Pediatric cardiology

htchb

Pediatric cardiology
儿科心脏病学
Factors Associated With Mortality and Reoperation in 377 Children With Total Anomalous Pulmonary Venous Connection

Background— We sought to determine era-specific changes in the incidence of mortality and reoperation in children with
total anomalous pulmonary venous connection.
Methods and Results— We reviewed the records of 377 children presenting from 1946 to 2005 with total anomalous pulmonary venous connection. Multivariable parametric regression models determined the incidence and risk factors for death and reoperation after repair. Pulmonary venous connection was supracardiac in 44%, infracardiac in 26%, cardiac in 21%, and mixed in 9%. Pulmonary venous obstruction was present in 48% at presentation, most frequently with infracardiac connection type (P<0.001). In total, 327 patients were repaired (median age, 1.7 months). Overall survival from repair was 65±6% at 14 years, with a current survival of 97%. Significant (P<0.01) incremental risk factors for postrepair death were cardiac connection type, earlier operation year, younger age at repair, use of epinephrine postoperatively, and postoperative pulmonary venous obstruction. More recent operation year was associated with younger age at repair (P<0.001), decreased use of deep hypothermic circulatory arrest (P<0.001), and use of specific drugs postoperatively (P<0.001). Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2005 is 37% (95% CI, 8 to 80) compared with 96% (95% CI, 91 to 99) for a patient with favorable morphology repaired at 1 year of age. Freedom from reoperation was 82±6% at 11 years after repair, with increased risk associated with mixed connection type (P=0.04) and postoperative pulmonary venous obstruction (P<0.001).

Conclusions— Mortality after total anomalous pulmonary venous connection repair has decreased but remains highest in young patients and in those with cardiac connection type or pulmonary venous obstruction. Unfavorable anatomic characteristics remain important determinants of postrepair survival despite improved perioperative care.

377名完全性肺静脉异常连接儿童死亡率及再手术率相关因子
背景—本文旨在阐明儿童完全性肺静脉异常连接的发病率,死亡率以及再手术率的时代特异性改变.
方法和结果--我们回顾分析了1946到2005年之间377名完全性肺静脉连接异常儿童病人的病历.利用多变量参数回归模型计算死亡和术后再手术的风险和发生率. 所有病人中,肺静脉连接位于心脏上方的占44%,心脏下方的26%,心脏同水平的21%,混合型9%.48%的病人存在肺静脉梗塞,并且其中大部分为心脏下连接型(p<0.01).修复后14年总生存率为65±6%,目前生存率为97%. 是手术后风险因子明显(p<0.01)增加的因素有心脏同水平的连接型, 早年进行的手术,修复时年龄过小,术后应用肾上腺素以及术后肺静脉梗阻等. 晚近手术风险增加常与修复时年龄过小(p<0.01), 深低温停循环技术试用的下降(P<0.001),和术后使用特殊药物(P<0.001). 风险调整预测, 不利形态的病例出生后即行手术的一年生存率为37% (95% CI, 8 to 80), 而有利形态的病例出生后即行手术的一年生存率为96%(95% CI, 91 to 99). 修复后11年不需手术的比率为82±6%, 是该比率升高的危险因素有混合连接型(p<0.04),和术后肺静脉梗阻(p<0.001)
结论—-虽然完全性肺静脉异常连接修复后病人的死亡率有所下降,但是在年轻病人,心脏连接型和肺静脉梗塞者仍然是最高的. 虽然改善了围手术期的护理,但是不利的解剖特征仍然是修复后生存率的重要决定因素.