• Journal of Chest Surgery
• /
• v.17 no.3
• /
• pp.371-380
• /
• 1984

Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.

• Clinical and Experimental Pediatrics
• /
• v.55 no.11
• /
• pp.414-419
• /
• 2012

Purpose: This study aimed to assess early development in very low birth weight (VLBW) infants with mild intraventricular hemorrhage (IVH) or those without IVH and to identify the perinatal morbidities affecting early neurodevelopmental outcome. Methods: Bayley Scales of Infant Development-II was used for assessing neurological development in 49 infants with a birth weight <1,500 g and with low grade IVH (${\leq}$grade II) or those without IVH at a corrected age of 12 months. Results: Among the 49 infants, 19 infants (38.8%) showed normal development and 14 (28.6%) showed abnormal mental and psychomotor development. Infants with abnormal mental development (n=14) were mostly male and had a longer hospitalization, a higher prevalence of patent ductus arteriosus (PDA) and bronchopulmonary dysplasia (BPD), and were under more frequent postnatal systemic steroid treatment compared with infants with normal mental development (n=35, P<0.05). Infants with abnormal psychomotor development (n=29) had a longer hospitalization and more associated PDA compared to infants with normal psychomotor development (n=20, P<0.05). Infants with abnormal mental and psychomotor development were mostly male and had a longer hospitalization and a higher prevalence of PDA and BPD compared to infants with normal mental and psychomotor development (n=19, P<0.05). Using multiple logistic regression analysis, a longer duration of hospitalization and male gender were found to be significant risk factors. Conclusion: Approximately 62% of VLBW infants with low grade IVH or those without IVH had impaired early development.

• Clinical and Experimental Pediatrics
• /
• v.56 no.3
• /
• pp.112-115
• /
• 2013

Purpose: To investigate the association between necrotizing enterocolitis (NEC) and red blood cell transfusions in very low birth weight (VLBW) preterm infants. Methods: We studied were 180 VLBW preterm infants who were admitted to the neonatal intensive care unit of CHA Gangnam Hospital from January of 2006 to December of 2009. The subjects were divided into 2 groups: an NEC group (greater than stage II on the modified Bell's criteria) and a control group (less than stage II on the modified Bell's critieria). We defined red blood cell transfusion before NEC diagnosis as the frequency of transfusion until NEC diagnosis (mean day at NEC diagnosis, day 18) in the NEC group and the frequency of transfusion until 18 days after birth in the control group. Results: Of the 180 subjects, 18 (10%) belonged to the NEC group, and 14 (78%) of these 18 patients had a history of transfusion before NEC diagnosis. The NEC group received $3.1{\pm}2.9$ transfusions, and the control group received $1.0{\pm}1.1$ transfusions before the NEC diagnosis (P=0.005). In a multivariate logistic regression corrected for gestational age, Apgar score at 1 minute, the presence of respiratory distress syndrome, patent ductus arteriosus, premature rupture of membrane, disseminated intravascular coagulopathy and death were confounding factors. The risk of NEC increased 1.63 times (95% confidence interval, 1.145 to 2.305; P=0.007) with transfusion before the NEC diagnosis. Conclusion: The risk for NEC increased significantly with increased transfusion frequency before the NEC diagnosis.

• Journal of Yeungnam Medical Science
• /
• v.5 no.2
• /
• pp.101-110
• /
• 1988

Between April, 1984 and September, 1988, 459 patients underwent cardiovascular surgery at the Yeungnam University Hospital. Of these, 355 cases were open heart surgeries and 104 cases were non-open heart surgeries. There were 237 patients of acyanotic congenital cardiac anomalies, 40 patients of cyanotic congenital cardiac anomalies, and 85 patients of acquired heart diseases. The sex ratio of cardiovascular diseases was represented as 1:1.3 in male and female. The age distribution was ranged from 1 day to 65 years old. The common congenital cardiovascular anomalies were ventricular septal defect(38.7%), patent ductus arteriosus(25.5%), atrial septal defect(20.7%), Tetralogy of Fallot(8.3%), and pulmonary stenosis(2.4%) in order of frequency. Among 87 acquired cardiovascular diseases, 81 patients underwent operation for cardiac valvular lesions. 51 patients had mitral valve replacement and 13 patients had aortic valve replacement and 17 patients had double valve replacement. The overall mortality of cardiovascular surgery was 3.3% and mortality of open heart surgery was 3.9%.

