• Journal of Chest Surgery
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• v.32 no.2
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• pp.124-129
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• 1999

Background: Ventricular septal defect(VSD) that causes pulmonary hypertension increase right ventricular workload. Echocardiographic assessment of right ventricular systolic time interval (RVSTI) has been used to predict pulmonary artery pressure in various cardiopulmonary diseases. This study was undertaken in infants with simple VSD to observe the alteration of the right ventricular workload through the changes of RVSTI after repair of VSD. Material and Method: We evaluated heart rate, the ratio of the left atrium/aortic root diameter (LA/Ao), right ventricular pre-ejection period(RVPEP), right ventricular ejection time(RVET), and its ratio(RVPEP/RVET) as a predictor of right ventricular workload in 12 children with simple VSD. These were measured three times at the preoperative period, at the 3 month and between 6 month and 1 year(average 9.5${\pm}$1.8month) after repair of VSD by M-mode & Doppler echocardiograph from the pulmonic valve echogram. Result: Heart rate was decreased significantly after repair(137.1${\pm}$13.7 vs 114.4${\pm}$21.1 and 104.1${\pm}$10.2, p<0.01). LA/Ao ratio was decreased significantly after repair(1.71${\pm}$0.32 vs 1.47${\pm}$0.33 and 1.39${\pm}$0.23, p<0.05). RVPEP/RVET were decreased after repair (0.38${\pm}$0.09 vs 0.32${\pm}$0.08 and 0.29${\pm}$0.09, p<0.01). Heart rate corrected RVPEP/RVET were significantly decreased only after 6 months(0.32${\pm}$0.03 vs 0.30${\pm}$0.05 and 0.28${\pm}$0.06, p<0.05). Conclusion: We found elevated right ventricular workload was progressively decreased until more than 6 months after repair and the RVSTI may serve a useful guide in postoperative care for children with VSD.

• Korean Journal of Cleft Lip And Palate
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• v.3 no.1
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• pp.23-31
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• 2000

Objective: When an alveolar cleft is too large to close with adjacent mucobuccal flaps or large secondary fistula following a primary bilateral palatoplasty exists, a one-stage procedure for bone grafting becomes challenging. In such a case, we used the tongue flap to repair the fistula and cleft alveolus in the first stage, and bone grafting to the cleft defect was performed in the second stage several months later. The purpose of this paper is to report our experiences with the use of an anteriorly-based Y-shaped tongue flap to fit the palatal and labial alveolar defects and the ultimate result of the bone graft. Patients: A series of 14 patients underwent surgery of this type from January 1994 to December 1998.The average age of the patients was 15.8 years old (range: 5 to 28 years old). The mean period of follow-up following the 2nd stage bone raft operation was 45.9 months (range: 9 to 68 months). In nine of the 14 cases, the long-fork type of a Yshaped tongue flap was used for extended coverage of the labial side alveolar defects with the palatal fistula in the remaining cases the short-forked design was used. Results: All cases demonstrated a good clinical result after the initial repair of cleft alveolus and palatal fistula. There was no fistula recurrence, although Partial necrosis of distal margin in long-forked tongue flap was occurred in one case. Furthermore, the bone graft, which was performed an average of 8 months after the tongue flap repair, was always successful. Occasionally, the transferred tongue tissue was bulging and interfering with the hygienic care of nearby teeth; however, these problems were able to be solved with proper contour-pasty performed afterwards. No donor site complications such as sensory disturbance, change in taste, limitations in tongue movement, normal speech impairments or tongue disfigurement were encountered. Conclusion: This two-stage reconstruction of a bilateral cleft alveolus using a Y-shaped tongue flap and iliac bone graft was very successful. It may be indicated for a bilateral cleft alveolus patient where the direct closure of the cleft defect with adjacent tissue or the buccal flap is not easy due to scarred fibrotic mucosa and/or accompanied residual palatal fistula.

