BACKGROUND: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.METHODS: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.RESULTS: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; P<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.CONCLUSIONS: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 noted for those born after 1975.

中文翻译：

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患有先天性心脏病的成年人：中年以后的无事件生存趋势

背景：在过去的几十年里，患有先天性心脏病的儿童的存活率大幅提高，目前有 97% 的人已经长大成人。先进治疗对成年先天性心脏病 (CHD) 成年幸存者未来死亡率和发病率的总体影响尚不清楚。出生于 1950 年至 1999 年之间，现年 18 岁。从总人口登记册中确定的 10 个对照与出生年份和性别以及每个患有 CHD 的患者相匹配。随访时间为 1968 年至 18 岁，直至死亡或研究结束（2017 年）。使用 Kaplan-Meier 生存函数计算全因死亡率和 95% CI 的生存百分比。使用具有风险比 (HR) 和 95% CI 的 Cox 比例风险回归模型来估计全因死亡率的风险。结果：我们纳入了 37 278 名成人冠心病 (ACHD) 患者和 412 799 名对照。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 人和每 1000 人年 0.84 人。死亡率高出 3.2 倍（95% CI，3.0–3.4；P<0.001) 在 ACHD 患者中与匹配的对照组相比。在最长 50 年的随访中，>75% 的 ACHD 患者仍然存活。圆锥体缺损患者的死亡率最高 (HR, 10.13 [95% CI, 8.78–11.69])，但良性病变患者的死亡率也显着更高，房间隔缺损患者的死亡率最低 (HR, 1.36 [95%]置信区间，1.19–1.55]）。至少 75% 的 ACHD 患者在 18 岁时还活着，并且活过中年并成为六十多岁的人。与没有 ACHD 的匹配对照相比，68 岁以下的人患病率高 >3 倍。尽管如此，至少有 75% 的冠心病患者活到 18 岁，活过中年，成为六十多岁的人。1975 年以后出生的 ACHD 患者死亡率显着降低。