Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007
Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007
复制标题
DOI：
10.1016/j.athoracsur.2008.01.057
复制
发表时间：
2008-05-01
影响因子：
4.6
通讯作者：
Quintessenza, James A.
中科院分区：
文献类型：
--
作者：
Jacobs, Jeffrey P.;O'Brien, Sean M.;Quintessenza, James A.
背景。我们回顾了我院治疗左心发育不全综合征及相关畸形的全部手术经验。截至2007年10月1日，239名患有HLHS和相关畸形的患者在佛罗里达先天性心脏研究所接受了手术治疗。本文的重点是199例最初接受诺伍德1期治疗的患者。199名患者最初接受了诺伍德一期治疗。单因素分析显示以下重要的死亡率预测因素：右室优势（p = 0.0023）， 1期前机械循环支持（p = 0.0192），以及显著的非心脏异常或综合征，包括唐氏综合征、特纳综合征、异位、脾功能不全、多脾功能不全、胆道闭锁或其他染色体异常（p < 0.0001）。多变量logistic回归分析显示，1期前出现明显的非心脏异常或综合征、早产小于35周或机械循环支持是死亡率的重要预测因子（p < 0.0001）。在该患者系列研究的14年中，157例采用诺伍德分期缓和治疗的“低风险”患者（在1期之前没有明显的非心脏异常或综合征或早产少于35周或机械循环支持的患者）在1期后30天、1期后出院和1期后1年的生存率分别为86%、80%和69%。有几种治疗方案可用于HLHS和相关畸形。适当的治疗策略必须与个体患者相匹配，考虑到解剖变量以及其他患者特异性特征。大多数患有HLHS和相关畸形的患者可以成功地进行分阶段姑息治疗，但风险不同，根据2008年胸外科学会的一些报告
Background. We reviewed our entire programmatic operative experience with children with hypoplastic left heart syndrome (HLHS) and related malformations.Methods. As of October 1, 2007, 239 patients with HLHS and related malformations underwent surgical treatment at the Congenital Heart Institute of Florida. This manuscript focuses on the 199 initially treated with Norwood stage 1.Results. One hundred and ninety-nine patients were initially treated with Norwood stage 1. Univariate analysis demonstrated the following significant predictors of mortality: right ventricular dominance ( p = 0.0023), mechanical circulatory support before stage 1 ( p = 0.0192), and significant noncardiac abnormality or syndrome, including Down syndrome, Turner syndrome, heterotaxy, asplenia, polysplenia, biliary atresia, or other chromosomal abnormality ( p < 0.0001). Multivariable logistic regression analysis revealed the presence of a significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support before stage 1 to be a significant predictor of mortality ( p < 0.0001). Over the 14 years of this patient series, survival for the 157 "low-risk" patients managed with Norwood staged palliation (those patients without significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support prior to Stage 1) was 86%, 80%, and 69% at 30 days after Stage 1, hospital discharge after Stage 1, and 1 year of after Stage 1, respectively.Conclusions. Several treatment options are available for HLHS and related malformations. The appropriate treatment strategy must be matched to the individual patient, taking into consideration anatomic variables as well as other patient-specific characteristics. The majority of patients with HLHS and related malformations can undergo successful staged palliation with risk that varies according to several c 2008 by The Society of Thoracic Surgeons