• Journal of Periodontal and Implant Science
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• v.34 no.1
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• pp.223-241
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• 2004

The purpose of this present study evaluated the osseous response around Ca-P coated xenogenic bone and compared osteogenic potential of Ca-P coated xenogenic bone to that of combination with type I collagen derived from bovine tendon as a biocompatible binder to prevent migration of bone particle on the repair of calvarial defects in rabbits. To study the effects of Ca-P coated xenogenic bone and collagen on bone healing, four 5-mm-diameter skull defect were made in calvaria with trephine filled with an autogenous bone chip or Ca-P coated xenogenic bone or Ca-P coated xenogenic bone and type I collagen (1:1 mixture by volume) or left empty. The defects were evaluated histologically at 1, 2, 4 and 8 weeks following implantation. Ca-P coated xenogenic bone at the calvarial defects of rabbits showed osteoconductivity at the margin of defect in the early stage of bony healing, but no direct contact with new bone was observed. With time passed by, it was resorbed slowly and showed consistent inflammatory reaction. An additional use of type I collagen derived from bovine tendon improved clinical handling, but no new bone formation was observed histologically. Above all, autogenous bone graft showed most prominent healing in quantity and density of new bone formation. According to this study, the use of Ca-P coated xenogenic bone alone and combination with type I collagen did not showed effective healing in quantity and density of new bone formation.

• The Journal of Korean Academy of Prosthodontics
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• v.50 no.3
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• pp.169-175
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• 2012

Purpose: In this study, brazing characteristics of $ZrO_2$ and Ti-6Al-4V brazed joints with increasing temperature were investigated. Materials and methods: The sample size of the $ZrO_2$ was $3mm{\times}3mm{\times}3mm$ (thickness), and Ti-6Al-4V was $10mm(diameter){\times}5mm(thickness)$. The filler metal consisted of Ag-Cu-Sn-Ti was prepared in powder form. The brazing sample was heated in a vacuum furnace under $5{\times}10^{-6}$ torr atmosphere, while the brazing temperature was changed from 700 to $800^{\circ}C$ for 30 min. Results: The experimental results shows that brazed joint of $ZrO_2$ and Ti-6Al-4V occurred at $700-800^{\circ}C$. Brazed joint consisted of Ag-rich matrix and Cu-rich phase. A Cu-Ti intermetallic compounds and a Ti-Sn-Cu-Ag alloy were produced along the Ti-6Al-4V bonded interface. Thickness of the reacted layer along the Ti-6Al-4V bonded interface was increased with brazing temperature. Defect ratios of $ZrO_2$ and Ti-6Al-4V bonded interfaces decreased with brazing temperature. Conclusion: Thickness and defect ratio of brazed joints were decreased with increasing temperature. Zirconia was not wetting with filler metal, because the reaction between $ZrO_2$ and Ti did not occur enough.

• Journal of Genetic Medicine
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• v.6 no.1
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• pp.95-99
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• 2009

Prenatal diagnosis of rare autosome mosaicism involvingchromosomes other than chromosome 13, 18, 21 or the sex chromosome is encountered prognostic dilemma during genetic counseling. We report four cases of level III uncommon mosaicism of trisomy 5, 16 and 20,diagnosed prenatally. In case 1 with mosaic trisomy 20, there was a higher mosaic ratio of trisomy 20 in the repeat amniocentesis (62.1%) than in the first (36.6%) with normal fetal ultrasound finding except for a relatively small aorta on a 3-vessel view of the fetal heart. Case 2 showed a low rate of mosaic trisomy 20 (5.25%) in cultured amniocytes but normal karyotype in the repeat amniocentesis, who delivered a normal healthy baby. Case 3 showed a 13.6% of trisomy 16 mosaicism in the 30 cells of cultured amniocytes. Sixty cells from a fetal blood sample at termination showed non-mosaic 46,XX normal karyotype, while skin fibroblasts had 22.5% trisomy 16 in 40 metaphases. The autopsy showed ventricular septal defect (VSD). Case 4 with low grade mosaicism (10.5%) of trisomy 5 resulted in elective termination, though the ultrasoumd showed growsly normal fetus. Although level III mosaicism is regarded as true mosaicism, it is difficult to predict the outcome of the fetus with rare mosaic autosome trisomy. Therefore mosaic autosome trisomy of fetus should be carefully interpreted with more various approaches including repeat sampling and targeted fetal ultrasound.

