• Journal of Chest Surgery
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• v.21 no.3
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• pp.497-509
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• 1988

Current therapy of aortic dissections remains unstandardized because of the relative rarity of these catastrophic events and conflicting reported results of various therapeutic strategies. Hence, we reviewed our current results and planned to purify our method of interpretation of results and so, to standardize therapeutic managements. This study comprised unselected, consecutive 27 patients with aortic dissections who were operated at Seoul National University Hospital from Jan 1983 to March 1988. The results from analysis of their preoperative, operative and postoperative finding were as follows: 1] 7 patients had acute type A, 14 had chronic type A, 4 had acute type B, and 2 had chronic type B. 2] The causes of dissections were unclear, but 8 patients had Marfan`s syndromes, 2 had previous operative histories on cardiovascular systems and 2 had congenital heart diseases. 3] Multiple preoperative variables were found to correlate significantly with operative mortality and complications. The prevalences of such preoperative major complicating factors were significantly more frequent in acute than chronic [P < 0.05] and type A than type B [P < 0.01]. 4] Operations were performed according to the type of the dissections and whether it was acute or chronic. Usually dacron tube graft replacements were performed[25/26]. Intraluminal sutureless graft replacement was performed in 11 patients. Of the 14 patients with combined aortic regurgitation, concomitant aortic valve resuspension in 4, seperative aortic valve replacement in 1, and aortic valve replacement with coronary reimplantation were performed in 9 patients. 2 patients had concomitant arch vessel managements. 5] Over-all operative mortality rate was 33% and 54% for acute type A, 25% for acute type B, 29% for chronic type A, 0% for chronic type B respectively. The main causes of operative mortality were cardiovascular complications [mainly CPB-weaning failure] in acute cases and hemorrhagic complications in chronic cases.

• Journal of Chest Surgery
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• v.49 no.3
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• pp.151-156
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• 2016

Background: Survival of children experiencing cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest. Methods: Patients who were <18 years and underwent ECPR between November 2013 and January 2016 were including in this study. We retrospectively investigated patient medical records. Results: Twelve children, median age 6.6 months (range, 1 day to 11.7 years), required ECPR. patients' diseases spanned several categories: congenital heart disease (n=5), myocarditis (n=2), respiratory failure (n=2), septic shock (n=1), trauma (n=1), and post-cardiotomy arrest (n=1). Cannulation sites included the neck (n=8), chest (n=3), and neck to chest conversion (n=1). Median duration of extracorporeal membrane oxygenation was five days (range, 0 to 14 days). Extracorporeal membrane oxygenation was successfully discontinued in 10 (83.3%) patients. Nine patients (75%) survived more than seven days after support discontinuation and four patients (33.3%) survived and were discharged. Causes of death included ischemic brain injury (n=4), sepsis (n=3), and gastrointestinal bleeding (n=1). Conclusion: ECPR plays a valuable role in children experiencing refractory cardiac arrest. The weaning rate is acceptable; however, survival is related to other organ dysfunction and the severity of ischemic brain injury. ECPR prior to the emergence of end-organ injury and prevention of neurologic injury might enhance survival.

• Journal of Chest Surgery
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• v.25 no.11
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• pp.1327-1336
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• 1992

Between Jan. 1986 and Aug. 1992. 72 patients underwent Rastelli procedure. There were 43 male and 29 female, aged 46 days to 16 years [mean age, 5.2 years] with 18 patients less than 2 years of age. All patients had complex defect, 27 pulmonary atresia with ventricular septal defect, 18 corrected transposition of great arteries with pulmonary atresia or punmonary stenosis, 10 truncus arteriosus, 10 double outlet right ventricle with pulmonary atresia or stenosis, 7 complete transposition of great artersia with pulmonary atresia or pulmonary stenosis. The types of extracardiac valved conduit used were prosthetic valve[n=47, 24 car-bomedics, 19 Ionescu-Shiley, 4 Bjork-shiley] and hand-made trileaflet valve using pericardium. [n=23, 20 bovine pericardium, Z autologous pericardium, 1 equine pericardium] The mean size of valved cinduit was 5.25mm larger in diameter than the size of main pulmonary artery. [normalized to the patient`s body surface area] There were 17 hospital death[24%] and 4 late deaths[5.6%]. Postoperative complication rate was 38.9%a, none of which was conduit-related. All patients were followed pos-toperatively for 1 to 73 months. [mean 25.8 months] During follow-up period, reoperation was done in 6 patients due to stenosis of valved conduit. Mean interval between intial repair and reoperation was 20.3 months. In our experience, li recently extracardaic valved conduits between right ventricle [or pulmonary ventricle] and pulmonary artery were inserted with increasing frequency in infants less than 2 year, but hospital mortality was decreased, 2] Risk of reoperation due to conduit stenosis is low, so that the effect of graft failure on overall survival is minimized. 3] Nevertheless, because any type of extracardaic valved conduit is not ideal in children, we recommended that Lecompte should be done if cardiac anatomy is permitted.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.89-93
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• 2014

