• Journal of Chest Surgery
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• v.22 no.2
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.83-87
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• 1997

Taussig-Bing anomaly is infrequently associated with interrupted aortic arch and size discrepancy of great arteries makes it difficult to undergo arch reconstruction and arterial switch operation. A 20-day old male infant was admitted with the diagnosis of Taussig-Bing anomaly with type B Interrupted aortic arch. Multi-organ failure, due to the diminution of ductal flow, was stabilized after 3 weeks of prostaglandin El and controlled ventilatory support. The surgical correction consisted of VSD closure, arterial swtich and extended aortic arch reconstruction. The marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery was overcome by constructing distal neoaorta using both native ascending and descending aortic tissue. The patient was extubated on postoperative 2nd day Postoperative catheterization showed no left ventricular outflow obstruction, no intracardiac shunt, and no incompetence of neoaortic valve.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.651-658
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• 2004

Runx2 is a transcription factor in homologous with Drosophila runt gene and it is essential for bone formation during embryogenesis and a critical gene for osteoblast differentiation and osteoblast function. Runx2-haploinsufficency causes cleidocranial dysplasia (CCD). CCD is an autosomal-dominant inherited disorder characterized by hypoplastic clevicle and delayed ossification in fontanelles and wormian bones. Dental defects are possibly shown to CCD patients : multiple supernumerary teeth, irregular and compressed permanent tooth crowns, hypoplastic and hypomineralized defects in enamel and dentin, an excess of epithelial root remnants, the absence of cellular cementum, and abnormally shaped roots. In addition, delayed eruption of the secondary dentition is a constant finding. The aim of this study is to evaluate the role of Runx2 in the tooth development and eruption through analyzing the expression pattern of Runx2 by in situ hybridization during crown (late bell stage) and root formation of tooth, using postnatal day 1, 4, 7, 14 and 21 mice mandibular molar teeth. mRNA of Runx2-full length is expressed in dental follicle and surrounding tissue at postnatal day1 and 4. At postnatal day 7, it is expressed in ameloblasts of occlusal surface of enamel and bone area surrounding the tooth. In comparison with previous stage, at postnatal day 14, it is expressed in ameloblasts of proximal surface of enamel. At postnatal day 21 it's expression is observed only in bone area. mRNA of Runx2-typeII is not expressed. At postnatal day 1 and 7. At postnatal day 14 and 21, it's expression is observed in the bone area. In this study, we suggest that Runx2 have a relation of ameloblasts differentiation and an important role to tooth eruption made by dental follicle during intraosseous eruption stage. Also we can confirm that Runx2 has a role to bone formation.

• International Journal of Oral Biology
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• v.34 no.1
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• pp.21-28
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• 2009

Mutations in DLX3 are associated with both autosomal dominant hypoplastic hypomaturation amelogenesis imperfecta (ADHHAI) and tricho-dento-osseous (TDO) syndrome. ADHHAI is caused by a c.561_562delCT (2bp-del DLX3) mutation whereas TDO syndrome is associated with a c.571_574delGGGG (4bp-del DLX3) mutation. However, although the causal relationships between DLX3 and an enamel phenotype have been established, the pathophysiological role of DLX3 mutations in enamel development has not yet been clarified. In our current study, we prepared expression vectors for wild type and deletion mutant DLX3 products (4bp-del DLX3, 2bp-del DLX3) and examined the effects of their overexpression on the expression of the enamel matrix proteins and proteases. Wild type DLX3 enhanced the expression of matrix metalloprotease 20 (MMP20) mRNA and protein in murine ameloblast-like cells. However, neither a 4bp-del nor 2bp-del DLX3 increased MMP20 expression. Wild type DLX3, but not the above DLX3 mutants, also increased the activity of reporters containing 1.5 kb or 0.5 kb of the MMP20 promoter. An examination of protein stability showed that the half-life of wild type DLX3 protein was less than 12 h whilst that of both deletion mutants was longer than 24 h. Endogenous Dlx3 was also found to be continuously expressed during ameloblast differentiation. Since inactivating mutations in the gene encoding MMP20 are associated with amelogenesis imperfecta, the inability of 4bp-del or 2bp-del DLX3 to induce MMP20 expression suggests a possible involvement of such mutations in the enamel phenotype associated with TDO syndrome or ADHHAI.

