Inserm, UMR S953, Recherche épidémiologique sur la santé périnatale et la santé des femmes et des enfants, UPMC, Université Paris-6, Maternité de Port-Royal, Paris, France.
Heart. 2012 Nov;98(22):1667-73. doi: 10.1136/heartjnl-2012-302543. Epub 2012 Aug 11.
To assess the prevalence, timing of diagnosis and infant mortality of congenital heart defects (CHD) with population-based data and using a classification that allows regrouping of the International Paediatric and Congenital Cardiac Code into a manageable number of categories based on anatomic and clinical criteria (ACC-CHD).
Population-based cohort study.
Greater Paris.
All cases (live births, terminations of pregnancy for foetal anomaly (TOPFA), foetal deaths) diagnosed prenatally, or up to 1 year of age in the birth cohorts, May 2005-April 2008, for women in Greater Paris (n=317 538 births). Diagnoses were confirmed in specialised centres and subsequently coded and classified into the categories of ACC-CHD by paediatric cardiologists in the study group.
The total number of CHD was 2867, including 2348 live births (82%), 466 TOPFA (16.2%) and 53 foetal deaths (1.8%). The total prevalence of CHD was 90 per 10 000. After exclusion of ventricular septal defects (VSD), 40% of 'isolated' CHD was diagnosed prenatally with about one half of the remaining diagnosed before 7 days of age. Nevertheless, one in five cases of these major CHD was diagnosed after the fourth week. Infant mortality of 'isolated' CHD-VSD excluded was 8.5% with 40% of deaths occurring after the fourth week of life. These outcomes varied substantially across categories of ACC-CHD.
Timing of diagnosis, TOPFA, risk and timing of infant mortality were highly variable across the categories of CHD in ACC-CHD, suggesting that it may be a useful measure of severity, and hence, predictor of outcomes of CHD.
利用基于人群的数据和一种分类方法，评估先天性心脏病（CHD）的患病率、诊断时间和婴儿死亡率，该分类方法根据解剖和临床标准，将国际儿科和先天性心脏病代码（IPCCC）重新组合成可管理的类别数（ACC-CHD）。
基于人群的队列研究。
大巴黎地区。
2005 年 5 月至 2008 年 4 月期间，大巴黎地区的所有女性（活产儿、因胎儿异常而终止妊娠（TOPFA）、胎儿死亡），在产前诊断或出生队列中 1 岁以内，诊断为 ACC-CHD 的病例。这些诊断在专门中心得到确认，随后由研究组的儿科心脏病专家对病例进行编码和分类。
共诊断出 2867 例 CHD，包括 2348 例活产儿（82%）、466 例 TOPFA（16.2%）和 53 例胎儿死亡（1.8%）。CHD 的总患病率为 90/10000。排除室间隔缺损（VSD）后，40%的“孤立性”CHD 在产前诊断，其余约一半在出生后 7 天内诊断。然而，这些主要 CHD 中有五分之一是在第四周后诊断的。排除孤立性 CHD-VSD 后，婴儿死亡率为 8.5%，40%的死亡发生在第四周后。这些结果在 ACC-CHD 的 CHD 类别中差异很大。
在 ACC-CHD 的 CHD 类别中，诊断时间、TOPFA、婴儿死亡率的风险和时间变化很大，这表明它可能是严重程度的一个有用指标，因此是 CHD 结局的预测指标。
