Nicholson Calum, Strange Geoff, Ayer Julian, Cheung Michael, Grigg Leeanne, Justo Robert, Maxwell Ryan, Wheaton Gavin, Disney Patrick, Yim Deane, Stewart Simon, Cordina Rachael, Celermajer David S
University of Sydney, Camperdown, NSW, 2050, Australia.
Heart Research Institute, 7 Eliza St, Newtown NSW 2042, Australia.
Int J Cardiol Congenit Heart Dis. 2024 Aug 24;17:100538. doi: 10.1016/j.ijcchd.2024.100538. eCollection 2024 Sep.
Although several National Data Registries for Congenital Heart Disease (CHD) exist, few are comprehensive and contemporary. A National Australian CHD Registry has been developed that aims to redress this by creating the first comprehensive data collection for CHD children and adults, initially across Australia.
We defined and collected a minimum dataset of demographics, diagnoses, and procedures from people with CHD presenting at participating quaternary CHD services Australia-wide. Data were collected from a range of clinical data sources. Diagnoses and procedures were standardised to the European Paediatric Congenital Code - Short List. Methodological limitations were carefully documented.
From 8 participating institutions, an initial 359,084 patient records were assessed for eligibility and 68,234 unique individuals with structural CHD have been included in the current dataset. There were 20,395 (30 %) people with mild CHD, 25,157 (37 %) with moderate CHD, and 13,530 (20 %) with severe CHD (6 % unknown complexity). The most common diagnoses were Ventricular Septal Defect (16,781, 25 %), Atrial Septal Defect (6,607, 10 %), Aortic Valve Disorders (5516 8 %), Coarctation of the Aorta (5,321, 8 %), Tetralogy of Fallot (4,489, 7 %), Transposition of the Great Arteries (4,009, 6 %).
The data presented here represents the most comprehensive cohort collected for the Australian CHD population thus far and is comparable with the largest contemporary CHD registries around the world. This Registry represents a key resource for improved understanding of the CHD population and will drive better care and outcomes for people living with CHD.
尽管存在多个先天性心脏病（CHD）国家数据登记处，但很少有登记处是全面且与时俱进的。澳大利亚已开发了一个国家先天性心脏病登记处，旨在通过首次为澳大利亚各地的先天性心脏病儿童和成人创建全面的数据收集来解决这一问题。
我们定义并收集了澳大利亚全国参与的四级先天性心脏病服务机构中先天性心脏病患者的人口统计学、诊断和手术的最小数据集。数据从一系列临床数据源收集。诊断和手术被标准化为欧洲儿科先天性编码 - 简短列表。仔细记录了方法学上的局限性。
从8个参与机构中，初步评估了359,084份患者记录的资格，目前的数据集中包括68,234名患有结构性先天性心脏病的独特个体。有20,395人（30%）患有轻度先天性心脏病，25,157人（37%）患有中度先天性心脏病，13,530人（20%）患有重度先天性心脏病（6%复杂性未知）。最常见的诊断是室间隔缺损（16,781例，25%）、房间隔缺损（6,607例，10%）、主动脉瓣疾病（5516例，8%）、主动脉缩窄（5,321例，8%）、法洛四联症（4,489例，7%）、大动脉转位（4,009例，6%）。
此处呈现的数据代表了迄今为止为澳大利亚先天性心脏病人群收集的最全面的队列，并且与世界上最大的当代先天性心脏病登记处相当。这个登记处是增进对先天性心脏病人群了解的关键资源，并将推动为先天性心脏病患者提供更好的护理和改善治疗结果。
