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目的探讨联合右心房-肺动脉切口径路手术一期根治婴幼儿法洛四联症(TOF)的疗效,总结其临床经验。方法回顾性分析2006年1月至2012年1月解放军第91中心医院采用联合右心房-肺动脉切口径路行一期根治术治疗69例婴幼儿TOF的临床经验,其中男47例,女22例;年龄5个月至2岁7个月;体重5～16 kg。心脏超声心动图提示:左心室舒张期末容积指数(LVEDVI)24.5～36.9 ml/m2,Nakata指数>120 mm2/m2,McGoon比值1.15～2.20。结果围术期死亡2例,死亡率2.9%,其中术后死于肺部感染1例,渗漏综合征1例。术后患者血流动力学平稳,不需使用大剂量的血管活性药物。呼吸机辅助呼吸时间(11.7±9.3)h,住ICU时间(38.2±20.7)h。生存的67例患者均顺利出院,动脉血氧饱和度由术前平均85%上升至99%,临床症状改善,活动耐量增加,心功能(NYHA分级)恢复至Ⅰ～Ⅱ级。随访67例,随访率100%,随访时间11～70个月。随访期间无死亡,1例于术后3个月因大量心包积液行心包穿刺引流,1例于术后8个月因肺动脉瓣狭窄再次行手术矫治,其余患者恢复良好,心功能(NYHA分级)Ⅰ～Ⅱ级。结论联合右心房-肺动脉切口径路手术一期根治婴幼儿TOF安全、可行,较常规手术缩短了体外循环时间,减少了右心室创伤,避免患者远期右心室功能衰竭、室性心律失常导致的猝死,有利于患者远期生存,效果满意。
Objective To investigate the curative effect of primary right atrial-pulmonary artery bypass grafting in the treatment of tetralogy of Fallot (TOF) in infants and young children, and to summarize its clinical experience. Methods A retrospective analysis of clinical experience of 69 cases of infants with TOF treated with combined right atrium-pulmonary artery approach at 91st Central Hospital of PLA from January 2006 to January 2012 was retrospectively reviewed. There were 47 males and 22 females ; Age 5 months to 2 years and 7 months; weight 5 to 16 kg. Echocardiographic echocardiography suggested that the left ventricular end-diastolic volume index (LVEDVI) was 24.5-36.9 ml / m2, the Nakata index was> 120 mm2 / m2, and the McGoon ratio was 1.15-2.20. Results Perioperative mortality in 2 cases, the mortality rate of 2.9%, of which 1 died of pulmonary infection after surgery, 1 case of leakage syndrome. Postoperative hemodynamic stability, do not need to use large doses of vasoactive drugs. Ventilator assisted breathing time (11.7 ± 9.3) h, ICU time (38.2 ± 20.7) h. Survival of 67 patients were successfully discharged, arterial oxygen saturation increased from 85% preoperative to 99%, clinical symptoms improved, increased activity tolerance, cardiac function (NYHA classification) returned to Ⅰ ~ Ⅱ level. Follow-up of 67 cases, follow-up rate was 100%, followed up for 11 to 70 months. One died of pericardial drainage at 3 months after operation due to a large amount of pericardial effusion. One patient underwent reoperation due to pulmonary valve stenosis at 8 months postoperatively. The rest of the patients recovered well and cardiac function (NYHA classification ) Ⅰ ~ Ⅱ level. Conclusions It is safe and feasible to treat TOF in infants and children in the first phase of right atrium-pulmonary artery bypass grafting. Compared with conventional surgery, it can shorten the time of cardiopulmonary bypass, reduce the right ventricular trauma and avoid the long-term right ventricular failure and ventricular arrhythmia Sudden death, is conducive to long-term survival of patients with satisfactory results.