• Journal of Chest Surgery
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• v.24 no.7
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• pp.674-684
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• 1991

The surgical treatment of tetralogy of Fallot [TOF] was initiated by Blalock and Taussig in 1945 with the establishment of the subclavian artery to pulmonary artery anastomosis. In an imaginative and daring effort, in 1954, Lillehei and collaborators [1955] using controlled cross-circulation, carried out the first intracardiac repair of TOF by closing the ventricular septal defect [VSD] and relieving the pulmonary stenosis under direct vision. Nowadays, total correction is the ideal operation for treatment of TOF and is accomplished with extracorporeal circulation. And the results of surgery for TOF have steadily improved over the years, thanks to important contributions of many surgeons. Nevertheless because of its protean physiologic and anatomic presentation, TOF continues to offer challenges to cardiologist and cardiac surgeons. Thirty two cases of TOF have undergone total corrective surgery using extracorporeal circulation in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, Inje University, from Oct. 1985 to Feb. 1990. Clinical considerations were applied to these cases and the results were obtained as follows. 1. The heart lung machine used for extracorporeal circulation was SarnsO 7000, 5-head roller pump, and the number and type of oxygenators were 10 of bubble type and 22 of membrane type. The mean bypass time was 148.9 minutes and the mean aortic cross clamp time was 123.8 minutes. The GIK [glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 2. 20 cases were male and 12 were female, the mean age was 8 years old and the mean body weight was 25Kg. 3. The preoperative symptoms were cyanosis [29 cases], squatting [27 cases] and etc. The mean values of preoperative Hb., Hct., and SaO2 were 16.5 gm /dl, 50.3%, and 78.5%. 4. Combined anomalies were noticed in 16 cases [50%]. Among them 10 cases were PFO and 6 cases were ASD. 5. The degree of aorta overriding were 25% in 5 cases, 25 ~ 50% in 22 cases and above 50% in 5 cases. The dPA/Ao [ratio of diameter of pulmonary artery trunk to ascending aorta] were below 25% in 5 cases, 25 ~ 50% in 10 cases, 50 ~ 70% in 6 cases and above 75% in 11 cases. 6. The types of RVOT [right ventricular outflow tract] stenosis were valvular and infundibular in 14 cases [43.6%], diffuse hypoplastic type in 12 cases [37.5%], infundibular in 5 cases, and valvular and supravalvular in 1 case. 7. One stage radical corrective surgery was applied to the all cases. In widening of the RVOT, 3 types of patches were used: MVOP [monocusp ventricular outflow patch, Polystan BioprosthesesO] in 3 cases, knitted Dacron vessel patches in 2 cases, and double layer with bovine pericardium and woven Dacron prosthesis in 26 cases. 8. Postoperative complications were occurred in 15 cases. Among them, low output syndrome were occurred in 10 cases [31.3%] and 2 of them were expired postoperatively.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.4
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• pp.704-709
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• 1998

Amelogenesis imperfecta represents a group of hereditary conditions that manifest enamel defects without evidence of generalized or systemic disorders. These enamel disorders are apparently heterogeneous in the basic chemical structure, resulting in a diverse presentation of clinical characteristics. The reported prevalence of amelogenesis imperfecta varies from 1 in 14,000-16,000 to 1.4 in 1,000 depending on specific population studied with the autosomal dominant hypocalcification type of amelogenesis imperfecta believed to be the least prevalent. The most widely accepted current classification system for delineating the amelogenesis imperfecta types considers the mode of inheritance and clinical manifestations. Three major groups are recognized; hypoplastic, hypocalcified, and hypomaturation types. Delineating specific types of amelogenesis imperfecta can be confusing due to the phenotypical similarity of many forms and that the most recent classification lists 14 different types. A 12 year-old female patient came to our pediatric dentistry clinic complaining of the ugly shape and color of her teeth, especially the upper front area. Although the goal of the treatment was mainly focused on the improvement of patient's esthetics, longevity of the restorations was also considered in selecting the appropriate restorative system, resin jacket crown, which can satisfy the both aspects.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.207-214
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• 1979

