• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1216-1220
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• 2009

Purpose:To analyze and compare various cases in which peritoneal drainage was used as the primary treatment method in preterm infants with intestinal perforation. Methods:Among the preterm infants of less than 28 weeks of gestation who were admitted to the neonatal intensive care unit (NICU) at the Gangnam Severance Hospital from April 2006 to April 2009, 7 who had developed intestinal perforation were studied retrospectively. We investigated the clinical characteristics, secondary operation performances, morbidities, complications, and mortalities. Results:Among the 7 infants, 5 survived. Of the 5 cases, 3 received laparotomy, of which 2 were confirmed as having necrotizing enterocolitis. Of the 2 infants who died, 1 had received laparotomy before 48 h of peritoneal drainage, while the other had not received any subsequent treatment. Of the 7 children, 4 had patent ductus arteriosus (PDA), of which 3 had received indomethacin injection. Five infants had begun enteral feeding before they developed intestinal perforation. Of the 5 infants who survived, 4 were diagnosed with cholestasis. Of the 7 infants, 4 developed periventricular leukomalacia (PVL) and 3 developed rickets. Conclusion:Although the use of peritoneal drainage as the primary management of intestinal perforation in preterm infants is controversial, we suggest that it can be used for treating extreme premature neonates. Further randomized controlled study will be required to determine the feasibility of using this method.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.277-283
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• 2005

Arterial oxygen saturation $(SaO_2)$ instability frequently takes place after systemic-pulmonary shunt without shunt occlusion. We analyzed actual incidence and risk factors for $SaO_2$ instability after shunt operations, and possible mechanisms were speculated on. Material and Method: Ninety three patients, who underwent modified Blalock-Taussig shunt from January 1996 to December 2000, were enrolled in this study. Adequacy of shunt was verified in all patients, either by ensuing one ventricle or biventricular repair later on or by appropriate pulmonary artery growth on postoperative angiogram. Age, body weight, hemoglobin level at operation were 3 day to 36 years (median: 1.8 months), 2.5kg to 51kg (median: 4.1kg) and $10.7\~24.3$ gm/dL (median: 15.2 gm/dL) respectively. Preoperative diagnoses were functional single ventricle with pulmonary stenosis or atresia in 39, tetralogy of Fallot in 38 and pulmonary atresia with intact ventricular septum in 16. Pulmonary blood flow (PBF) was maintained pre-operatively by patent ductus or previous shunt in 64 and by forward flow through stenotic right ventricular outflow tract (RVOT) in 29. $SaO_2$ instability was defined as $SaO_2$ less than $50\%$ for more than 1 hour with neither anatomic obstruction of shunt nor respiratory problem. Result: 10 patients $(10.7\%)$ showed $SaO_2$ instability after shunt operation. After shunt occlusion was ruled out by echocardiogram, they received measures to lower pulmonary vascular resistance (PVR), which worked within a few hours in all patients. Risk factors for $SaO_2$ instability included older age at operation (p=0.039), lower preoperative $SaO_2$ (p=0.0001) and emergency operation (p=0.001). PBF through stenotic RVOT showed marginal statistical significance (p=0.065). Conclusion: $SaO_2$ instability occurs frequently after shunt operation, especially in patients with severe hypoxia pre-operatively or unstable clinical condition necessitating emergency operation. Temporary elevation of pulmonary vascular resistance is a possible mechanism in this specific clinical setting.

• Journal of Chest Surgery
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• v.42 no.4
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• pp.456-463
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• 2009

