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Congenital Heart Disease in Neonatal Period and Infancy .

Journal of the Korean Pediatric Society 1984;27(6):570-582.
Published online June 30, 1984.
Congenital Heart Disease in Neonatal Period and Infancy .
Jung Yun Choi, Hey Sun Lee
Department of Pediatrics, College of Medicine, Seoul National Universityt Seoul, Korea
新生兒 및 嬰兒의 先天性 心疾患에 對한 임상적 관찰
최정연, 이혜선
서울대학교 의과대학 소아과학교실
From July 1980 to June 1983, total 468 cases of infants of congenital heart disease were studied, who needed intensive care in infancy at Seoul National University Hospital. 1)Among 468 cases, 285 cases were diagnosed by cardiac catheterization or cardiac surgery and the other 183 cases by only clinical base. 2)Relative frequencies of these individual anomalies were in order of ventricular septal defect 34.8%, transposition of great arteries 10.9%, tetralogy of Fallot 7.3%, patent ductus arter iosus 5.8%, and pulmonary atresia 5.1%. The frequency of coarctation of aorta was only 2.1% and pulmonic stenosis 0.9%, hypoplastic left heart syndrome 0.2%. There was no-case of aortic stenosis. 3)Male babies predominated at 58.3%. The high proportion of males were seen for transposition of great arteries, pulmonary atresia, heterotaxia, single ventricle and total anomalous pulmonary venous return. Infants with patent ductus arteriosus or atrial septal defect were predominantly female. 4) First visit to our hospital for 5.8% of total cases was during first week of life, when transposition of great arteries and pulmonary atresia were the most common lesion. During first month of life after 1 week, 9.0% of total cases first visited,. when transposition of great arteries and ventricular septal defect were the common lesion. 26.9% of total cases first visited in the period of second and third month of life, when ventricular septal defect and transposition of great arteries were common. 34.4% of total cases first visited after 3 months of age and before 6 months of age, and ventricular septal defect and patent ductus arteriosus were common lesion encountered during this period. 5)The proportion of infants who weighed less than 2.5kg and more than 4.0kg at birth was 6% and 5% respectively. 24% of transposition of great arteries weighed more than 4.0kg at birth. The premature baby less than 37 weeks of gestational period was 3% of total cases, and postmaturity more than 42 weeks of gestation, 1%. These congenital heart disease infants tended to have high maternal age such as 30% of them was more than 30 years old, 6) There were 61 cases (13%) who had associated extracardiac anomalies, which were common in musculoskeletal and gastrointestinal system. Down syndrome was present in 12 cases and accounted for 20% of the extracardiac anomalies. The association rate of extracardiac anomalies in individual cardiac lesions were 42.9% in endocardial cushion defect, which was most common, 22.2% in patient ducts arteriosus, 20.8% in pulmonary atresia, 15.6% in tetralogy of Fallot, 14.3% in total anomalous pulmonary venous return and 14.1% in ventricular septal defect. 7)The overall first-year mortality was 32.1%. The first-year mortality of infants who visited in the first week of life was 71.4% and in the first month after 1 week of life, 41.9%; 1~3 months of age, 42%; 3~6 months of age, 24.4% ; and 6~12 months of age, 22.2%. The high mortality rate was noted in transposition of great arteries (59%), single ventricle (50%), tricuspid atresia (63%), total anomalous pulmonary venous return (64%) and coarctation of aorta(50%). The mortality rate of infants with medical management was 39.5% and with surgical management 20.3%. Medical mortality was hirfi in total anomalous pulmonary venous return (100%), transposition of great arteries(61%) and coarctation of aorta (60%). Opera tive mortality was high in transposition of great arteries(58%), pulmonary atresia (30%),double outlet right ventricle(50%) and heterotaxia (33%). Mortality related with cardiac catheterization was 3.9% and mostly noted with single ventricle, tricuspid atresia and total anomalous pulmonary venous return. Of the 285 confirmed cases, 128(45%) underwent cardiac surgery during the first year of life. Of these, 28 (20%) expired mostly by 30 days after surgery. Mortality with open heart surgery was 17% and with closed heart surgery, 27.5%.
Key Words: Congenital heart disease, Neonate, Infancy

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