RESUMEN
PURPOSE: This is a retrospective descriptive study to determine the incidence of congenital heart disease(CHD) in Jeju and to estimate the suitability of the Jeju population as the subject for a regional birth cohort study. METHODS: All patients with CHD diagnosed by two dimensional echocardiography in Cheju National University Hospital and Halla General Hospital from January 1999 to March 2003 were included in this study. RESULTS: From April 1999 to March 2002, the crude incidence rate was 10.06 per 1,000 live births (236 cases/23,464 live births). Among the 236 cases, the proportion of each type was as follows: Ventricular septal defect(44.9%), atrial septal defect(21.2%), pulmonary stenosis(11.0%), patent ductus arterios us(9.3%), tetralogy of Fallot(3.4%), transposition of great arteries(2.1%), aortic stenosis(1.3%), hypoplastic left heart syndrome(1.3%), coarctation of aorta(0.8%), total anormalous pulmonary venous return(0.8%), tricuspid atresia(0.8%), heterotaxia(0.4%), single ventricle(0.4%), atrioventricular septal defect(0.4%), Ebstein anomaly(0.4%). The male to female ratio was 1:1.380(P=0.011). For unknown reasons, females showed significantly higher incidence in VSD(P=0.005) and PDA(P=0.019). Mortality rate was 0.34 per 1,000 live births. The types of VSD were 72.1% of perimembranous, 16.4% of trabecular muscular, 5.7% of subarterial, and 5.7% of unknown. CONCLUSION: This is the first regional study on the incidence of CHD in Korea. The incidence of CHD in Jeju was 10.06 per 1,000 live births. Most of our cases were VSDs and ASDs. Jeju would be a good subject for a regional cohort study in the future.