Background: Although the reports on re-operative coronary revascularization (redo-CABG) have increased, there are only limited reports on redo-CABG using arterial grafts. The aim of this study was to analyze the safety and feasibility of using various arterial grafts for redo-CABG. Material and Method: A consecutive series of patients who underwent 33 redo-CABGs from March 2001 to July 2008 were retrospectively reviewed. We performed conventional CABG in 17 patients, on-pump beating CABG in 7, off-pump CABG in 7 and minimally invasive direct coronary artery bypass in 2. The grafted that were used included 34 internal thoracic arteries (ITA), 14 radial arteries, 14 right gastroepiploic arteries and others. Arterial composite grafts were constructed in 26 patients. Of these, a previously patent in-situ left ITA was re-used as the in-flow of a composite graft in 10 patients. Result: No hospital deaths or major wound problems occurred. The post-operative complications included 2 myocardial infarctions (6%), 1 intra-aortic balloon pump insertion (3%), 5 cases of atrial fibrillation (15.1 %) and 3 neurologic complications (9.1%). The meanfollow-up duration was 31.1$\pm$22.7 months and the 3 year survival rate was 86.4%. There were 4 late deaths (2 cardiac deaths) and no recurrent angina during the follow-up period. Conclusion: Redo-CABG with using various arterial grafts is currently a safe, feasible procedure, but further investigation and long term follow-up are needed.

• Journal of Life Science
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• v.31 no.1
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• pp.17-27
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• 2021

This study was performed to investigate the antioxidant activities of subfractions of Peucedanum insolens Kitagawa root in various organic solvents and their anti-inflammatory effects on LPS-treated RAW264.7 cells. First, P. insolens Kitagawa roots were dried at room temperature for one week, chopped, and extracted with 70% ethanol. The resulting extracts were successively sub-fractionated with hexane, chloroform, ethyl acetate, and water. The antioxidant potential of the fractions was evaluated using a DPPH (1,1-diphenyl-2-picrylhydrazyl) radical scavenging assay and by measuring total polyphenol and flavonoid contents. The anti-inflammatory potency of the fractions was evaluated by measuring the inhibition levels of the expressions of inflammatory-mediated genes and proteins (e.g., iNOS, COX-2, IL-1β, and IL-6) in RAW264.7 cells. The results clearly showed that the ethyl acetate fraction of the P. insolens Kitagawa root contained relatively high total flavonoid (34.08±1.68 ㎍ of quercetin equivalents per mg) and total polyphenol (154.1±3.2 ㎍ of gallic acid equivalents per mg) contents. The DPPH assay results showed that the P. insolens Kitagawa root possessed strong free radical scavenging activity in the ethyl acetate fraction. Both the ethyl acetate and hexane fractions showed strong inhibitory potencies to nitric oxide production induced by lipopolysaccharide (1 ㎍/ml) treatment for 24 hr in RAW264.7 cells. The results also showed that both the hexane and ethyl acetate fractions of the P. insolens Kitagawa root strongly inhibited mRNA levels of iNOS, IL-1β, and IL-6, which were overexpressed by LPS treatment for 24 hr in the RAW264.7 cells. These results suggest that P. insolens Kitagawa root may contain compounds that possess strong potency for anti-inflammatory activity. Further studies are needed to discover more detailed modes of action of P. insolens Kitagawa root fractions against inflammation modulation, such as the regulation of cytokine signaling and inflammatory signaling pathways.

• Journal of Chest Surgery
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• v.44 no.6
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• pp.392-398
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• 2011

Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Journal of Chest Surgery
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• v.35 no.11
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• pp.779-784
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• 2002

Recently, the number of coronary artery bypass surgery(CABG) is increasing according to the increasing incidence of coronary artery disease. However, CABG is not a definite corrective surgery; therefore, in some patients, redo-CABG may be required. We retrospectively reviewed our redo-CABG experiences to help future redo-CABG. Material and Method: From January 1991 to April 2001, 14 cases of redo-CABG were performed in Yonsei Cardiovascular Center(M:F＝12:2) and mean age was 61,7 $\pm$ 7.1(47-72) years. Mean time from 1st. CABG to redo-CABG was 121.9 $\pm$ 50.5(6.1-179.6) months. Thirteen cases were conventional on-pump CABG and one case was off-pump CABG. In two patients, mitral valve re-replacement and mitral valve repair were performed each. All redo-CABG were performed through mid-sternotomy. During redo-CABG, left internal mammary artery and saphenous vein grafts were used in 6 patients, left internal mammary artery and left radial artery grafts were used in 2 patients, left internal mammary artery and gastroepiploic artery were used in one patient and only greater saphenous veins were used in 5 cases(In one case, cephalic vein was also used). The number of mean distal anastomosis was 2.1 $\pm$ 0.9(1-4). Result: There were no operative death and no perioperative myocardial infarctions and cerebrovascular accidents or other heart related complications. Mean follow up duration was 40.1 $\pm$ 38.6(1.1-118.5) months. During follow up period, angina was re-developed in one patient 13 months after operation. Two patients died of end-stage renal failure 14.8 months and 116.3 months after redo-CABG, respectively. During follow up period, coronary angiography was performed in 3 patients, and all grafts were patent. At last follow up, mean Canadian class was 1.3. Kaplan-Meier survival at 9 years was 90.0 $\pm$ 9.5% and event free survival at 9 years was 71.4 $\pm$ 6.9%. Conclusion: After redo-CABG, all patients improved their angina symptom and daily activity. And long-term survival after redo-CABG was excellent. Therefore, if patients have indications for redo-CABG, thenredo-CABG must be strongly recommended and performed.

