• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.195-202
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• 2010

Purpose : Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. Methods : We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (${\geq}8$ Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. Results : Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs ${\geq}1.5$). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. Conclusion : Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

• Clinical and Experimental Pediatrics
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• v.45 no.12
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• pp.1528-1533
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• 2002

Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

• Pediatric Infection and Vaccine
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• v.12 no.1
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• pp.67-74
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• 2005

Purpose : The incidence of mortality associated with respiratory difficulties is decreasing nowadays contributed to the development of neonatology. However, complications associated with mechanical ventilator are increasing. This study is to determine clinical manifestations, diagnositc availability of the endotracheal tip culture in patients with Ventilator-Associated Pneumonia(VAP) in neonatal intensive care unit(NICU). Methods : A retrospective analysis of 50 neonates who were admitted to the NICU of Kangnam Sacred Heart Hospital and had given mechanical ventilator from 1 January 2000 to 30 June 2003. VAP group defined as neonates who had pneumonia with mechanical ventilation longer than 48 hours. They were classified into VAP group(n=13) and control group (n=37) and the prevalence, microorganisms cultured from the endotracheal tube tip and risk factors were investigated. Results : The prevalence of VAP was 26.0%(n=13) and the most dominant microorganism cultured in our NICU was methicillin-resistant coagulase negative staphylococcus(MR-CNS) in 4 cases. Other microorganisms were Pseudomonas, Enterobacter, methicillin-resistant Staphylococcus aureus(MRSA) and Klebsiella. Gestational age, birth weight, Apgar score, respiratory distress syndrome, retinopathy of prematurity, bronchopulmonary dysplasia, sepsis, renal failure, pulmonary hemorrhage, pneumothorax were not different significantly between two groups except intraventricular hemorrhage(P<0.001) and patent ductus arteriosus(P<0.05). Duration of hospital stay and mortality rate were also not different significantly. Conclusion : VAP occurred at a significant rate among mechanically ventilated NICU patients. Despite of limitation of encotracheal tip culture, the most common microorganism was MR-CNS. We should be aware of occurrence of VAP in NICU neonate who were with mechanical ventilator and should treat with great care.

• Neonatal Medicine
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• v.17 no.1
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• pp.21-33
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• 2010

Purpose : The increased survival of preterm infants in the neonatal intensive care unit recently has resulted in an increased frequency of bronchopulmonary dysplasia (BPD), especially with atypical forms. However, there have been few studies compairing classic and atypical BPD. The aim of this study was to investigate the differences between these two types of BPD. Methods : Infants with a gestational age less than 32 weeks born at the Seoul National University Hospital and Bundang Seoul National University Hospital from May 2004 to April 2007 were included. The data were categorized in 2 groups, classic and atypical BPD. We determined the incidence of BPD, and compared perinatal factors and postnatal managements between two groups. Results : Among 260 study subjects, 141 (54.2%) infants had BPD. Classic BPD infants were 64 and atypical BPD infants were 77. Comparison of differences between 2 groups, classic BPD infants were associated with respiratory distress syndrome, patent ductus arteriosus, intrauterine growth restriction, more high-frequency ventilator (HFV) use, low 1 and 5 minute Apgar scores. Atypical BPD infants were associated with antenatal steroid use, maternal premature rupture of membrane and chorioamnionitis (CAM). In multivariate analysis, more HFV use was associated with classic BPD. Antenatal steroid use, clinical CAM and histological CAM were associated with atypical BPD. Conclusion : The results of this study showed that antenatal factors (antenatal steroid use, clinical CAM, histological CAM) were associated with atypical BPD and postnatal factors (HFV used more) were associated with classic BPD. Further studies are needed for prevention and treatment of BPD.

• Neonatal Medicine
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• v.16 no.2
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• pp.197-204
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• 2009

Purpose: The objective of this study was to describe the frequency of hepatobiliary dysfunction (HD) at our hospital and determine the possible risk factors and complications associated with the development of HD in very low birth weight infants (VLBWI) treated with parenteral nutrition (PN). Methods: A retrospective study of VLBWI (n=92) that required PN between 2004 and 2008 in the NICU at the Bucheon St. Marys Hospital of Catholic University was performed. HD was defined by a direct bilirubin (DB) >2 mg and a transaminase of 60 IU/L defined cholestasis and liver injury. Groups I, II, and III were limited to cases of cholestasis, liver injury without cholestasis, and no abnormalities, respectively. The VLBWI were compared to each other. Results: Thirty-six subjects (39.1%) had cholestasis and 51 (55.4%) had liver injury. In addition, 36 (39.1%), 19 (20.7%), and 37 (40.2%) subjects were classified as groups I, II, and III, respectively. The three groups showed significant differences in gestational age, 1- and 5-minute Apgar scores, use of surfactant, duration of parenteral nutrition, frequency of RBC transfusions, bronchopulmonary dysplasia (BPD), and patent ductus arteriosus (PDA) (P<0.05). The multiple regression analysis with cholestasis as the dependent variable, showed a significant correlation with gestational age, use of surfactant, frequency of RBC transfusions, and PDA. Conclusion: Various factors, such as birth weight, gestational age, 1- and 5-minute Apgar scores, use of surfactant for respiratory distress syndrome (RDS), frequency of RBC transfusions, BPD, and PDA may be related to hepatobiliary dysfunction in VLBWI treated with PN.

• Neonatal Medicine
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• v.17 no.1
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• pp.94-101
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• 2010

Purpose:In recent years, Korea has showed a steady increase in the frequency of teenage birth, while the overall birth rate has declined. As the teenage birth is known as a high risk pregnancy itself, we examined perinatal complications of teenage mothers and whose neonates in aspects of medical problem, and social status and support. Methods:We examined the perinatal characteristics of teenage mothers and whose babies, who were hospitalized at Korea University Ansan Hospital from January 2004 to July 2009 using medical records retrospectively. Twenty-seven teenage mothers and their 28 babies were enrolled in this study. Results:Teenage mothers were all unmarried and showed high rates of preterm labor, maternal anemia, and unexpected delivery. Among them, 11 (40.7%) were from families that were separated. Eleven mothers (40.7%) did not have any antenatal care. There were high rates of prematurity and low birth weight (60.7% and 64.3%, respectively). The complication included: respiratory distress syndrome, patent ductus arteriosus and necrotizing enterocolitis. Fourteen babies (51.9%) were not going to be brought up by their biological parents. Conclusion:Teenage pregnancy had high rates of preterm labor and associated complications, often caused by the lack of proper antenatal care. Babies from unmarried mothers were likely to be adopted and this could be a social burden. Therefore, to reduce unplanned teenage pregnancy and births, sex education and social supports should be provided to all teenagers.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.380-391
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• 2010

Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. Methods : On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. Results : During the winter season, RSV caused 20-0% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. Conclusion : To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.