65例成人法洛四联症的外科治疗

目的:总结成人法洛四联症(tetralogy of Fallot,TOF)的外科治疗经验,分析影响远期预后的因素。方法:2006年02月至2012年10月,共有65例成人TOF患者接受外科根治手术,其中男性30例,女性35例,年龄16~54岁,平均(26.70±10.50)岁,体质量35~65kg,平均(43.70±8.40)kg,术前血氧饱和度65%~95%,平均(75.40±12.60)%,血红蛋白135~247g/L,平均(164.30±35.10)g/L。术前经超声心动图、双源CT心脏大血管成像(CTA)确诊。均经胸骨正中切口,低温体外循环下行TOF根治术,42例行右心室流出道补片加宽,23例采用跨瓣环补片加宽肺动脉和右心室流出道,其中2例合并中-重度主动脉瓣关闭不全,同期行主动脉瓣置换术,1例合并三尖瓣狭窄行三尖瓣置换术。结果:体外循环时间70~162min,平均(94.60±25.30)min,主动脉阻断时间36~94min,平均(53.40±20.50)min,手术时间153~264min,平均(210.60±38.70)min。该组患者死亡2例,病死率3.1%,均死于多脏器衰竭。低心排出量综合征(低心排)7例(10.8%);6例出现灌注肺(9.2%),4例行体肺侧枝栓塞术;再次气管插管3例(4.6%),气管切开2例(3.1%);二次开胸止血4例(6.2%);胸腔积液12例(18.5%);心律失常8例(12.3%)。随访60例,随访时间6~63个月,平均(23.70±8.20)个月,4例术后6个月发现室间隔缺损残余漏(6.2%),2例行二次手术,其中1例开胸行修补手术,另1例行封堵术。肺动脉瓣跨瓣压差40 mmHg(1mmHg=0.133kPa)4例,重度肺动脉瓣反流3例,三尖瓣中度反流2例。1例术后18个月出现频发室性早搏。60例患者心功能(NYHA)均恢复至I~II级,无晚期死亡。结论:成人TOF手术治疗效果满意,积极采取相应措施,可降低并发症并改善预后。

Surgical treatment of tetralogy of Fallot in 65 adults

Objective：To summary the experiences of surgical treatment of TOF （tetralogy of Fallot） in adults and evaluate the influencing factors of long-term prognosis. Methods ： From February 2006 to October 2012,65 adult patients underwent radical correction of TOF. Among them,there were 30 males and 35 females with ages ranged from 16 to 54 years, mean （26. 70 ± 10. 50）years, body weight at 35 -65kg, mean （43.70 ± 8.40） kg, preoperative oxygen saturation of blood 65 % -95 %, mean（75.40 ± 12. 60）% and hemoglobin 135 - 247g/L,mean（ 164. 30 ±35. 10）g/L. All patients were diagnosed by echocardiography and dual source cardiac CT angiography （CTA） before operation. All patients received radical correction of TOF in the hypothermia cardiopulmonary bypass （CPB）through median sternotomy. A total of 23 patients had transannular right ventric ular outflow tract patch and 42 patients had a non-transannular patch. Among them, two patients with aortic in sufficiency underwent aortic valve replacement, and one with tricuspid stenosis underwent tricuspid valve re placement concomitantly. Results：CPB times was 70 - 162min, mean （94. 60 ± 25.30）rain, aortic cross-clamp times 36 - 94min, mean（ 53.40± 20. 50） min, and operative times 153 - 264min, mean （ 210. 60± 38.70 ） min, respectively. There were 2 perioperative deaths with an operative mortality at 3.08%. The cause of death wasmultiple organ failure. The postoperative complications included low output cardiac syndrome in 7 cases,perfu- sion lung in 6 （ 9. 23 % ） , reoperation for excessive bleeding in 4 （ 6. 15 % ） , pleural effusion in 12 （ 18.46% ） ; arrhythmia in 8（ 12. 31% ）. 60 patients were followed up from 6 to 63 months,mean（23.70 -＋8. 20）months with a follow-up rate of 92. 31% （60/65）. During the follow-up ,4 patients had residual shunt of VSD （ventricular septal defect） at 6 months postoperatively. Among them ,2 patients underwent reoperation （ 1 case underwent rerepair, 1 case occlusion）. The peak systolic pressure gradient of right ventricle to pulmonary artery exceeded 40 mmHg in 4 patients,3 patients had severe pulmonary insufficiency, and moderate tricuspid insufficiency in 2 cases. Frequent premature ventricular contraction existed in 1 patient at 18 months postoperatively. All 60 patients had class I - II heart function （NYHA）. No late death occurred. Conclusion： The effectiveness of surgical treatment for adult with TOF is satisfying. Relative measures should be taken to decrease complications and im prove prognosis.