Julius Center for Health Sciences and Primary Care, University Medical Center Utrecht, Utrecht, The Netherlands.
Eur Heart J. 2010 May;31(10):1220-9. doi: 10.1093/eurheartj/ehq032. Epub 2010 Mar 5.
Mortality in adults with congenital heart disease is known to be increased, yet its extent and the major mortality risks are unclear.
The Dutch CONCOR national registry for adult congenital heart disease was linked to the national mortality registry. Cox's regression was used to assess mortality predictors. Of 6933 patients, 197 (2.8%) died during a follow-up of 24 865 patient-years. Compared with the general national population, there was excess mortality, particularly in the young. Median age at death was 48.8 years. Of all deaths, 77% had a cardiovascular origin; 45% were due to chronic heart failure (26%, age 51.0 years) or sudden death (19%, age 39.1 years). Age predicted mortality, as did gender, severity of defect, number of interventions, and number of complications [hazard ratio (HR) range 1.1-5.9, P < 0.05]. Several complications predicted all-cause mortality beyond the effects of age, gender, and congenital heart disease severity, i.e. endocarditis, supraventricular arrhythmias, ventricular arrhythmias, conduction disturbances, myocardial infarction, and pulmonary hypertension (HR range 1.4-3.1, P < 0.05). These risks were similar in patients above and below 40 years of age. Almost all complications predicted death due to heart failure (HR range 2.0-5.1, P < 0.05); conduction disturbances and pulmonary hypertension predicted sudden death (HR range 2.0-4.7, P < 0.05).
Mortality is increased in adults with congenital heart disease, particularly in the young. The vast majority die from cardiovascular causes. Mortality risk, particularly by heart failure, is increased by virtually all complications. Complications are equally hazardous in younger as in older patients.
已知成人先天性心脏病的死亡率增加，但死亡率的程度和主要死亡风险尚不清楚。
荷兰成人先天性心脏病 CONCOR 国家注册处与国家死亡率登记处相联系。使用 Cox 回归评估死亡率预测因素。在 6933 例患者中，有 197 例（2.8%）在 24865 患者年的随访期间死亡。与一般人群相比，死亡率过高，尤其是在年轻人中。死亡的中位年龄为 48.8 岁。所有死亡中，77%有心血管起源；45%死于慢性心力衰竭（26%，年龄 51.0 岁）或猝死（19%，年龄 39.1 岁）。年龄预测死亡率，性别、缺陷严重程度、干预次数和并发症数量也是如此[风险比（HR）范围 1.1-5.9，P<0.05]。几种并发症预测全因死亡率，超出了年龄、性别和先天性心脏病严重程度的影响，即心内膜炎、室上性心律失常、室性心律失常、传导障碍、心肌梗死和肺动脉高压（HR 范围 1.4-3.1，P<0.05）。这些风险在 40 岁以上和以下的患者中相似。几乎所有并发症都预测心力衰竭死亡（HR 范围 2.0-5.1，P<0.05）；传导障碍和肺动脉高压预测猝死（HR 范围 2.0-4.7，P<0.05）。
成人先天性心脏病患者的死亡率增加，尤其是年轻人。绝大多数死于心血管原因。几乎所有并发症都会增加死亡率，特别是心力衰竭的风险。并发症在年轻患者和老年患者中的危害是相同的。