Background: This study was conducted to investigate the epidemiological features, clinical courses, and diagnoses of neonates who are transferred to neonatal intensive care unit of Yeungnam University Hospital due to tachypnea soon after birth. Methods: Based on medical records, we performed a retrospective study of neonatal intensive care unit admissions due to tachypnea from January 2010 to December 2013. Results: A total of 311 neonates were included in this study. The patient characteristics showed male predominance at 2.65:1. Among the 311 neonates with tachypnea, 127 (40.8%) neonates needed oxygen supply, and 54 (17.4%) neonates needed assisted mechanical ventilation. Transient tachypnea of the newborns (TTN) (158, 50.8%) showed the highest incidence, followed by pneumonia (63, 20.3%), extrapulmonary infection (37, 11.9%), respiratory distress syndrome (21, 6.8%), air leak (16, 5.1%), meconium aspiration syndrome (12, 3.9%), congenital heart disease (5, 1.6%), metabolic acidosis (3, 1%), primary pulmonary hypertension of newborns (2, 0.6%) and anemia (2, 0.6%). Conclusion: Although the neonates with tachypnea showed no other respiratory distress symptom, clinicians should be aware of the possibility of other pulmonary diseases as well as TTN and their extra-pulmonary causes. If tachypnea does not improve within a few hours, the clinician should consider further evaluation and management as soon as possible.

• Journal of Chest Surgery
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• v.57 no.3
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• pp.231-239
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• 2024

Background: Although the modified Blalock-Taussig shunt remains the mainstay method of palliation for augmenting pulmonary blood flow in various congenital heart diseases, the shunt must be carefully designed to achieve the best outcomes. This study investigated the effect of shunt configuration on pulmonary artery growth and growth discrepancy. Methods: Twenty patients with successful modified Blalock-Taussig shunt takedown were analyzed. Pulmonary artery and shunt characteristics were obtained using computed tomography scans. Differences in the baseline and follow-up diameter ratios and growth in the ipsilateral and contralateral arteries were calculated. The angle between the shunt and pulmonary artery, as well as the distance from the main pulmonary artery bifurcation, were measured. Correlations between pulmonary arteries and shunt configurations were analyzed. Results: The median interval time between shunt placement and takedown was 154.5 days (interquartile range, 113.25-276.25 days). Follow-up values of the ipsilateral-to-contralateral pulmonary artery diameter ratio showed no significant correlation with the shunt angle (ρ=0.429, p=0.126) or distance (ρ=0.110, p=0.645). The shunt angle and distance from the main pulmonary bifurcation showed no significant correlation (ρ=-0.373, p=0.189). Pulmonary artery growth was negatively correlated with shunt angle (ipsilateral, ρ=-0.565 and p=0.035; contralateral, ρ=-0.578 and p=0.030), but not with distance (ipsilateral, ρ=-0.065 and p=0.786; contralateral, ρ=-0.130 and p=0.586). Conclusion: Shunt configuration had no significant effect on growth imbalance. The angle and distance of the shunt showed no significant correlation with each other. A more vertical shunt was associated with significant pulmonary artery growth. We suggest a more vertical graft design for improved pulmonary artery growth.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.846-856
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• 2005

