Department of Paediatric Cardiology, Freeman Hospital, Freeman Road, Newcastle upon Tyne, UK.
Arch Dis Child. 2012 Apr;97(4):326-30. doi: 10.1136/adc.2010.210534. Epub 2011 Aug 11.
The prevalence of cardiovascular anomalies in Down's syndrome is well described, but there are few data on spectrum, management and outcome. The authors aimed to provide this information for infants with Down's syndrome in a defined population over a 22-year period.
The regional paediatric cardiology database in Newcastle upon Tyne provided information on all cardiovascular anomalies, surgical treatment and outcome. Data was subdivided into two eras, 1985-1995 and 1996-2006, and surgical results and outcomes compared. Data on live births with Down's syndrome were obtained from the Northern Congenital Abnormality Survey (NorCAS). Denominator data on all live births in the region were obtained from UK Statistics.
In 1985-2006 there were 754,486 live births in the population. 821 infants were live-born with Down's syndrome (1.09 per 1000 live births). 342 (42%) infants with Down's syndrome had a cardiovascular anomaly. The commonest anomaly was complete atrioventricular septal defect in 125 (37%) infants. Three patients had univentricular physiology. In 1985-1995, 101/163 (62%) infants had surgery with 30% mortality; in 1996-2006, 129/180 (72%) had surgery with 5% mortality. One patient underwent Fontan completion. There were two cardiac transplants for cardiomyopathy. One-year survival in Down's syndrome with a cardiovascular anomaly improved from 82% in 1985-1995 to 94% in 1996-2006.
The incidence of cardiovascular anomalies in Down's syndrome was 42%. There has been a significant reduction in postoperative mortality and improvement in 1-year survival. Treatment modalities such as single ventricle palliation and cardiac transplantation are now considered in these patients.
唐氏综合征患者中心血管异常的发生率已有详细描述，但有关其谱、管理和结局的数据却很少。作者旨在为在 22 年期间的特定人群中患有唐氏综合征的婴儿提供此类信息。
纽卡斯尔的区域儿科心脏病学数据库提供了所有心血管异常、手术治疗和结局的信息。数据分为两个时期，1985-1995 年和 1996-2006 年，并对手术结果和结局进行了比较。唐氏综合征活产儿的数据来自北方先天性异常调查（NorCAS）。该地区所有活产儿的分母数据来自英国统计数据。
在 1985-2006 年期间，该人群中有 754486 例活产儿。821 例唐氏综合征婴儿为活产儿（每 1000 例活产儿中有 1.09 例）。342 例（42%）唐氏综合征婴儿存在心血管异常。最常见的异常是 125 例（37%）婴儿完全性房室间隔缺损。3 例婴儿存在单心室生理学。在 1985-1995 年，163 例中有 101 例（62%）婴儿接受了手术，死亡率为 30%；在 1996-2006 年，180 例中有 129 例（72%）婴儿接受了手术，死亡率为 5%。1 例患者接受了 Fontan 完成术。有 2 例心肌病患者进行了心脏移植。唐氏综合征伴心血管异常患者的 1 年生存率从 1985-1995 年的 82%提高到 1996-2006 年的 94%。
唐氏综合征中心血管异常的发生率为 42%。术后死亡率显著降低，1 年生存率提高。目前，在这些患者中考虑了单心室姑息治疗和心脏移植等治疗方式。
