Sydney Medical School, University of Sydney, Camperdown, NSW 2006, Australia.
Heart Research Institute, 7 Eliza St, Newtown, NSW 2042, Australia.
Int J Cardiol. 2021 Sep 1;338:89-94. doi: 10.1016/j.ijcard.2021.05.007. Epub 2021 Jun 24.
We aim to establish a new and informative bi-national Registry for Congenital Heart Disease (CHD) patients in Australia and New Zealand, to document the burden of disease and clinical outcomes for patients with CHDs across the lifespan. When planning for the implementation of this Registry, we sought to evaluate the strengths and weaknesses of existing national and large regional CHD databases.
We characterised 15 large multi-institutional databases of pediatric and/or adult patients with CHD, documenting the richness of their datasets, the ease of linkage to other databases, the coverage of the target cohort and the strategies utilised for quality control.
The best databases contained demographic, clinical, physical, laboratory and patient-reported data, and were linked at least to the national/regional death registry. They also employed automatic data verification and regular manual audits. Coverage ranged from around 25% of all eligible CHD cases for larger databases to near 100% for some smaller registries of patients with specific CHD lesions, such as the Australia and New Zealand Fontan Registry.
Existing national and regional CHD databases have strengths and weaknesses; few combine complete coverage with high quality and regularly audited data, across the broad range of CHDs.
我们旨在建立一个新的、信息丰富的澳新先天性心脏病（CHD）患者国家注册中心，以记录全生命周期 CHD 患者的疾病负担和临床结局。在规划该注册中心的实施时，我们评估了现有的国家和大型地区性 CHD 数据库的优缺点。
我们对 15 个大型多机构儿科和/或成人 CHD 患者数据库进行了特征描述，记录了其数据集的丰富程度、与其他数据库的链接便利性、目标队列的覆盖范围以及用于质量控制的策略。
最好的数据库包含人口统计学、临床、体格、实验室和患者报告数据，并至少与国家/地区的死亡登记处相关联。它们还采用了自动数据验证和定期手动审核。覆盖范围从较大数据库中约 25%的所有合格 CHD 病例到某些特定 CHD 病变（如澳大利亚和新西兰 Fontan 注册中心）的较小患者注册中心的近 100%不等。
现有的国家和地区性 CHD 数据库有其优缺点；很少有数据库能够在广泛的 CHD 范围内，结合全面覆盖、高质量和定期审核的数据。
