Institute of Medicine, Department of Molecular and Clinical Medicine, Sahlgrenska Academy, University of Gothenburg, Sweden (M.D., K.W.G., P.E., M.F., A.A., A.R., Z.M.).
Adult Congenital Heart Disease Unit (M.D., P.E., A.A., Z.M.), Sahlgrenska University Hospital, Gothenburg, Sweden.
Circulation. 2023 Mar 21;147(12):930-938. doi: 10.1161/CIRCULATIONAHA.122.060834. Epub 2022 Dec 26.
The survival of children with congenital heart disease has increased substantially over the past decades, with 97% currently reaching adulthood. The total effect of advanced treatment on future mortality and morbidity in adult survivors with congenital heart disease (CHD) is less well described.
We used data from the Swedish National Inpatient, Outpatient, and Cause of Death Register to identify patients with CHD who were born between 1950 and 1999 and were alive at 18 years of age. Ten controls identified from the Total Population Register were matched for year of birth and sex and with each patient with CHD. Follow-up was from 1968 and 18 years of age until death or at the end of the study (2017). Survival percentage with 95% CI for all-cause mortality were performed with Kaplan-Meier survival function. Cox proportional hazard regression models with hazard ratios (HRs) and 95% CI were used to estimate the risk of all-cause mortality.
We included 37 278 patients with adult CHD (ACHD) and 412 799 controls. Mean follow-up was 19.2 years (±13.6). Altogether, 1937 patients with ACHD (5.2%) and 6690 controls (1.6%) died, a death rate of 2.73 per 1000 person-years and 0.84 per 1000 person years, respectively. Mortality was 3.2 times higher (95% CI, 3.0-3.4; <0.001) among patients with ACHD compared with matched controls. Up to the maximum of 50 years of follow-up, >75% of patients with ACHD were still alive. Mortality was highest among patients with conotruncal defects (HR, 10.13 [95% CI, 8.78-11.69]), but also significantly higher for the more benign lesions, with the lowest risk in patients with atrial septal defects (HR, 1.36 [95% CI, 1.19-1.55]). At least 75% of patients with ACHD alive at 18 years of age lived past middle age and became sexagenerians.
In this large, nationwide, register-based cohort study of patients with ACHD surviving to 18 years of age, the risk of mortality up to 68 years of age was >3 times higher compared with matched controls without ACHD. Despite this, at least 75% of patients with CHD alive at 18 years of age lived past middle age and became sexagenerians. A notable risk decline in the mortality for patients with ACHD was seen for those born after 1975.
在过去几十年中，患有先天性心脏病的儿童的存活率大幅提高，目前有 97%的儿童能够成年。先进治疗对成年先天性心脏病（CHD）幸存者未来死亡率和发病率的总体影响描述得较少。
我们使用瑞典全国住院、门诊和死因登记处的数据，确定了 1950 年至 1999 年期间出生且在 18 岁时存活的 CHD 患者。从总人口登记处中选择了 10 名与 CHD 患者出生年份和性别相匹配的对照者，并与每位 CHD 患者相匹配。随访时间从 1968 年和 18 岁开始，直到死亡或研究结束（2017 年）。使用 Kaplan-Meier 生存函数计算全因死亡率的生存率和 95%置信区间。使用 Cox 比例风险回归模型和 95%置信区间估计全因死亡率的风险。
我们纳入了 37278 名患有成人 CHD（ACHD）的患者和 412799 名对照者。平均随访时间为 19.2 年（±13.6）。共有 1937 名 ACHD 患者（5.2%）和 6690 名对照者（1.6%）死亡，死亡率分别为每 1000 人年 2.73 例和 0.84 例。与匹配的对照组相比，ACHD 患者的死亡率高 3.2 倍（95%CI，3.0-3.4；<0.001）。在最长 50 年的随访中，>75%的 ACHD 患者仍然存活。在法洛四联症患者中死亡率最高（HR，10.13[95%CI，8.78-11.69]），但良性病变的死亡率也明显升高，而房间隔缺损患者的风险最低（HR，1.36[95%CI，1.19-1.55]）。至少 75%的 18 岁时存活的 ACHD 患者能够存活到中年并成为 60 多岁的老人。
在这项对存活至 18 岁的 ACHD 患者进行的大型、全国性、基于登记的队列研究中，与没有 ACHD 的匹配对照组相比，患者在 68 岁之前的死亡风险高出 3 倍以上。尽管如此，至少 75%的 18 岁时存活的 CHD 患者能够存活到中年并成为 60 多岁的老人。对于 1975 年后出生的患者，ACHD 患者的死亡率显著下降。
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