This 3-year-old girl was observed frequent exertional dyspnea and cyanosis at crying since birth. She was not premature baby and delivered at full term normally. On physical examination, she was underdeveloped-body weight 13.5 kg, height 99 cm.- and cyanotic. There was severe clubbing on fingers. There was grade II/VI ejection systolic murmur on left lateral border of the sternum. The preoperative examinations [EKG, echocardiogram, cardiac catheterization and biventriculogram] showed that complicated T.G.A. combined vena cava[S.D.D.]. Preoperatively, we decided the corrective surgery of Rastelli operation using a. pulmonary valved conduit. The operation was performed under total circulatory arrest using deep profound hypothermia combining with extracorporeal circulation. On operation, the anatomy of the heart showed that, 1. The subaortic conus was seen and subaortic muscles were hypertrophied. 2. The VSD[type II], behind the subaortic conus-about 1 cm. in diameter, was visible only through LV cavity and, 3. The pulmonary valve ring was hypoplastic and pulmonary valvular stenosis was seen also. The subpulmonic area [LV outflow tract] was obstructed with hypertrophied muscle and mitral valve. 4. Left superior vena cava was drained to RA via coronary sinus. 5. LAD coronary artery was originated from right coronary artery and ran anterior to the pulmonary artery. According to above anatomy, we performed the VSD closure with Teflon patch, and Mustard operation combined with LV-to-pulmonary artery bypass graft using the valve contained [Hancock 16 mm] conduit. Postoperatively, adequate blood pressure could be maintained under the state of using inotropic agent [epinephrine]. On the second postoperative day, the patient died of cardiac arrest due to low cardiac output syndrome, acute renal failure and pulmonary edema.

• Journal of Chest Surgery
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• v.23 no.3
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• pp.474-481
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• 1990

We have experienced 44 cases of coarctation of aorta in the age of infancy and children from April 1986 to September 1989 at Seoul National University Children`s hospital. Patients were thirty males and fourteen females, and their age ranged from one month to ten years[mean 23.84 $\pm$33.06 months] with thirty-two infant cases. In the infantile age, congestive heart failure was the most common chief complaint[18/32], and above that age, frequent upper respiratory infection was most common[8/12]. We experienced thirteen cases of isolated COA, twenty-two cases of COA with VSD, eight cases of COA with VSD, eight cases of COA with intracardiac complex anomalies and one case of COA with atrial septal defect. The associated intracardiac complex anomalies were three Taussig-Bing type double outlet right ventricle, one single ventricle, one transposition of great arteries, one atrioventricular septal defect, one hypoplastic aortic arch with left heart hypoplasia, and one Tetralogy of Fallot. Operative techniques of COA were twenty-three subclavian flap arterioplasty, 12 resection and end to end anastomosis, eight onlay patch angioplasty, and I direct angioplasty after resection of web. Among the cases with other cardiac anomalies, staged operation was done in twenty-nine patients, and single stage total correction was performed only in three patients. There were seven operative mortality[15.9%], all being in infantile age group, and among fourteen cases associated with large VSD[Qp/Qs>2.0, mean pulmonary arterial pressure>50mmHg], four patients were died, but there was no mortality in patients with small VSD. With above results, we are intended to discuss about the interval between staged operation, the fate of VSD after coarctoplasty in case of COA with VSD, causes of death, complications etc.

• Journal of Chest Surgery
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• v.55 no.2
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• pp.151-157
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• 2022

Background: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). Methods: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. Results: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). Conclusion: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

• The Journal of Korean Academy of Prosthodontics
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• v.52 no.4
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• pp.317-323
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• 2014

Dentinogenesis Imperfecta, with a high incidence rate of 1 : 6 - 8000, is inherited by autosomal dominant genetic transmission. This dental disorder causes discoloration of the teeth and the enamel and dentin show hypoplastic or hypocalcified defects which lead to frequent fractures and rapid attrition. Therefore, timely treatment is necessary for the preservation of the remaining teeth. In this particular case, a 19-year-old patient suffering from Type 1 dentinogenesis imperfecta showed signs of brownish hued teeth with multiple fractures, a loss of vertical dimension, excessive interdental space in the maxillary anterior teeth, and a lack of 5 posterior teeth. To improve the esthetic appearance of the anterior teeth, the vertical dimension was increased. Resin caps were used to alleviate the difficulty of taking an impression of multiple teeth at once. Monolithic zirconia materials used in this case showed high fracture strength and the ability to mask the discoloration of the teeth and therefore, functionally and esthetically favorable results were achieved.