Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period
Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period
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DOI：
10.1016/j.jtcvs.2009.04.061
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发表时间：
2010-01-01
影响因子：
6
通讯作者：
Welke, Karl F.
中科院分区：
文献类型：
--
作者：
Karamlou, Tara;Diggs, Brian S.;Welke, Karl F.
目的：我们的目的是描述在过去的18年中，在美国的新生儿左心发育不良综合征的管理策略，并确定机构管理decisions.Methods的决定因素：新生儿左心发育不良综合征的回顾性确定使用全国住院病人样本1988-2005。治疗分为（1）移植，（2）诺伍德手术（定义为先天性心脏病手术风险调整），（3）转移到另一个设施，或（4）没有手术干预（舒适护理）。其中，2%（348 +/- 47）接受了移植，16%（2767 +/- 286）接受了诺伍德手术，25%（4143 +/- 156）被转移到另一个机构，57%（9523 +/- 436）接受了舒适护理。随着时间的推移，实践模式发生了变化，越来越多的新生儿接受诺伍德，伴随着接受移植的人数减少（P <0.001）。随着时间的推移，诺伍德手术的偏倚使诺伍德手术的住院死亡率显著下降，几乎呈线性下降，从最早的六分位数的86%下降到最近的六分位数的24%（P < .001）。随着时间的推移，转移到确定性护理医院的患病率保持不变，婴儿（约一半）的数量没有接受手术（舒适护理）。结论：尽管改善了手术效果，大多数婴儿继续没有接受手术护理。在过去的20年里，接受诺伍德手术治疗左心发育不全综合征的婴儿人数有所增加，这似乎主要是由于移植手术的减少。产前诊断的出现并没有减少在不具备提供最终护理条件的机构出生的新生儿的比例。（《胸血管外科杂志》2010; 139：119-27）
Objectives: We aimed to describe management strategies for neonates with hypoplastic left heart syndrome over the past 18 years in the United States and to identify determinants of institutional management decisions.Methods: Neonates with hypoplastic left heart syndrome were retrospectively identified by use of the Nationwide Inpatient Sample 1988-2005. Treatment was categorized as (1) transplantation, (2) Norwood operation (as defined by Risk Adjustment in Congenital Heart Surgery), (3) transfer to another facility, or (4) no surgical intervention (comfort care).Results: A total of 3286 neonates were identified, yielding a national estimate of 16,781 +/- 586 cases. Of these, 2%(348 +/- 47) underwent transplantation, 16%(2767 +/- 286) had Norwood operations, 25%(4143 +/- 156) were transferred to another facility, and 57% (9523 +/- 436) had comfort care. Changes in practice patterns occurred over time, with an increasing number of neonates undergoing Norwood, concomitant with decreasing numbers undergoing transplantation (P < .001). Bias toward the Norwood operation over time paralleled a significant, nearly linear decrease in the in-hospital mortality rate for the Norwood operation, from 86% in the earliest sextile to 24% in the most recent sextile (P < .001). Prevalence of transfer to definitive care hospitals remained constant over time, as did the number of infants (approximately half) who received no surgery (comfort care).Conclusions: Despite improved surgical outcomes, the majority of infants continue to receive no surgical care. There has been an increase in the number of infants offered the Norwood operation for hypoplastic left heart syndrome over the past 2 decades, which seems to have come mostly owing to a decrease of transplants. The advent of prenatal diagnosis has not decreased the proportion of neonates born at institutions unequipped to provide definitive care. (J Thorac Cardiovasc Surg 2010; 139: 119-27)