• Journal of Chest Surgery
• /
• v.11 no.1
• /
• pp.46-57
• /
• 1978

Ventricular septal defect was the most common congenital Heart disease admitted to the Severance Hospital from December, 1963 to June 30. 1977. A total of 1,811 cardiac patients received cardiac catheterization in the CardiacCenter of Yonsei University Medical College. Out of the 1,811 cardiac patients, 791 cases had congenital heart disease and of these 172 cases had ventricular septal defect. This amounted to 21.7% of all those with congenital heart disease. Seventy cases of ventricular septal defect operated on in the chest surgery department are presented. Of these 70 cases of ventricular septal defect, 54 cases were male and 16 cases were female. Their ages ranged from 4/12 months to 32 years. The ratio of systolic pressure of the main pulmonary to systemic artery [Pp/Ps] for 29 of the 59 isolated ventricular septal defects was below 45 percent. Pp/Ps ratio for 19 of the 59 isolated ventricular septal defects was 75 percent. The patients were mostly below fifteen years of age. Out of 64 cases, there were 36 cases of type II defects, 20 cases of type I, 4 cases of type III , 2 cases of type IV, one case of both type II and one case of left ventricular right atrial communication. The anomalies associated with ventricular septal defect were 13 in all; 4 cases of aortic insufficiency, 3 cases of ruptured aneurysm of the sortie sinus of valsalva, 2 cases of ruptured aneurysm of the sortie sinus of valsalva with aortic insufficiency, 2 cases of patent ductus arteriosus, one case of ruptured aneurysm of the aortic sinus of valsalva with atrial septal defect [secundum type] and one case of atrial septal defect [secundum type]. Overall mortality was 8.6 percent. The mortality in pulmonary artery banding was 37.5 percent. The causes of mortality were in one case congestive heart failure, in one case asphyxia and in one case respiratory insufficiency. Tile mortality in ventricular septal defect associated with pulmonary hypertension was 7.1 percent. The cause of mortality was in one case low cardiac output syndrome. There was no mortality in the ventricular septal defects without pulmonary hypertension and mortality in the ventricular septal defect. In ventricular septal defect associated with combined anomalies, the causes mortality were in one case respiratory insufficiency and in one case congestive heart failure.

• Journal of Chest Surgery
• /
• v.23 no.6
• /
• pp.1057-1066
• /
• 1990

The possible relationship between pulmonary vascular pathologic changes and an increased pulmonary blood flow and pulmonary blood pressure has been the subject of investigation for many years. In an attempts to study the effects of increased pulmonary blood flow. long-term observations have been made in neonatal goats in which the total pulmonary vascular bed was restricted by means of ligation of left pulmonary artery, thereby diverting the total right ventricular output through the right lung. The left pulmonary artery and patent ductus arteriosus were ligated in 6 neonatal goats of under 3 weeks of age, and the goats were put to death at interval between 1 and 7 months of age. Pulmonary arterial development in both right and left lungs was studied by applying quantitative morphometric techniques, and compared with control group of goats between 1 and 6 months of age. The axial pulmonary artery and its branches were larger in the right lung than in the control group in all animals, and they were abnormally small in the left lung. In the right lung, arteries smaller than 50\ulcornerm showed abnormal increase in `% wall thickness’ in postoperative 2,3,5 and 6th months[p<0.05]. The proportion of non-muscular arteries was over 50% at postoperative 1st month in both right and left lungs, but an increase in proportion of partially muscular and wholly muscular arteries occurred thereafter. The ratio of alveoli/arteries was lower than normal in the right lung of postoperative 1st month[p<0.05], but was elevated thereafter. The failure to perfuse one pulmonary artery in neonatal goats changed growth and development of both lungs.

