Capozzi Giovanbattista, Caputo Salvatore, Pizzuti Renato, Martina Lucia, Santoro Michele, Santoro Giuseppe, Sarubbi Berardo, Iacono Carola, D'Alto Michele, Bigazzi Maurizio Cappelli, Pacileo Giuseppe, Merlino Ettore, Caianiello Giuseppe, Russo Maria Giovanna, Calabrò Raffaele
Pediatric Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
J Cardiovasc Med (Hagerstown). 2008 Apr;9(4):368-74. doi: 10.2459/JCM.0b013e3282eee866.
Congenital heart disease (CHD), the most common cardiac malformation in the fetal and neonatal period, represents a heterogeneous group of defects with little known cause. The aim of this study was to investigate the incidence rate (IR) of CHD without spontaneous resolution in the first year of life, the age at diagnosis, the most common type of CHD, and the IR changes in severe and nonsevere CHD during the study period.
From January 1997 to December 2002, 4559 live births with CHD (diagnosed until December 2004) were enrolled in the Campania Region. Number of live-born children by month was derived from national registries.
About 70% of CHD patients were diagnosed within the first year of life. The overall mean regional IR of CHD was 11.1 (confidence interval 10.8-11.4) patients per 1000 live-born children. The overall mean IR of CHD was significantly different in the five provinces of the Campania Region, but the annual IR remained stable in each of them throughout the study period. The relative risk in live-born children and residents in the areas with an elevated IR of CHD was higher for nonsevere CHD, but of low significance for severe CHD. At least one-third of CHD patients had atrial septal defects.
We described the highest IR of CHD from one of the largest samples used up to now for this purpose (410 181 live-born children), but we think that this value could be higher (poor surveillance in some areas). Moreover, this study increases the awareness that CHD patients with a few symptoms are frequently underdiagnosed in areas with inadequate health services and that by an improved availability of pediatric echocardiographic assesments seem warranted. Eight years of surveillance and an increasing attention of both pediatricians and cardiologists to mild CHD in some provinces allowed us to demonstrate that, despite previous reports, the most common CHD in our region was atrial septal defect.
先天性心脏病（CHD）是胎儿和新生儿期最常见的心脏畸形，是一组病因不明的异质性缺陷。本研究的目的是调查出生后第一年未自然消退的CHD发病率、诊断年龄、最常见的CHD类型以及研究期间重度和非重度CHD的发病率变化。
1997年1月至2002年12月，坎帕尼亚地区纳入了4559例患有CHD的活产婴儿（截至2004年12月诊断）。每月活产婴儿数量来自国家登记处。
约70%的CHD患者在出生后第一年内被诊断。CHD的总体区域平均发病率为每1000例活产婴儿中有11.1例（置信区间10.8 - 11.4）。坎帕尼亚地区五个省份的CHD总体平均发病率有显著差异，但在整个研究期间每个省份的年发病率保持稳定。CHD发病率升高地区的活产婴儿和居民中，非重度CHD的相对风险较高，但重度CHD的相对风险较低。至少三分之一的CHD患者患有房间隔缺损。
我们描述了目前用于此目的的最大样本之一（410181例活产婴儿）中CHD的最高发病率，但我们认为这个值可能更高（一些地区监测不足）。此外，本研究提高了人们的认识，即在医疗服务不足的地区，有一些症状的CHD患者经常被漏诊，并且似乎有必要提高儿科超声心动图评估的可及性。八年的监测以及一些省份的儿科医生和心脏病专家对轻度CHD的日益关注使我们能够证明，尽管有先前的报道，但我们地区最常见的CHD是房间隔缺损。
