• Journal of Chest Surgery
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• v.29 no.3
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• pp.292-296
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• 1996

Total anomalous pulmonary venous return is a rare but serious cardiac malformation, accounting for only about 1.5~3% of congenital heart disease. Surgical results have been dramatically improved in the last two decades, largely owing to improved techniques of cardiopulmonary bypass and perloperative management. Seven patients ranging in age from 15 days to 11 years with total anomalous pulmonary venous return underwent repair between 1984 and 1995. The types of anomalous return were supracardiac in 5, and cardiac in 2. There were 5 boys and 2 girls. There were two hospital death, occurred in 15-day-old, and 40-day-old infants with supracardiac type. Follow-up periods have ranged from 3 months to 11 years, and all survivors have remained asymptomatic with normal growth and development.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.552-555
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• 2001

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1202-1206
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• 1996

Ebstein's anomaly is an unusual and a rare congenital cardiac malformation but characteristic abnormality of the development of the tricuspid valve, right atrium and ventricle. The characteristic findings are a displaced tricuspid valve with a septal leaflet spiralling into the right ventricle. The surgical correction is variable and are left unsolved problem in surgical treatment of anomaly. Between January 1988 ＆ December 1995, we experienced 11 cases of Ebstein's anomaly and associated cardiac anomalies are ASD, PFO, ASD with VSD. The typically displaced tricuspid valve leaflet was found in all cases. In the NYHA functional classification, three were in class II, seven were in class III, and one was in class IV. Nine patients were operated by tricuspid annuloplasty ＆ plication with Danielson's method and two patients were done by tricuspid valve replacement with plication, and in all cases associated anomaly was corrected. Postoperatively, five patients suffered from complication - low cardiac output syndrome, arrhythmia and wound infection. There was no operative mortality and the postoperative courses were relatively good conditions with more improvement of symptoms.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.99-103
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• 2013

Purpose: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. Materials and methods: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. Results: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. Conclusion: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.

• Journal of Chest Surgery
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• v.18 no.2
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• pp.288-292
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• 1985

The division of the right ventricle into two chambers by aberrant muscle bands traversing the sinus portion, Double Chambered Right Ventricle[DCRV], is a relatively rare congenital cardiac malformation. Double Outlet Right Ventricle[DORV], basically recognized by the origin of both great arteries from the morphologic right ventricle, is also a rare anomaly; its frequency has been reported as approximately 0.09 case per 1,000 birth. DORV associated with DCRV, unusual combination, is even more rare; only a few known cases have been recorded previously in the literature. This report presents our surgical experience with this rare anomaly, DORV with DCRV.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.34-39
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• 1983

The syndrome of anomalous muscle bundle dividing the right ventricle into two pressure chambers has been described by many authors. The malformation should not be confused with tetralogy of Fallot itself because the obstructive hypertrophic bands are usually proximal to the right ventricular infundibulum. One case [11 years old male] of double chambered right ventricle due to aberrant muscle bundle with intact ventricular septum is presented. The pressure gradient was 68 mmHg between inflow and outflow tracts of right ventricle on cardiac catheterization. On opening the right ventricle, there noted stenosis of outflow tract by infundibular membrane, hypertrophied anomalous muscle bundle, thickened moderator band & hypertrophied anterior papillary muscle. Open heart surgery was carried out with the aid of extracorporeal support, Anomalous muscle bundle [1.0 cm x 4.0 cm] and infundibular membrane were resected safely. The hypertrophied moderator band was cut at mid-portion and anterior papillary muscle was split vertically. The postoperative course was uneventful and discharged in good condition 2 weeks later.

• Journal of Chest Surgery
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• v.13 no.2
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• pp.105-109
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• 1980

Tetralogy of Fallot has shown its familial aggregation in several familial studies. This reported case is another example which occurred in a family in two brothers. They revealed no cytogenetic abnormalities, but the anatomical cardiac malformation of them showed much similarity, total conal defect in ventricular septum and .one had patent foramen ovale, the other atrial septal defect. The familial recurrence tendency of Tetralogy of Fallot as well as other congenital heart diseases could be explained on multifactorial inheritance as shown in many reports. In spite that we couldn`t find out any environmental trigger or teratogens, our case may be accepted on the base of multifactorial mechanism.

• Journal of Chest Surgery
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• v.21 no.3
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• pp.553-557
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• 1988

Anomalous origin of right coronary artery from pulmonary artery is a rare congenital cardiac anomaly. Anomalous right coronary artery often be a incidental finding without serious cardiovascular sequale. The only characteristic physical finding is a continuous murmur with diastolic accentuation. There are no diagnostic EKG or chest X-ray changes. Diagnosis is made best by selective left coronary arteriography showing retrograde filling of right coronary artery from collateral vessels. Here, we present a case of twenty-four months old aged girl with anomalous origin of right coronary artery combined with pulmonary stenosis. This is the first pediatric patient with anomalous right coronary artery and the first patient to have surgical correction for this malformation.

• Journal of Korean Physical Therapy Science
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• v.6 no.4
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• pp.153-165
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• 1999

The transcranial Doppler(TCD) is a technique for measuring blood flow velocity of intracranial and extarcranial arteries. This examination based on Doppler effect which was first formulated in 1842 by the Austrian physicist Christian Doppler. In 1982, Rune Aaslid first maked 2MHz pulsed probe and recording intracranial vessels with transcranially. There are six criteria utilized in gaining positive identification of the intracranial vessels. The six criteria are as follows l)acoustical windows 2)depth of sample volume 3)direction of flow 4)spatial relationship of ACA and MCA bifurcation 5)mean velocity and 6)response common carotid artery compression and/or oscillation test. The affected factors for TCD examination are angle of insonation, posture of subject, age, gender, hematocrit, metabolic factors, and cardiac output. Clinical application of TCD are detection of stenosis, occlusion, emboli, thrombsis in intracranial and extracranial arteries and evaluation of cerebral arterovenous malformation, collateral capacity in the circle of Willis, ischemia cerebrovascular disease, stroke patient and vertebrobasilar system.

• Journal of Chest Surgery
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• v.10 no.1
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• pp.113-117
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• 1977

The tetralogy of Fallot is one of the most frequent and serious congenital cardiac malformation accompanied cyanosis. For relief of cyanosis, the Waterston operation is a successful, palliative procedure in infant & young child under age of five with obstructive lesions of the right: side of the heart who require a systemic-pulmonary arterial shunt for survival. A patient, aged 3 and weighing 13 kg., who had been cyanotic since one month after birth,. was admitted to the University of Severance Hospital under a diagnosis of tetralogy of Fallot, The side to side anastomosis between the right pulmonary artery & the ascending aorta was performed in March 1976. The anastomotic channel was made only 4 ram. in diameter, thereafter massive unilateral pulmonary congestion on the side of the anastomosis developed shortly after operation. And the. patient died of congestive heart failure within a hour. And so the purpose of this report is describe the immediate & late effect of systemic-pulmonary shunt for T.O.F. with review of literatures.