Aims Updated knowledge on the rates and causes of death among children with severe congenital heart defects (CHDs) is needed to further improve treatment and survival. This study investigated nationwide mortality rates in children with severe CHDs with an emphasis on unexpected mortality unrelated to cardiac intervention. Methods and results Data on all pregnancies and live-born children in Norway from 2004 to 2016 were obtained from national registries, the Oslo University Hospital’s Clinical Registry for CHDs and medical records. Among 2359 live-born children with severe CHDs, 234 (10%) died before 2 years of age. Of these, 109 (46%) died in palliative care, 58 (25%) died of causes related to a cardiac intervention and 67 (29%) died unexpectedly and unrelated to a cardiac intervention, either before (n=26) or following (n=41) discharge after a cardiac intervention. Comorbidity (38/67, 57%), persistent low oxygen saturation (SaO2; <95%; 41/67, 61%), staged surgery (21/41, 51%), residual cardiac defects (22/41, 54%) and infection (36/67, 54%) were frequent in children who died unexpectedly unrelated to an intervention. Two or more of these factors were present in 62 children (93%). The medical reports at hospital discharge lacked information on follow-up in many patients who died unexpectedly. Conclusions The numbers of unexpected deaths unrelated to cardiac intervention in children <2 years of age without comorbidity were low in Norway. However, close follow-up is recommended for infants with comorbidities, persistent low oxygen saturation, staged surgery or residual cardiac defects, particularly when an infection occurs. Data are stored in ‘eReg Versjon 2.4.0 OUS-HF’ and are available on reasonable request. They are not to be reused without approval from the Regional Committee for Medical and Health Research Ethics South East Norway.

中文翻译：

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2004-2016 年挪威严重先天性心脏缺陷儿童的意外死亡

目的 需要对严重先天性心脏病 (CHD) 儿童的死亡率和死亡原因进行更新，以进一步改善治疗和生存。本研究调查了全国范围内严重 CHD 儿童的死亡率，重点是与心脏干预无关的意外死亡率。方法和结果 2004 年至 2016 年挪威所有妊娠和活产儿的数据来自国家登记处、奥斯陆大学医院的 CHD 临床登记处和医疗记录。在 2359 名重度 CHD 活产儿中，234 名（10%）在 2 岁前死亡。其中，109 人 (46%) 死于姑息治疗，58 人 (25%) 死于与心脏介入相关的原因，67 人 (29%) 意外死亡且与心脏介入无关，在心脏介入治疗后出院之前 (n=26) 或之后 (n=41)。合并症 (38/67, 57%)、持续低氧饱和度 (SaO2; <95%; 41/67, 61%)、分期手术 (21/41, 51%)、残余心脏缺陷 (22/41, 54%) ) 和感染 (36/67, 54%) 在与干预无关的意外死亡儿童中很常见。62 名儿童 (93%) 存在两个或更多这些因素。出院时的医疗报告缺乏许多意外死亡患者的随访信息。结论 在挪威，与心脏干预无关的 2 岁以下无合并症儿童的意外死亡人数较低。但是，建议对有合并症、持续低氧饱和度、分期手术或残余心脏缺陷的婴儿进行密切随访，尤其是在发生感染时。数据存储在“eReg Versjon 2.4.0 OUS-HF”中，可根据合理要求提供。未经挪威东南部医学与健康研究伦理区域委员会批准，不得重复使用。