• Journal of Chest Surgery
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• v.28 no.4
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• pp.340-345
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• 1995

Between 1983 and 1993, 250 patients over 16 years of age who had undergone a surgical correction of a congenital cardiovascular disease were reviewed. 222 patients were divided into acyanotic group and 28 patients were cyanotic group. The most common defects were atrial septal defect [96 patients and ventricular septal defect [95 patients . There were 128 patients in the third decade, 71 patients under 20 years of age, 40 patients in the fourth decade and 11 patients over 40 years of age. The male to female ratio was 1.05:1. Operative mortality was 6.8% [4.1% in the acyanotic group and 26.8% in the cyanotic group and the most common cause of death was low cardiac output syndrome.

• Journal of Chest Surgery
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• v.19 no.3
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• pp.528-533
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• 1986

Total anomalous pulmonary venous connection is relatively rare cyanotic congenital heart diaease, which represents 1-4% of all congenital cardiac defects. Generally in the majority cases, severe heart failure and cyanosis develops in the early infancy. Because of high mortality in the untreated infants and surgical risk, there are still many things to be improved. Two patients with total anomalous pulmonary venous connection are presented, which we recently experienced. The one was 10 year old female with supracardiac type drained through left innominate vein, and survived the operation and continuous to do well for 1 year. The other 5 year old female with mixed type (right pulmonary vein drained via coronary sinus and left pulmonary vein through left innominate vein) was operated successfully under hypothermia and extracorporeal circulation, and followed up for 6 months without problem. It was very rare case in the literature.

• Korean Journal of Bronchoesophagology
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• v.11 no.2 s.22
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• pp.32-35
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• 2005

Laryngotracheoesophageal clefts are rare congenital anmalies of the upper aerodigestive tract. They range in severity from small soft tissue defects in the interarytenoid region to complete clefts of the larynx, trachea, and esophagus. Minor clefts with mild symptoms can be managed conservatively. But, major clefts requires prompt surgical managemet fur the best result. We report a case of a 1 month old male with laryngotracheoesophageal cleft. This cleft was corrected by thoracotomy and anterior laryngofissure approach.

• Korean Journal of Veterinary Research
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• v.31 no.3
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• pp.235-240
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• 1991

The abnormal male Newhampshire of five months age with three hindlimbs was examined macroscopically and radiographically The external feature included two normal wings, two normal hindlimbs and an underdeveloped extra hindlimb, which was attached to the pelvic region. The extra hindlimb consisted of an underdeveloped femur like bone, tibiotarsus likebone, tarsometatarsus like bone and three rows of digits with claw. The femur like bone was fused with pelvic girdle like bone. The bones of extra hindlimb were fused into one except one row of digit, which was attached to the tibiotarsus like bone. Three ceca and one cloaca with two vents were also observed.

• Journal of Chest Surgery
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• v.28 no.7
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• pp.698-703
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• 1995

Dextrocardia means right-sided position of the heart in the chest irrespective of the cause. For the absolute diagnosis of the dextrocardia, the segmental analysis of heart is necessary. Once the segmental analysis of the dextrocardia is made, it is often relatively easy to identify the presence of any associated defects based on conventional methods including physical examination, EKG, echocardiography, and angiocardiography. Two cases of dextrocardia with congenital heart disease were treated surgically.A eleven - months old boy was operated under diagnosis of ASD, VSD, and bilateral SVC with mirror - image dextrocardia {I,L,I} by primary closure of ASD and VSD.A twenty-four months old girl was operated under diagnosis of ASD, VSD, and PS with corrected TGA {I,D,D} by primary closure of ASD, VSD and dilatation of pulmonary stenosis. Both of them were discharged healthily after operation.

• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.169-175
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• 2018

Thyroid dyshormonogenesis is characterized by impairment in one of the several stages of thyroid hormone synthesis and accounts for 10%-15% of congenital hypothyroidism (CH). Seven genes are known to be associated with thyroid dyshormonogenesis: SLC5A5 (NIS), SCL26A4 (PDS), TG, TPO, DUOX2, DUOXA2, and IYD (DHEAL1). Depending on the underlying mechanism, CH can be permanent or transient. Inheritance is usually autosomal recessive, but there are also cases of autosomal dominant inheritance. In this review, we describe the molecular basis, clinical presentation, and genetic diagnosis of CH due to thyroid dyshormonogenesis, with an emphasis on the benefits of targeted exome sequencing as an updated diagnostic approach.

• Journal of Chest Surgery
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• v.52 no.5
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• pp.368-371
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• 2019

A unicuspid aortic valve is a rare congenital malformation that frequently presents with valvular dysfunction and dilatation or aortic aneurysm, requiring combined aortic valve surgery and aortic repair. Some patients show severe valve calcification extending into the interventricular septum, possibly resulting in damage to the conduction system during debridement for valve replacement. We present a rare case of severe aortic stenosis with a unicommissural unicuspid aortic valve diagnosed by preoperative transesophageal echocardiography in a 36-year-old man. After composite graft replacement of the aortic valve, aortic root, and ascending aorta, a permanent pacemaker was placed because of postoperative complete heart block.

• Korean Journal of Veterinary Research
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• v.64 no.1
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• pp.5.1-5.6
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• 2024

A 6-month-old male Ragdoll cat presented with exercise intolerance. On physical examination, there was a grade 2/6 systolic murmur at the right apex. Diagnostic tests, including SpO₂ measurement, blood tests, radiography, echocardiography, contrast echocardiography, and electrocardiography, were performed. Severe right atrial dilation, tricuspid valve leaflets and orifice displacement, right ventricular atrialization, septal leaflet adherence, anterior leaflet tethering, and right atrioventricular junction dilation were noted on echocardiography, alongside a right-to-left atrial septal defect. Cor triatriatum dexter and left ventricular aneurysm were observed. We diagnosed this case as having Ebstein anomaly with rare congenital heart deformities; which is rare in cats.

• 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.14 no.1
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• pp.9-16
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• 1981

Twenty eight patients with conpnital heart disuse underwent lung biopsy to assets pulmonary obstructive vascular disease at cardiac surpry. Thirteen patients had patent ductus arteriosus, 10, ventricular septal defects and S, atrial septal defects. The aaes were between 2 and 30 years. In patients with patent ductus arteriosus lung biopsy was performed from the IIngular Hlment. The anterior seament of the right upper lobe was blopsled in cases with ventricular septal defect and atrial septal defect. Grading of pulmonary obstructive vascular disease could not be assessed In 9 cases. In 2 cases poor quality of the slides made us impossible to evaluate and In 7 cases there were no suitable small muscular arteries to evaluate in the slides of lung tissue especially taken from the IIngular seament. Nineteen cases were evaluated pulmonary obstructive vascular disease. Among them 17 cases had Heath-Edwards changes of grade 1 and 2 patients had that of grade 3. The thickness of media was measured. It was expressed as percentage of medial thickness to outer diameter of artery. The medial thickness was correlated proportionally with elevation of pulmonary arterial pressure and pulmonary vascular resistance to systemic vascular resistance ratio. There were no complications related to the procedure of lung biopsy.