• Clinical and Experimental Pediatrics
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• v.53 no.2
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• pp.203-209
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• 2010

Purpose : Growth impairment is usually observed in children with congenital heart disease (CHD). Studies on the final height and weight of this population are insufficient. Our aim was to evaluate the final height of children with CHD and to identify the relationship between CHD and growth. Methods : We recorded the values of final height and weights of 105 CHD patients (age, <19 years) who visited Chonnam National University Hospital between November 2000 and March 2009, and we reviewed the medical records. Results : The mean values of weight and height of male CHD patients were significantly lower than those of the normal subjects. Patients with severe growth impairment (below the third percentile of normal) included 5 males (8.6%) and 4 females (8.5%) with height less than normal and 9 males (15.5%) and 8 females (17.0%) with weight less than normal. The mean growth of the patients in the cyanotic heart disease group was lower than that of normal subjects, but a statistically significant difference was noted in the weights of males. In a comparative study based on the type of CHD, a significant difference was noted in weights of males. Patients with patent ductus arteriosus and those with tetralogy of Fallot had the highest and lowest mean values of weight, respectively. Conclusion : Growth impairment was more evident in children with CHD than normal children. Patients should be treated during an optimal time frame. Thus, CHD patients should be treated if follow-up studies indicate growth impairment.

• 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.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.182-187
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• 2002

Background: There have been many reports of coronary angiographic findings after coronary bypass grafting, most of which are focused on the graft patency rate of the bypass conduits. However, postoperative angiography can provide numerous informations other than patency rates that are useful for establishing operative strategy. Material and Method: We studied 73 patients in whom coronary angiography was done after more than 1 month of CABG. Mean interval from the operation to coronary angiography was 10.6 months and the reasons for coronary angiography follow up were residual or recurrent angina in 54 patients, abnormalities on myocardial perfusion scan or echocardiography in 13 patients, and for simple follow up in 6 patients. Result: Overall graft patency rate was 80.9% (internal thoracic artery 100%, saphenous vein 75.0%) in patients of simple follow up and 61.6%(internal thoracic artery 81.1%, saphenous vein 55.3%) in patients with ischemia. Progression of native coronary arterial disease proximal to the grafting site was found in 50 patients(68.5%). Among 201 coronary arterial branches that had not been completely occluded preoperatively, ninty five branches(47.3%) revealed progression of diameter stenosis by more than 20% on the follow up study. Among them, 64 branches(31.8%) progressed to total occlusion. The incidence of disease progression was highter in the coronary arteries with patent grafts(57.5%) than in those with occluded grafts(36.3%)(p<0.05), Comparing internal thoracic artery graft with saphenous vein graft, internal thoracic artery was superior to saphenous vein, not only in terms of patency(83.3% vs 56.6%), but also in terms of result of later percutaneous intervention success rate(100% vs 62%, p<0.05). Conclusion: Due to the considerable incidence of progression of native coronary artery stenosis in the early postoperative periods, bypass grafting of a vessel with borderline stenosis, especially with vein graft, must be done prudently. And it was confirmed again that revascularization of left anterior descending artery is most important and that internal thoracic artery was superior to saphenous vein.