Nationwide Birth Prevalence of Crucial Congenital Heart Defects From 2014 to 2018 in Korea

Abstract

Background and objectives: A comprehensive survey of congenital heart disease (CHD) prevalence has not yet been conducted in South Korea. This study aimed to investigate the prevalence of CHDs in Korean children and lay the foundation for national CHD epidemiology.

Methods: Target patients were infantile crucial CHDs, which include critical CHDs (requiring urgent procedures after birth with common hypoxemic defects) and diverse categorical defects excluding simple shunt defects. Data were obtained from the National Health Insurance Service over a 5-year period (2014-2018). Birth prevalence (new cases per 1,000 live births) of CHDs in Korea was analyzed and compared with that of other countries.

Results: The birth prevalences of right heart obstructive defects (pulmonary valve stenosis and pulmonary atresia), conus anomalies (tetralogy of Fallot and double outlet right ventricle), and total anomalous pulmonary venous return showed significant increases in the East Asian group (P<0.001), whereas those of left heart obstructive defects (coarctation of aorta, aortic stenosis, and hypoplastic left heart syndrome), truncus anomalies (D-transposition of great artery and persistent truncus arteriosus), atrioventricular septal defect, and hypoplastic right heart syndrome were significantly decreased in the East Asian group (P<0.001).

Conclusions: The overall birth prevalence of crucial CHDs in Korea was similar to that of critical CHDs in previous studies from other countries. Some subtypes of right heart obstructive defects, left heart obstructive defects, and conotruncal anomalies showed significant differences between East Asian and Western populations. This study contributes to a foundation for national CHD epidemiology in Korean children.

Keywords: Congenital heart defect; Critical illness; Korea; Prevalence.

Figure 1. Flow chart of the selection of patients with crucial CHDs.

ASD = atrial septal defects; CHD = congenital heart diseases; PDA = patent ductus arteriosus; TTE = transthoracic echocardiography.

Figure 2. The trend differences of crucial CHDs between East Asian and Western groups (data were based on Table 3 and horizontal black lines are median values).

(A) The subtypes of crucial CHDs which showed significantly or insignificantly higher in the East Asian group than in the Western group. (B) The subtypes of crucial CHDs which showed significantly or insignificantly higher in the Western group than in the East Asian group. (C) The subtypes of crucial CHDs which did not show significant differences between the East Asian group and the Western group. AoS = aortic stenosis; AVSD = atrioventricular septal defects; CHD = congenital heart defect; CoA = coarctation of aorta; cTS = critical tricuspid stenosis; DORV = double outlet right ventricle; D-TGA = dextro-transposition of great artery; EbA = Ebstein anomaly; HLHS = hypoplastic left heart syndrome; HRHS = hypoplastic right heart syndrome; PTAr = persistent truncus arteriosus; PuA = pulmonary atresia; PVS = pulmonary valve stenosis; SV = single ventricle; TAPVR = total anomalous pulmonary venous return; TOF = tetralogy of Fallot.