Objectives: 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.
Methods: 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.
Results: 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.
Conclusions: 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.