Institute of Cardiovascular Science University College London UK.
Great Ormond Street Hospital Biomedical Research Centre London UK.
J Am Heart Assoc. 2024 Nov 5;13(21):e035166. doi: 10.1161/JAHA.124.035166. Epub 2024 Oct 29.
There is strong interest in the evaluation of longer-term outcome metrics for congenital heart diseases (CHDs); however, registries focus on postoperative metrics.
Informed by user online discussion forums and scoping of national data, we selected sentinel CHDs and long-term outcome metrics suitable for routine monitoring. We then developed sentinel CHD phenotypes and algorithms for identifying treatment pathway procedures using clinical codes. Finally, we calculated the metrics within a retrospective national cohort analysis. The 9 selected sentinel CHDs had a higher-than-average prevalence, typically involved surgery in infancy, and were associated with an increased risk of late mortality. The selected metrics of survival and reinterventions at 1, 5, and 10 years were both important and feasible. The cohort included 29 319 (41.3% of all operated CHD births) English and Welsh children born with sentinel CHDs in 2000 to 2022. Example metrics at age 10 years included: survival-hypoplastic left heart syndrome: 57.6% (95% CI, 54.9%-60.4%), functionally univentricular heart: 86.7% (95% CI, 84.6%-88.9%), transposition of the great arteries: 93.1% (95% CI, 92.2%-93.9%), pulmonary atresia: 81.0% (95% CI, 79.1%-82.9%), atrioventricular septal defect: 88.5% (95% CI, 87.5%-89.5%), tetralogy of Fallot: 95.1% (95% CI, 94.4%-95.8%), aortic stenosis: 94.4% (95% CI, 93.3%-95.6%), coarctation: 96.7% (95% CI, 96.2%-97.3%), and ventricular septal defect: 96.9% 95% CI, (96.4%-97.3%); and (2) cumulative incidence of reintervention-hypoplastic left heart syndrome : 54.5% (95% CI, 51.5%-57.3%), functionally univentricular heart: 57.3% (95% CI, 53.9%-60.5%), transposition of the great arteries: 20.9% (95% CI, 19.5%-22.3%), pulmonary atresia: 66.8% (95% CI, 64.2%-69.1%), atrioventricular septal defect: 21.6% (20.3%-23.0%), tetralogy of Fallot: 26.6% (95% CI, 25.2%-28.0%), aortic stenosis: 31.2% (95% CI, 28.8%-33.6%), coarctation: 19.8% (95% CI, 18.6%-21.1%), and ventricular septal defect: 6.1% (95% CI, 5.5%-6.8%).
It is feasible to report important long-term outcomes of survival and reintervention for sentinel CHDs using routinely collected procedure records, adding value to national audit.
人们对先天性心脏病（CHD）的长期预后指标评估非常感兴趣；然而，登记处主要关注术后指标。
通过用户在线讨论论坛和国家数据的范围界定，我们选择了有代表性的 CHD 和适合常规监测的长期预后指标。然后，我们使用临床代码为每种 CHD 选择了代表性表型和确定治疗途径程序的算法。最后，我们在回顾性全国队列分析中计算了这些指标。所选的 9 种有代表性的 CHD 具有较高的患病率，通常在婴儿期接受手术治疗，并且与晚期死亡率增加相关。1 年、5 年和 10 年的生存和再干预指标都很重要且可行。该队列包括 2000 年至 2022 年在英国威尔士出生的 29319 名（所有接受 CHD 手术出生儿的 41.3%）有代表性的 CHD 患儿。10 岁时的示例指标包括：生存-左心发育不良综合征：57.6%（95%CI，54.9%-60.4%），功能性单心室：86.7%（95%CI，84.6%-88.9%），大动脉转位：93.1%（95%CI，92.2%-93.9%），肺动脉闭锁：81.0%（95%CI，79.1%-82.9%），房室间隔缺损：88.5%（95%CI，87.5%-89.5%），法洛四联症：95.1%（95%CI，94.4%-95.8%），主动脉瓣狭窄：94.4%（95%CI，93.3%-95.6%），缩窄性动脉炎：96.7%（95%CI，96.2%-97.3%）和室间隔缺损：96.9%（95%CI，96.4%-97.3%）；以及（2）再干预累计发生率-左心发育不良综合征：54.5%（95%CI，51.5%-57.3%），功能性单心室：57.3%（95%CI，53.9%-60.5%），大动脉转位：20.9%（95%CI，19.5%-22.3%），肺动脉闭锁：66.8%（95%CI，64.2%-69.1%），房室间隔缺损：21.6%（20.3%-23.0%），法洛四联症：26.6%（95%CI，25.2%-28.0%），主动脉瓣狭窄：31.2%（95%CI，28.8%-33.6%），缩窄性动脉炎：19.8%（95%CI，18.6%-21.1%）和室间隔缺损：6.1%（95%CI，5.5%-6.8%）。
使用常规收集的程序记录报告有代表性的 CHD 的生存和再干预的重要长期预后是可行的，这为国家审计增加了价值。
