Concord Repatriation General Hospital, Concord, New South Wales, Australia.
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Heart. 2018 Oct;104(20):1678-1682. doi: 10.1136/heartjnl-2017-312777. Epub 2018 Apr 17.
The life expectancy of patients with congenital heart disease (CHD) has significantly improved with advances in their paediatric medical care. Mortality patterns are changing as a result. Our study aims to describe survival and causes of death in a contemporary cohort of adult patients with CHD.
We reviewed 3068 patients in our adult CHD database (age ≥16 years, seen at least once in our centre between 2000 and 2015), and documented the number and causes of death, via Australia's National Death Index. Survival and mortality patterns were analysed by complexity of CHD and by underlying congenital diagnosis.
Our cohort comprised 3068 adult patients (53% male). The distribution of patients (per the Bethesda classification) was 47% simple, 34% moderate and 18% complex (1% not classifiable). Over a median follow-up of 6.2 years (IQR 3.5-10.4), 341 patients (11%) died with an incidence of 0.4 deaths/100 patient years (py). Survival was significantly worse with increasing complexity of CHD (p<0.001); mortality rate in the simple group was 0.3 deaths/100 py with a median age of death 70 years, and in the complex group was 1.0 death/100 py with a median age of death 34 years. Overall, non-cardiac causes of death outnumbered cardiac causes, at 54% and 46%, respectively. The leading single cause of death was heart failure (17%), followed by malignancy (13%). Simple adult CHD patients mostly died due to non-cardiac causes such as malignancy. Perioperative mortality only accounted for 5% of deaths.
Premature death is common in adults with CHD. Although heart failure remains the most common cause of death, in the contemporary era in a specialist CHD centre, non-cardiac related deaths outnumber cardiac deaths, particularly in those with simple CHD lesions.
随着小儿心脏病治疗的进步，先天性心脏病（CHD）患者的预期寿命显著提高。由此导致死亡率模式发生变化。本研究旨在描述当代成年 CHD 患者的生存和死亡原因。
我们回顾了我们的成人 CHD 数据库中的 3068 名患者（年龄≥16 岁，在 2000 年至 2015 年间至少在我们中心就诊过一次），并通过澳大利亚国家死亡索引记录了死亡人数和死因。通过 CHD 的复杂性和先天性诊断，分析了生存和死亡率模式。
我们的队列包括 3068 名成年患者（53%为男性）。患者的分布（根据贝塞斯达分类）为 47%简单，34%中度和 18%复杂（1%不可分类）。在中位数为 6.2 年（IQR 3.5-10.4）的随访期间，341 名患者（11%）死亡，死亡率为 0.4 例/100 患者年（py）。CHD 复杂性增加，生存显著恶化（p<0.001）；简单组的死亡率为 0.3 例/100 py，中位死亡年龄为 70 岁，复杂组的死亡率为 1.0 例/100 py，中位死亡年龄为 34 岁。总体而言，非心脏原因的死亡人数超过心脏原因，分别为 54%和 46%。主要单一死亡原因是心力衰竭（17%），其次是恶性肿瘤（13%）。简单的成年 CHD 患者大多死于非心脏原因，如恶性肿瘤。围手术期死亡率仅占死亡人数的 5%。
先天性心脏病成年患者的过早死亡很常见。尽管心力衰竭仍然是最常见的死亡原因，但在当代专科 CHD 中心，非心脏相关死亡人数超过心脏死亡人数，尤其是在简单 CHD 病变患者中。
