect and severe pulmonary hypertension more than 6 months.Methods From January 2010 to October 2011,17 TGA cases with ventricular septal defect and severe pulmonary hypertension.There were 13 male and 4 female.≥0.5-< 1.0 years old 6 cases,≥ 1.0-< 3.0 years old 3,≥3.0 years old 8 cases.Combine anomalies: patent ductus arteriosus in 6 cases,atrial septal defect in 5 cases,valve insufficency in 2 cases.All preoperative cases were performed echocardiography,right-sided heart catheterization 3 cases,coronary CT examinationll cases.After diagnostic-treatment 2-4 weeks,all cases performed arterial switch operation under compound intravenous and inhaled anesthesia.Results No operative death.After diagnotic-treatment,SPO2 improved 10%-21%,and mPAP decreased 10-20 mm Hg.Follow-up 11.2 (6,20) months,one dead.Postoperative residual pulmonary arterial hypertension in 35.29%,6/17cases,all of them were ≥3 years old.Continue to pulmonary arterial hypertension targeted drugs treatment for 6-20 months later,pulmonary artery pressure decreased obviously.Conclusion The TGA infants with ventricular septal defect pulmonary arterial hypertension more than 6 months,can be selectively performed arterial switch operation under went diagnostic-treatment-repair strategy,continue to pulmonary arterial hypertension targeted drug therapy postoperation,the effect is good.