McHugh Kimberly E, Hillman Diane G, Gurka Matthew J, Gutgesell Howard P
Division of Cardiology, Department of Pediatrics, University of Virginia Health System, Charlottesville, VA 22908, USA.
Congenit Heart Dis. 2010 Jan-Feb;5(1):8-15. doi: 10.1111/j.1747-0803.2009.00367.x.
The objective of this study was to describe current management and short-term outcomes for patients with hypoplastic left heart syndrome representative of nationwide experience. Additionally, this study identifies risk factors associated with mortality in patients undergoing staged surgical palliation.
The University HealthSystem Consortium database was queried to identify all hospital admissions between 1998 and 2007 with a diagnosis of hypoplastic left heart syndrome. Procedure codes were used to determine surgical management, and outcomes were ascertained by discharge status (discharged, transferred, or expired).
Discharge data were present from 118 hospitals in the United States. First-stage surgical palliation was performed in 1949 neonates with 30% mortality. Mortality decreased from 43% in 1998 to 18% in 2007. Large institutional case volume and later era of surgery were associated with improved operative mortality in first-stage palliation. Primary cardiac transplantation was performed in 28 neonates, and rescue transplantation in 11, with 36% mortality. Second-stage palliation was performed in 1244 patients with 5.2% mortality, and third-stage palliation was performed in 1084 patients with 4.1% mortality. An additional 62 patients over 1 month of age at time of admission received cardiac transplantation with 15% mortality.
First-stage palliative mortality for hypoplastic left heart syndrome fell dramatically over the past decade, while that for second- and third-stage procedures remained stable. The cumulative operative mortality for three-staged repair of hypoplastic left heart syndrome was 39% over the decade, but fell to 24% for procedures in 2007.
本研究的目的是描述代表全国经验的左心发育不全综合征患者的当前管理情况和短期预后。此外，本研究确定了接受分期手术姑息治疗患者的死亡相关危险因素。
查询大学卫生系统联盟数据库，以识别1998年至2007年间所有诊断为左心发育不全综合征的住院病例。使用手术编码确定手术管理情况，并通过出院状态（出院、转院或死亡）确定预后。
美国118家医院提供了出院数据。1949例新生儿接受了一期手术姑息治疗，死亡率为30%。死亡率从1998年的43%降至2007年的18%。机构病例量大和手术时间较晚与一期姑息治疗手术死亡率的改善相关。28例新生儿接受了原位心脏移植，11例接受了挽救性移植，死亡率为36%。1244例患者接受了二期姑息治疗，死亡率为5.2%，1084例患者接受了三期姑息治疗，死亡率为4.1%。另外62例入院时年龄超过1个月的患者接受了心脏移植，死亡率为15%。
在过去十年中，左心发育不全综合征的一期姑息治疗死亡率大幅下降，而二期和三期手术的死亡率保持稳定。在这十年中，左心发育不全综合征三期修复的累计手术死亡率为39%，但在2007年的手术中降至24%。
