• Journal of Chest Surgery
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• v.27 no.11
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• pp.893-901
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• 1994

Forty nine patients [M: 31, F: 18], age from 2 months to 17 years [mean= 4.9 years], underwent operations, from April 1986 to December 1992, for the relief of subvalvular aortic stenosis in normal atrioventricular and ventriculoarterial connections.There were 4 anatomic types of subaortic stenosis : membranous in 29 cases [59.2%], fibromuscular in 11 [22.4%], diffuse tunnel type in 7 [14.3%], and miscellaneous in 2 cases. Thirty four patients [69.4%] had associated cardiac anomalies, of which ventricular septal defect was the most common [27 cases]. Other anomalies were patent ductus arteriosus, coarctation of the aorta, valvular aortic stenosis, double chambered right ventricle [DCRV], infundibular pulmonic stenosis, persistent left superior vena cava, and rigt aortic arch. Mean systolic pressure gradient between the left ventricle and ascending aorta was 26.4$\pm$17.6 mmHg : 13.1$\pm$17.6mmHg in the membranous type, 22.0$\pm$18.4mmHg in the fibromucular type, and 56.1$\pm$38.4mmHg in the diffuse tunnel type. Operative procedures were determined according to the type of subvalvular aortic stenosis : simple excision of subaortic membrane in the membranous type [29 cases], left ventricular myectomy with or without myotomy or fibrous tissue excision in the fibromuscular type [11 cases]. Among the 7 of diffuse tunnel type cases, ventricular myectomy was performed in 2 and a modified Konno operation was performed in 5 . Postoperative follow up was made with periodic echocardiography. The Mean postoperative follow up period was 33.8 months. There were 2 hospital mortalities [4.1%] and 2 late deaths. Residual stenosis remained in 3 cases and recurrence developed in 2 cases during the follow up period. 5 years actuarial survival rate was 91.8$\pm$3.9% and 5 year complication free rate was 72.3$\pm$10.4%. Conclusions : 1. Subvalvular aortic stenosis should be relieved completely as soon as possible when diagnosed, regardless of left ventricular outflow tract pressure gradient. 2. Good results were obtained using only simple excision of subaortic membrane in the membranous type of subaortic stenosis. However, aortoventriculoplasty [modified Konno prodedure] was necessary for good results in the diffuse tunnel type. 3. Periodic postoperative echocardiography was helpful in detecting the progression of residual stenosis and development of new stenosis.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.796-799
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• 2004

A 52 day-old male infant who had Taussig-Bing anomaly with coarctation of the aorta underwent initial palliative Damus-Kaye-Stansel (DKS) procedure including arch reconstruction because of suspected intramural coronary artery, size discrepancy of great arteries, potential subaortic stenosis, refractory pneumonia, and severe congestive heart failure. Total repair was done 44 months later, which was composed of VSD patch closure, DKS take-down, and arterial switch procedure, We report a successful case of DKS take-down and arterial switch operation for the reuse of native aortic and pulmonary valves rather than Rastelli-type procedure in a patient with Taussig-Bing anomaly having palliative DKS procedure.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.172-174
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• 2005

Chylothorax is a rare but serious and well-recognized complication of thoracic and cardiac procedures. A postoperative chylothorax developed in a 3-month-old male patient after ligation of patent ductus arteriosus and repair of coarctation of aorta. He was treated successfully with conservative management using a combination of parenteral octreotide and medium-chain triglyceride (MCT) -enriched fomula with pleural drainage. We report a case of successful conservative treatment using octreotide for postoperative chylothorax.

• The Journal of the Korean life insurance medical association
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• v.29 no.2
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• pp.29-32
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• 2010

Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. MMD is one of cerebrovacular accident,which is treated with sugical maeuver in pediatic neurosurgery. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. The exact etiology of moyamoya disease is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with moyamoya disease, including the following: 1) Immunological - Graves disease/thyrotoxicosis 2) Infections - Leptospirosis and tuberculosis 3) Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus 4) Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease 5) Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, 6)cranial trauma, radiation injury, parasellar tumors, and hypertension etc. These associations may not necessarily be causative but do warrant consideration due to impact on treatment.(Mainly neurosurgical operation.) The incidence of moyamoya disease is highest in Japan. The prevalence of MMD is 1 person per 100,000 population. The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.4:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade(5-10yr) or in the third and fourth decades (30-40yr)of life. Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. In aspect of life insurance, MR is 1700%, EDR is 16 per 1000 persons. Children and adults with moyamoya disease (MMD) may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. Recently increasing diagnosis of MMD with MRI, followed by surgical operation is noted. MMD needs to be considered as the "CI" state now in life insurance fields.

• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Clinical and Experimental Pediatrics
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• v.61 no.6
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• pp.187-193
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• 2018

Purpose: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. Methods: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1-4 years). Medical records were reviewed retrospectively. Results: The minimum lumen diameter (MLD) in PAS and CoA increased from $5.0{\pm}1.9mm$ and $8.4{\pm}1.6mm$ to $10.1{\pm}3.6mm$ and $12.3{\pm}2.5mm$, respectively (P<0.01). In the PAS group, pressure gradient decreased from $25.7{\pm}15.6mmHg$ to $10.4{\pm}10.1mmHg$, and right ventricular to aortic pressure ratio from $0.56{\pm}0.21$ to $0.46{\pm}0.19$. In the CoA group, the pressure gradient decreased from $50{\pm}33mmHg$ to $17{\pm}8mmHg$. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%-70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after $29{\pm}12months$. Conclusion: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.278-284
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• 1996

Nine infants with transposition of great arteries have undergone arterial switch operation from May 1989 to May 1994 in the Department of Thoracic and Cardiovascular Surgery, Yeungnam University Hospital. Patients' age ranged from 3 days to 90 days, averaging 30$\pm$21 days. Diagnosis was made by two-dimensional echocardiography in all patients. Eight patients were diagnosed as transposition of great arteries with ventricular septal defect and one patient was a simple transposition of great arteries. Associated anomalies were patent ductus arteriosus (8), atrial septal defect (7) and coarctation of aorta(1). The anatomy of the coronary arteries were 7 (77 %) type A and 2 (23 %) type D according to the Yacoub classification. Pulmonary artery reconstruction was done according to Lecompte maneuver with tautologous pericardial patch in 8 patients. Overall operative mortality rate was 55% Left heart failure and pulmonary hypertensive crisis were the cause of death on postoperative 1~2 days in three patients, and two succumbed to death due to sepsis on postoperative 2~ 3 weeks. The mean follow-up period was mean 17 months. No patient had clinically significant postoperative aortic regurgitation and supravalvular pulmonary stenosis. The excessive use of inotropic support postoperatively was identified as a stastically significant risk factor following the arterial switch operation. But other variables such as low body weight, long cardiopulmonary bypass time, excessive hemodilution during cardiopulmonary bypass, hypothermia and volume loading were not significant risk factors.

• 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.

• 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.