Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands; Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands.
Am Heart J. 2012 Oct;164(4):568-75. doi: 10.1016/j.ahj.2012.07.023.
The prevalence of adult patients with congenital heart disease (CHD) has been reported with a high degree of variability. Prevalence estimates have been calculated using birth rate, birth prevalence, and assumed survival and derived from large administrative databases. To report more robust prevalence estimate, we performed a systematic review for studies concerning CHD prevalence in adults. Moreover, to diminish bias of calculated estimates, we conducted an evidence-based calculation for the Netherlands.
A systematic database search was performed to identify reports on the prevalence of adult CHD. Bicuspid aortic valve, mitral valve prolapse, Marfan syndrome, cardiomyopathy, congenital arrhythmia, and spontaneously closed defects were excluded. In addition, CHD prevalence was calculated using birth rate, birth prevalence, and survival estimates.
Our search yielded 10 publications on the prevalence of CHD in adults. Four reported results from population wide cross-sectional data, whereas in 6, prevalence was calculated. Mean prevalence reported by empirical studies was 3,562 per million when unspecified lesions were included and 2,297 per million when these were excluded. Mean prevalence derived from calculation was 3,536. Our calculated estimate was 3,228 per million adults. Taking these estimates as well as the limitations inherent to their derivation into consideration, the prevalence of CHD in the adult population is approximately 3,000 per million adults.
This systematic review presents a comprehensive overview of publications on the prevalence of CHD in adults. The best available evidence suggests that overall prevalence of CHD in the adult population is in the region of 3,000 per million.
成人先天性心脏病（CHD）患者的患病率存在很大的变异性。患病率的估计值是通过出生率、出生时的患病率和假定的生存率计算得出的，并来自大型行政数据库。为了报告更可靠的患病率估计值，我们对成人 CHD 患病率的研究进行了系统评价。此外，为了减少计算估计值的偏差，我们对荷兰进行了循证计算。
系统地检索了数据库，以确定关于成人 CHD 患病率的报告。排除了二叶式主动脉瓣、二尖瓣脱垂、马凡综合征、心肌病、先天性心律失常和自然闭合的缺陷。此外，还使用出生率、出生时的患病率和生存率估计值来计算 CHD 的患病率。
我们的搜索结果显示了 10 篇关于成人 CHD 患病率的文献。其中 4 篇报告了来自全人群横断面数据的结果，而另外 6 篇则计算了患病率。来自经验研究的平均患病率为包括未指定病变时为每百万 3562 例，排除这些病变时为每百万 2297 例。计算得出的平均患病率为 3536 例。我们计算出的估计值为每百万成年人 3228 例。考虑到这些估计值及其推导所固有的局限性，成人人群中 CHD 的患病率约为每百万成年人 3000 例。
本系统评价全面概述了成人 CHD 患病率的出版物。现有最佳证据表明，成人人群中 CHD 的总体患病率约为每百万 3000 例。