Purpose : To measure the peak myocardial tissue velocities and patterns of longitudinal motion of atrioventricular(AV) annuli and assess body weight and heart rates-related changes in normal children. Methods : Using pulsed wave Tissue Doppler Imaging(TDI), we measured peak systolic, early and late diastolic myocardial velocities in 72 normal children at six different sites in apical-4 chamber (A4C) view and at four different sites in apical-2 chamber(A2C) view and compared those values with each other, also observing effects with body weights and heart rates. Longitudinal motions of the AV annuli were measured at three different sites in A4C. Results : There were no significant differences of the TDI parameters between gender, ECHO-machines and among the three Doctors performing TDI. Peak myocardial velocities were significantly higher at the base of the heart than in the mid-ventricular region and in the right lateral ventricular wall than in the left lateral ventricular wall or IVS. The TDI parameters showed no significant correlation with fractional shortening(%). Peak systolic and early diastolic myocardial velocities had no correlation with heart rates, but peak late diastolic velocities and A/E ratio correlated positively with heart rates. Correlations between the TDI parameters and body weight were inconsistent. Absolute longitudinal displacement and % displacement were not differ between gender and not correlated with the TDI parameters. Conclusion : We measured the peak myocardial velocities with TDI and the longitudinal motion of the AV annuli using M-mode echocardiography in normal children. With more large scale evaluation, we may establish reference values in normal children and broaden clinical applicabilities in congenital and acquired heart diseases.

• Journal of Chest Surgery
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• v.11 no.4
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• pp.404-415
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• 1978

The present study was carried out to develop the better measures for safety of open heart surgery under extracorporeal circulation (ECC) with Heart-Lung-Machine by preventing changes in the concentrations of serum electrolytes during and after ECC. For this purpose, the cocentrations of serum electrolytes were measured before, during, and after ECC in 21 patients with congenital and acquired heart diseases who received open heart surger, - under ECC using Heart-Lung-Machine. Also considered was the development of safety measured by which changes in serum electrolyte concentrations were prevented during and after open heart surgery under ECC. The mean values for serum sodium levels were observed to be ; $13.14{\pm}0.47$mEq./L. for the samples obtained before ECC. $139.59{\pm}0.68$mEq./L. for the samples obtained 10 minutes after ECC and $138.0{\pm}0.68$mEq./L. for the samples obt"ined 24 hours after ECC. These results indicate that serum sodium concentrations were \\'ithin normal range during and until 24 hours after ECC. 2) The concentrations of serum chloride were found to be $105.38{\pm}0.70$105.38$\pm$0. 70 mEq./L. for the samples collected before ECC, $105.07{\pm}1.01$mEq./L. for the Simples collected 24 minutes aiter ECC and $101.95{\pm}1.09$mEq./L. for the samples collectect 24 hours afte ECC. As was tile case with serum sodium levels, no significant changes were observed in serum chloride levels during and 24 hours after ECC. 3)With proper provisions of potassium chloride solution during ECC, the concentrations of serum potassium were found to be $4.22{\pm}0.06$mEq./L. for the samples removed before EeC, $4.06{\pm}0.14$mEq./L. for the samples removed 10 minutes after ECC and $4.39{\pm}0.07$ mEq./L. for the samples removed 24 hours after ECC. 4)The concentrations of serum calcium were also maintained within normal during and after ECC; $9.15{\pm}0.14$mg/dl for the serum collected before ECC, $8.36{\pm}0.21$mg/dI for the serum collected 10 minutes after ECC and $8.47{\pm}0.14$mg/dl 21 hours after ECC. The maintenance of serum calcium level within normal throughout ECC was achieved by parenteral administrations of calcium gluconate as frequent as required. 5) As were the cases with serum potassium and calcium, the concentrations of plasma bicarbonate was regulated within normal range during and after ECC, only when sodium bicarbonate solution was admini"tered parenterally as it was required; $23.7{\pm}0.50$mEq./L. for the serum collected before ECC. $22.33{\pm}1.09$mEq.lL. for the serum collected 10 minutes after ECC and $25.3{\pm}0.96$mEq./L. for the serum collected 24 hours after ECC. The above results indicate tha t during and after ECC serum sodium and chloride levels remined unchanged without any provision of normal saline, while serum potassium, calcium, and bicarbonate concentrations were kept within normal limits only when these ealectrolytes were administered through parenteral routes. With these results it can be concluded that serum potassium, calcium, and bicarbonate levels should be determined as often as possible during and after ECC and that in order to maintain serum electrolyte levels within normal these electrolytes in the forms of potassium chloride, calcium gluconate, and sodium bicarbonate shou'd be given parenterally as they were found to be required.

