Missed diagnosis of critical congenital heart disease

Missed diagnosis of critical congenital heart disease
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DOI:
10.1001/archpedi.162.10.969
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发表时间:
2008-10-01
影响因子:
--
通讯作者:
Rodriguez, Sandra
Rodriguez, Sandra
中科院分区:
其他
文献类型:
--
作者:
Chang, Ruey-Kang R.;Gurvitz, Michelle;Rodriguez, Sandra

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目的:评估重症先天性心脏病的漏诊率、临床和人口学特征。设计:对-2004年加州死亡登记数据进行基于人群的回顾性研究。地点:加利福尼亚州。参与者:研究队列包括898名出生1~364天、未接受手术或手术状态不明的危重先天性心脏病死亡的婴儿。在所有符合这些初始标准的患者中,我们检查了(1)是否进行了尸检并使用尸检结果来确定死因,(2)是否进行了尸检但结果没有用来确定死因,以及(3)患有发育不良的左心综合征(HLHS)的婴儿是否有潜在的舒适护理。主要观察指标:遗漏和可能延迟诊断CCHD。结果:在152名漏诊的CCHD婴儿中,死亡时的中位年龄为13.5天。超过50%的被漏诊的患者(n=78)死于家中或医院急诊科。最常见的诊断是HLHS和主动脉缩窄。在加利福尼亚州,平均每年有10名患者错过CCHD诊断,20名患者延迟诊断。每年漏诊或迟诊的总人数在1989-1999年间呈下降趋势,在2000-2004年间保持不变。结论:加州每年有多达30名婴儿死于漏诊或可能迟诊的CCHD。大多数因漏诊而死亡的原因是HLHS和主动脉缩窄。由于死亡年龄中位数小于2周,应在出生后3至5天第一次到儿科医生办公室就诊时对左心阻塞性冠心病进行仔细的心血管评估。
Objectives: To evaluate the rate and the clinical and demographic characteristics of missed diagnosis of critical congenital heart disease (CCHD).Design: Population-based retrospective study of 1989-2004 California statewide death registry data.Setting: California.Participants: The study cohort consisted of 898 infants who died of CCHD at 1 to 364 days of age who either did not undergo surgery or had an unknown surgery status. From all patients who met these initial criteria, we examined (1) whether autopsies were performed and autopsy results were used to establish the cause of death, (2) whether autopsies were performed but the results were not used to establish a cause of death, and (3) whether infants with hypoplastic left heart syndrome (HLHS) were potentially receiving comfort care.Main Outcome Measures: Missed and possibly late diagnosis of CCHD.Results: Among 152 infants with a missed CCHD diagnosis, the median age at death was 13.5 days. More than 50% of patients with a missed CCHD diagnosis (n = 78) died at home or in the hospital emergency department. The most common diagnoses were HLHS and coarctation of aorta. There were an average of 10 patients with missed CCHD diagnoses and 20 patients with late diagnoses in California per year. The total annual number of patients with missed or late diagnoses decreased in 1989-1999 and remained unchanged in 2000-2004.Conclusions: Up to 30 infants per year died of a missed or possibly late diagnosis of CCHD in California. Most deaths due to a missed diagnosis were from HLHS and coarctation of the aorta. Because the median age at death was younger than 2 weeks, a careful cardiovascular evaluation for left heart obstructive CHD should be performed during the first postdischarge visit to a pediatrician's office at 3 to 5 days of age.