• Childhood Kidney Diseases
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• v.15 no.2
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• pp.163-171
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• 2011

Purpose : To evaluate the prevalence of vesicoureteral reflux (VUR) according to the timing of voiding cystourethrography (VCUG) in infantile urinary tract infection (UTI). Methods : The data of 134 infants (1-12 months) with renal cortical defect in $^{99m}Tc$-2, 3-dimercaptosuccinic acid ($^{99m}Tc$-DMSA) scan with a diagnosis of UTI in two hospitals from 2000 to 2010 were retrospectively analyzed. The VCUG was performed after 2 weeks from the diagnosis of UTI in Group I (n=68), and the VCUG was performed within 2 weeks from the diagnosis of UTI in Group II (n=66). Results : There were no significant differences between the two groups in the duration of fever, white blood cell count, C-reactive protein levels, and abnormalities in ultrasonography (P>0.05). There was no significant difference between the two groups in the prevelence of VUR, bilateral VUR, and severe VUR. VCUG-induced UTI was detected 16 (23.5%) of patients in whom the procedure was performed 2 weeks after the diagnosis, and none of VCUG-induced UTI occurred in those in whom the procedure was performed 2 weeks within the diagnosis. Conclusion : We conclude that the prevalence of VUR according to the timing of VCUG did not differ between the two groups in infantile UTI with renal cortical defect in DMSA scan. We also found that performing VCUG with antibiotics can decrease risk of VCUG-induced UTI.

• 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.32 no.4
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• pp.368-372
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• 1999

Background: Although there have been few reports about minimal skin incision for the repair of congenital heart lesions, minimizing an unsightly scar is a particularly important factor in growing children. We have adopted a technique that permits standard full sternotomy, conventional open chest cardiopulmonary bypass, aortic cross-clamping, left atrial vent, and antegrade cardioplegia with minimal surgical scar. Material and Method: With minimal skin incision and full sternotomy, 40 patients with congenital heart disease underwent open heart surgery from April 1997 through September 1997. Defects repaired included 30 ventricular septal defects, 4 atrial septal defects, and 1 sinus Valsalva aneurysm in 35 children(M:F=17: 18), and 3 Atrial septal defects, 1 ventricular septal defect, and 1 partial atrioventricular septal defect in 5 adults(M:F=1:4). Midline skin incision was performed from the second intercostal space to 1 or 2 cm above the xiphoid process. For full sternotomy, we used the ordinary sternal saw in sternal body, and a special saw in manubrium under the skin flap. During sternal retraction, surgical field was obtained by using two retractors in a crossed direction. Result: The proportion of the skin incision length to the sternal length was 63.1${\pm}$3.9%(5.2∼11cm, mean 7.3cm) in children, and 55.0${\pm}$3.5%(10∼13.5cm, mean 12cm) in adults. In every case, the aortic and venous cannulations could be done through the sternal incision without additional femoral cannulation. There was no hospital death, wound infection, skin necrosis, hematoma formation, or bleeding complication. Conclusion: We conclude that minimal skin incision with full sternotomy can be a safe and effective alternative method for the repair of congenital heart diseases in children and adults.

• Journal of Chest Surgery
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• v.35 no.7
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• pp.511-516
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• 2002

Background: This study was undertaken in infant patients with isolated ventricular septal defect(VSD) to determine the effect of surgical closure on ventricular systolic time interval, as a parameter for ventricular performance, by echocardiography. Material and Method: Thirty patients were enrolled. Mean age of patients at operation was 6.5$\pm$3.2 months and all patients had non-restrictive VSD. We checked the left atrium/aorta(LA/Ao) ratio, left ventricle ejection fraction(EF), left ventricular systolic time interval(LVSTI), and right ventricular systolic time interval(RVSTI). Echocardiographic studies were done before surgical correction and postoperative periods(postopl: within 2 weeks, postop2: between 4 and 6 months, postop3: between 1 and 2 years). Result: LA/Ao ratio decreased significantly at immediate postoperative period compared to preoperative period and sustained during further follow-up period(from 1.74$\pm$0.37 to 1.36$\pm$0.24＊, 1.32$\pm$0.22＊, and 1.27$\pm$0.19＊, p<0.01). LV EF had not changed during follow-up periods(from 65.1$\pm$7.0 to 62.3$\pm$9.5, 62.8$\pm$5.7, and 64.1$\pm$6.9). LVSTI decreased significantly at postop2 and sustained during further follow-up period (from 0.46$\pm$0.13 to 0.46$\pm$0.11, 0.37$\pm$0.08＊, and 0.34$\pm$0.07＊, p<0.01). RVSTI decreased significantly at postop3(0.33$\pm$0.08 to 0.32$\pm$0.08, 0.31$\pm$0.07, and 0.27$\pm$0.05＊, p<0.01). Conclusion: We found that right and left ventricular systolic time intervals had decreased over the period of 1 year after surgical correction of VSD. Therefore, it is necessary to observe the change of ventricular function during that period.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.434-439
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• 2006

Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.