• Journal of the korean academy of Pediatric Dentistry
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• v.45 no.1
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• pp.115-122
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• 2018

Noonan syndrome is characterized by distinctive facial features, short stature, and congenital heart disease. It is a congenital genetic disorder with a prevalence of between 1/1,000 and 1/2,500 in both genders. An 11-year-old boy with Noonan syndrome visited the hospital with an ectopically positioned tooth. A pulmonary stenosis was diagnosed and his growth and development were delayed. In many cases of this diseases there is obvious hemostasis, which he was not experiencing. His facial appearance showed characteristic features of Noonan syndrome. The patient showed a dental class II relationship, labioversion of the upper anterior teeth, and a shallow overbite. Radiographic examination revealed that the upper right canine was ectopically positioned, which led to root resorption of the upper right lateral incisor. A lateral cephalometric radiograph revealed a craniofacial pattern that was within normal limits. Surgical opening and button attachment on the impacted upper right canine were performed and traction was applied on the impacted tooth using a removable appliance. This patient was mildly affected by Noonan syndrome and showed some dental problems. However, few studies have reported the oral characteristics of Noonan syndrome despite its high incidence. Thus, this case report describes the oral features and management of Noonan syndrome.

• Journal of Genetic Medicine
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• v.7 no.1
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• pp.37-44
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• 2010

Purpose : Kabuki syndrome is a multiple congenital malformation syndrome with mental retardation. It was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. To date, six Korean cases of Kabuki syndrome have ever been reported. Here, we present the phenotypic and genetic characteristics of six patients with Kabuki syndrome. Materials and Methods : Between 2003 and 2009, six Korean girls have been diagnosed and followed up as Kabuki syndrome at Center for Genetic Diseases of Ajou University Hospital. Their clinical and laboratory data were collected and analyzed by the retrospective review of medical records. Results : All six patients showed the characteristic facial dysmorphism and developmental delay. Persistent fingertip pads were also found in all patients. Most patients showed postnatal growth retardation (83.3%) and hypotonia (83.3%). Opthalmologic problems were common, particularly for strabismus (83.3%). Congenital heart defects were present in three patients (50%). Skeletal abnormalities including 5th finger shortening (83.3%), clinodactyly (50%), joint hypermobility (50%) and hip dislocation (16.7%) were also observed. There was no patient who had positive family history for Kabuki syndrome. Cytogenetic and molecular cytogenetic analyses including karyotyping and array CGH could not reveal any underlying genetic cause of Kabuki syndrome. Conclusion : Korean patients with Kabuki syndrome showed a broad spectrum of clinical features affecting multiple organ systems. Although clinical manifestations of Kabuki syndrome have been well established, our results failed to detect recurrent chromosome aberrations which could cause Kabuki syndrome. Its natural history and genetic background remains to be further studied for providing appropriate management and genetic counseling.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.490-494
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• 2003

Purpose : We'd like to determine the incidence of congenital heart disease and arrhythmia in elementary school children in Busan, and to provide adequate prevention and treatment. Methods : A total of 23,802(male 12,909, female 10,893) 1st grade elementary school children living in Busan were studied. All children were 7-8 years old. We obtained their medical history by questionnaire and checked elecrocardiography(ECG). Subsequent screening tests including a 2nd ECG, chest X-ray, phonocardiogram and CBC for the students who had abnormal findings at the first screening test. The third screening test was done for students who had cardiac murmurs or abnormal ECG findings in the second screening test by echocardiogram, treadmill test and 24-hour Holter monitoring. Results : Among 23,802 children participants, 605(2.54%) had abnormal ECG findings at the first screening test. Q wave abnormality(0.58%) was observed most frequently, and complete right bundle branch block(RBBB)(0.26%), sinus tachycardia(0.24%), right axis deviation(0.22%) and ventricular premature contraction(VPC)(0.21%) followed in order. Four hundred and twenty four children participated in the second ECG screening test. Two hundred and two children(47.6%) had an abnormality such as sinus tachycardia(18.8%), VPC(17.8%), or complete RBBB(17.3%). After completing the third examination including echocardiogram, we couldn't find the students with ventricular tachycardia (VT) or SVT at the third arrhythmia screening test. Conclusion : A high incidence of arrhythmia was found in the 1st grade elementary school children in Busan despite their healthy appearances, although fatal heart diseases were not noted. Early diagnosis, adequate preventative measures and treatment will prevent and decrease the frequency of emergent situations like syncope and sudden death.