• Clinical and Experimental Pediatrics
• /
• v.59 no.2
• /
• pp.91-95
• /
• 2016

We report the case of a 22-month-old boy with a new mosaic partial unbalanced translocation of 1q and 18q. The patient was referred to our Pediatric Department for developmental delay. He showed mild facial dysmorphism, physical growth retardation, a hearing disability, and had a history of patent ductus arteriosus. White matter abnormality on brain magnetic resonance images was also noted. His initial routine chromosomal analysis revealed a normal 46,XY karyotype. In a microarray-based comparative genomic hybridization (aCGH) analysis, subtle copy number changes in 1q32.1-q44 (copy gain) and 18q21.33-18q23 (copy loss) suggested an unbalanced translocation of t(1;18). Repeated chromosomal analysis revealed a low-level mosaic translocation karyotype of 46,XY,der(18)t(1;18) (q32.1;q21.3)[12]/46,XY[152]. Because his parents had normal karyotypes, his translocation was considered to be de novo. The abnormalities observed in aCGH were confirmed by metaphase fluorescent in situ hybridization. We report this patient as a new karyotype presenting developmental delay, facial dysmorphism, cerebral dysmyelination, and other abnormalities.

• Clinical and Experimental Pediatrics
• /
• v.58 no.6
• /
• pp.224-229
• /
• 2015

Purpose: Thyroid dysfunction is common in preterm infants. Congenital hypothyroidism causes neurodevelopmental impairment, which is preventable if properly treated. This study was conducted to describe the characteristics of thyroid dysfunction in very low birth weight infants (VLBWIs), evaluate risk factors of hypothyroidism, and suggest the reassessment of thyroid function with an initially normal thyroid-stimulating hormone (TSH) as part of a newborn screening test. Methods: VLBWIs (January 2010 to December 2012) were divided into two groups according to dysfunction-specific thyroid hormone replacement therapy, and associated factors were evaluated. Results: Of VLBWIs, 246 survivors were enrolled. Only 12.2% (30/246) of enrolled subjects exhibited thyroid dysfunction requiring thyroid hormone replacement. Moreover, only one out of 30 subjects who required thyroid hormone treatment had abnormal thyroid function in the newborn screening test with measured TSH. Most of the subjects in the treatment group (22/30) exhibited delayed TSH elevation. Gestational age, Apgar score, antenatal steroids therapy, respiratory distress syndrome, patent ductus arteriosus, sepsis, intraventricular hemorrhage, postnatal steroids therapy, and duration of mechanical ventilation did not differ between the two groups. Birth weight was smaller and infants with small for gestational age were more frequent in the treatment group. Conclusion: Physicians should not rule out suggested hypothyroidism, even when thyroid function of a newborn screening test is normal. We suggest retesting TSH and free thyroxine in high risk preterm infants with an initially normal TSH level using a newborn screening test.

• Journal of Chest Surgery
• /
• v.43 no.5
• /
• pp.518-521
• /
• 2010

A one-day-old baby was transferred for cyanosis and heart murmur. Echocardiographic evaluation revealed that he had severe neonatal Ebstein's anomaly (Carpentier type C), pulmonary atresia, and pulmonary circulation via patent ductus arteriosus. Because the wall of the atrialized right ventricle was very thin, showed decreased contractility, and the small right ventricle showed pulmonary atresia, we decided that a two-ventricular repair was impossible. When the patient was one-month-old, he underwent right atrium reduction-plasty, a right ventricular exclusion procedure (including atrialized right ventricle resection and functional right ventricle plication), and right modified Blalock-Taussig shunt. He was discharged without specific problems. He received a bidirectional cavopulmonary shunt successfully at 4 months later.

• Journal of Chest Surgery
• /
• v.41 no.4
• /
• pp.499-503
• /
• 2008

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.