Plantinga R F, Frohn-Mulder I M, Takkenberg H J, Bennink G B, Meijboom E J, Bogers A J
Universitair Medisch Centrum, Wilhelmina Kinderziekenhuis, Kinderhartcentrum, Postbus 85.090, 3508 AB Utrecht.
Ned Tijdschr Geneeskd. 2001 Jan 13;145(2):85-90.
To inventory the treatment of hypoplastic left heart syndrome (HLHS) in the Netherlands and its results.
Retrospective.
Data were collected from all patients (n = 117) diagnosed with HLHS in the Wilhelmina Children's Hospital-University Medical Center Utrecht and the University Hospital Rotterdam-Sophia Children's Hospital and born in the period 1 March 1988-31 May 2000. Type and time of intervention, and mortality were recorded and cumulative survival was analysed by Kaplan-Meier analysis. Cumulative survival was compared between early and late series and between the two hospitals.
The study group comprised 68 boys and 49 girls, all neonates. At the time of the investigation, the mean duration of follow-up was 185 days (range: 0-3855). Fifty-eight children had received no treatment; all of these had died. Fifty-nine children were scheduled for the Norwood procedure; six of them died before operation. The 53 patients who underwent the first stage of the Norwood procedure had 1-month, 1-year, 2-year, and 5-year survival chances of 55%, 30%, 27%, and 24% respectively. Survival chances between the two time periods and the two hospitals showed no significant differences.
The Norwood procedure was performed in almost half of the children with HLHS. It is only moderately successful; however, it seems the only realistic choice in the management.
统计荷兰对左心发育不全综合征（HLHS）的治疗情况及其结果。
回顾性研究。
收集了1988年3月1日至2000年5月31日期间在乌得勒支大学医学中心威廉明娜儿童医院和鹿特丹大学医院 - 索菲亚儿童医院出生且被诊断为HLHS的所有患者（n = 117）的数据。记录干预类型和时间以及死亡率，并通过Kaplan - Meier分析对累积生存率进行分析。比较早期和晚期系列以及两家医院之间的累积生存率。
研究组包括68名男孩和49名女孩，均为新生儿。在调查时，平均随访时间为185天（范围：0 - 3855天）。58名儿童未接受治疗；所有这些儿童均已死亡。59名儿童计划进行诺伍德手术；其中6人在手术前死亡。接受诺伍德手术第一阶段的53名患者1个月、1年、2年和5年的生存几率分别为55%、30%、27%和24%。两个时间段和两家医院之间的生存几率没有显著差异。
几乎一半的HLHS儿童接受了诺伍德手术。该手术仅取得了一定程度的成功；然而，它似乎是治疗中唯一现实的选择。
