Department of Cardiology, Rigshospitalet and Danish National Research Foundation Centre for Cardiac Arrhythmia (DARC), Copenhagen, Denmark.
Eur Heart J. 2014 Apr;35(13):868-75. doi: 10.1093/eurheartj/eht509. Epub 2013 Dec 15.
Hitherto, sudden cardiac death in children (SCDc)-defined as sudden cardiac death (SCD) in the 1-18 years old-has been incompletely described in the general population. Knowledge on incidence rates, causes of death and symptoms prior to death is sparse and has been affected by reporting and referral bias.
In a nationwide setting all deaths in children aged 1-18 years in Denmark in 2000-06 were included. To chart causes of death and incidence rates, death certificates and autopsy reports were collected and read. By additional use of the extensive healthcare registries in Denmark, we were also able to investigate prior disease and symptoms. During the 7-year study period there was an average of 1.11 million persons aged 1-18 years. There were a total of 1504 deaths (214 deaths per year) from 7.78 million person-years. A total of 114 (7.5%) were sudden and unexpected. A cardiac disease was known prior to death in 18% of all sudden unexpected death cases. In two-thirds of all sudden unexpected death cases no previous medical history was registered. Causes of death in autopsied cases were cardiac or unknown in 70%. Unexplained deaths, presumed to be a primary cardiac arrhythmia, accounted for 28% of autopsied sudden unexpected death cases. Autopsy rate was 77%. There were a total of 87 cases of SCDc (5.8% of all deaths). Prodromal symptoms were noted in 26% and antecedent symptoms in 45% of SCDc cases. The most frequent antecedent symptoms were seizures, dyspnoea, and syncope. In total, 61% of SCDc were not known with any prior disease; 23% were known with congenital or other heart disease prior to death. In total, 43 (49%) of all sudden unexpected deaths died of a potential inherited cardiac disease. The incidence rate of sudden unexpected death was 1.5 per 100 000 person-years. The highest possible incidence rate of SCDc was 1.1 per 100 000 person-years.
From a nationwide study of all deaths in a 7-year period more than half of all victims of SCDc experienced antecedent and/or prodromal symptoms prior to death. The incidence rate of sudden death and SCDc was 1.5 and 1.1 per 100 000 person-years, respectively. Cardiac symptoms in young persons should warrant clinical work-up and an autopsy should be performed in all cases of sudden unexpected death in which the deceased was not known with congenital heart disease prior to death. This is pivotal, in the subsequent familial cascade screening, to diagnose and treat potential inherited cardiac diseases in family members.
迄今为止，儿童心源性猝死（SCDc）-定义为 1-18 岁儿童的心源性猝死（SCD）-在一般人群中尚未得到充分描述。关于发病率、死因和死亡前症状的知识很少，并且受到报告和转诊偏倚的影响。
在全国范围内，纳入了丹麦 2000-06 年 1-18 岁儿童的所有死亡病例。为了绘制死因和发病率图，收集并阅读了死亡证明和尸检报告。通过在丹麦广泛使用医疗保健登记册，我们还能够调查先前的疾病和症状。在 7 年的研究期间，平均有 110 万人年龄在 1-18 岁之间。共有 1504 人（每年 214 人）死于 770 万人年。总共有 114 人（7.5%）是突然和意外的。在所有突然和意外死亡病例中，有 18%的病例在死亡前已知患有心脏疾病。在所有突然和意外死亡病例中，三分之二的病例没有记录先前的病史。尸检病例的死因在心脏或未知的占 70%。无法解释的死亡，推测为原发性心律失常，占尸检中突然和意外死亡病例的 28%。尸检率为 77%。共有 87 例 SCDc（所有死亡人数的 5.8%）。在 26%的病例中注意到前驱症状，在 45%的病例中注意到前驱症状。最常见的前驱症状是癫痫发作、呼吸困难和晕厥。总共有 61%的 SCDc 没有任何先前的疾病；23%在死亡前已知患有先天性或其他心脏病。总共有 43 例（49%）所有突然和意外死亡是潜在遗传性心脏病引起的。突然意外死亡的发病率为每 100000 人年 1.5 例。最高可能的 SCDc 发病率为每 100000 人年 1.1 例。
从一项为期 7 年的全国性研究来看，超过一半的 SCDc 患者在死亡前经历了前驱和/或前驱症状。猝死和 SCDc 的发病率分别为每 100000 人年 1.5 和 1.1 例。年轻人的心脏症状应进行临床检查，所有非先天性心脏病患者突然意外死亡均应进行尸检。这对于随后的家族级联筛查至关重要，以便在家族成员中诊断和治疗潜在的遗传性心脏病。
