婴儿期完全性肺静脉畸形引流矫治术近期疗效分析

The analysis of the recent effectiveness of the total anomalous pulmonary venous connection repairs on infants

目的 分析婴儿完全性肺静脉畸形引流矫治术的近期疗效,总结外科治疗经验,分析围手术期死亡的影响因素.方法 2001年1月至2008年7月共有145例1岁以内患儿因完全性肺静脉畸形引流接受矫治术,其中男性94例,女性51例;首次手术的平均年龄为（7±3）个月,平均体质量为（6.3±1.6）kg.肺静脉的引流方式心上型77例（53.1%）,心内型47例（32.4%）,心下型9例（6.2%）,混合型12例（8.3%）.术前超声评价21例有明确肺静脉梗阻（心上型12例,心内型3例,心下型3例,混合型3例）.结果 全组病例均行根治术治疗（合并复杂畸形不能行双心窜矫治已剔除）.围手术期死亡17例,病死率为11.7%.因2006年1月后手术技术和术后策略的综合改进,以此时间点为界分为两组（此前为A组,此后为B组）,病死率从A组的19.0%降至B组的6.2%（P=0.020）.回归分析显示病死率相关危险因素为组别、是否有肺静脉发育不良致梗阻以及房间隔交通的大小.手术技术以及围手术期治疗策略的综合改进是近3年围手术期终点结果满意的原因.全组无围手术期因肺静脉梗阻再次手术病例.结论 手术技术的改进和围手术期的积极综合治疗可降低完全性肺静脉畸形引流矫治术围手术期病死率.术前肺静脉发育不良致梗阻仍是影响患儿预后的重要因素.

Objective To review the efficacy of total anomalous pulmonary venous connection （TAPVC） repair and to conclude the factors impacting the peri-operative death rate. Methods The clinical data of 145 infants under 1 year old who underwent the TAPVC repair from January 2001 to July 2008 was analyzed. There were 94 male and 51 female patients. The mean age when the repair was performed was （7 ±3） months, and the average weight was （6.3 ± 1. 6） kg. As to the pulmonary connection type, 77 patients were supracardiac （ 53. 1 % ） , 47 patients were cardiac （32.4% ） , 9 patients were infraccardiac （6. 2% ） , and the remaining 12 patients were mixed （8. 3% ）. Pre-surgery echocardiography showed that 21 patients had pulmonary venous obstruction （12 patients were supracardiac type,3 patients were cardiac type, 3 patients were infraccardiac type, and 3 patients were mixed type）. Results All patients underwent two-ventricle anatomy correction （ the cases of complex malformations had been excluded）. Peri-operative mortality was 11.7% （17/145）. Because of the significant improvement in the surgical techniques, anesthesiology, cardiopulmonary bypass and the management of ICU in January 2006, the population was divided into two groups; A （before January 2006） and B （after January 2006）. Peri-operative mortality decreased from 19. 0% in group A to 6. 2% in group B（ P =0. 020）. After analysis, it was determined that the factors impacting mortality were which group the patient belongs to, whether he/she had preoperative pulmonary vein obstruction and how big the atril septel connection was. The operative technique to keep the anastomotic aperture adequate and prophylaxis pulmonary hypertensive episodes contributed to the improvement on the mortality rate. There had been no case of repeating the surgery because of pulmonary venous obstruction during peri-operative care period. Conclusions Improvements of the surgical techniqueas well as the treatment in preoperative and postoperative have led to the reduction of the mortality. Preoperative pulmonary vein obstruction is still an important factor that contributes to early mortality.