Ashburn David A, Blackstone Eugene H, Wells Winfield J, Jonas Richard A, Pigula Frank A, Manning Peter B, Lofland Gary K, Williams William G, McCrindle Brian W
Division of Cardiothoracic Surgery, Hospital for Sick Children, Toronto, Onario, Canada.
J Thorac Cardiovasc Surg. 2004 Apr;127(4):1000-7; discussion 1007-8. doi: 10.1016/j.jtcvs.2003.11.057.
We sought to define the prevalence of definitive end states and their determinants in children given a diagnosis of pulmonary atresia and intact ventricular septum during the neonatal period.
Between 1987 and 1997, 408 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective study by 33 institutions. Competing risks analysis was used to demonstrate the prevalence of 6 end states. Factors predictive of attaining each end state were identified by means of multivariable analysis with bootstrap validation.
Overall survival was 77% at 1 month, 70% at 6 months, 60% at 5 years, and 58% at 15 years. Prevalence of end states 15 years after entry were as follows: 2-ventricle repair, 33%; Fontan repair, 20%; 1.5-ventricle repair, 5%; heart transplant, 2%; death before reaching definitive repair, 38%; and alive without definitive repair, 2%. Patient-related factors discriminating among end states primarily included adequacy of right-sided heart structures, degree of aberration of coronary circulation, low birth weight, and tricuspid valve regurgitation. After adjusting for these factors, 2 institutions were predictive of 2-ventricle repair, 1 of Fontan repair, and 6 of death before definitive repair. Two institutions were predictive of both 2-ventricle and Fontan repair. These 2 institutions achieved a higher risk-adjusted prevalence of definitive repair and a lower prevalence of prerepair mortality.
Characteristics of neonates with pulmonary atresia and intact ventricular septum predict type of definitive repair. A morphologically driven institutional protocol emphasizing both 2-ventricle and Fontan pathways might mitigate the negative effect of unfavorable morphology. In the current era, 85% of neonates are likely to reach a definitive surgical end point, with 2-ventricle repair achieved in an estimated 50%.
我们试图确定新生儿期被诊断为肺动脉闭锁且室间隔完整的儿童中最终结局的发生率及其决定因素。
1987年至1997年间，33家机构对408例肺动脉闭锁且室间隔完整的新生儿进行了一项前瞻性研究。采用竞争风险分析来显示6种最终结局的发生率。通过多变量分析和自抽样验证确定预测每种最终结局的因素。
1个月时总体生存率为77%，6个月时为70%，5岁时为60%，15岁时为58%。入组15年后最终结局的发生率如下：双心室修复，33%；Fontan修复，20%；1.5心室修复，5%；心脏移植，2%；在达到确定性修复前死亡，38%；未进行确定性修复而存活，2%。区分不同最终结局的患者相关因素主要包括右侧心脏结构的充分性、冠状动脉循环异常程度、低出生体重和三尖瓣反流。在对这些因素进行调整后，2家机构预测为双心室修复，1家预测为Fontan修复，6家预测为在确定性修复前死亡。2家机构预测可进行双心室和Fontan修复。这2家机构实现了更高的风险调整后确定性修复发生率和更低的修复前死亡率。
肺动脉闭锁且室间隔完整的新生儿特征可预测确定性修复的类型。一种形态学驱动的机构方案，同时强调双心室和Fontan途径，可能会减轻不利形态学的负面影响。在当前时代，85%的新生儿可能会达到确定性手术终点，估计50%可实现双心室修复。
