• Journal of Chest Surgery
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• v.32 no.3
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• pp.303-306
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• 1999

This is a genuine case report of the Ross operation without the use of homografts or heterografts in reconstruction of the right ventricular outflow tract. A 8-year-old boy with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft and right ventricular outflow tract reconstruction with a pericardial conduit bearing autologous aortic monocusp. The postoperative echocardiography and cardiac angiography revealed good ventricular function and competent neoaortic valve. He has been followed up for 19 months.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.591-594
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• 1999

Supravalvular aortic stenosis is an uncommon, congenital narrowing of the ascending aorta which originates just distal to the level of the ostium of the coronary artery. We conducted a successful surgical treatment in a 39 year- old female patient with a congenital supravalvular aortic stenosis and aortic regurgitation who did not show signs of William's syndrome. After we performed an inverted Y-shaped aortotomy toward the noncoronary sinus and right coronary sinus, pantaloon shaped prosthetic patch(Vascutek, Ino, USA) was used to repair the narrowing sinotubular junction. The aortic valve was replaced concommittently using Sorin Bicarbon 19mm. Her postoperative course was uneventful. The patient discharged at 9th postoperative day in good health.

• Journal of Chest Surgery
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• v.33 no.9
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• pp.748-751
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• 2000

The diffuse form of supravalvar aortic stenosis represents a surgical challenge when the ascending aorta, aortic arch, proximal descending thoracic aorta and arch arteries are involved. It can be treated by a variety of surgical approaches. We report a case of severe diffuse supravalvar aortic stenosis combined with an aortic valve anomaly and occlusion of the right coronary artery ostium in a 14-year-old boy with Williams syndrome. We enlarged the aortic root(Nick's procedure), ascending aorta, aortic arch, proximal descending thoracic aorta, and innominate artery with patches and replaced aortic valve with 19 mm St. Jude valve. Deep hypothermic circulatory arrest and retrograde cerebral perfusion were used during repair of the arch and arch artery.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.361-363
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• 2009

A 17-year-old male patient was referred with symptoms of dyspnea. Multi-detector computerized tomography (MDCT) and echocardiography evaluation revealed quadricuspid aortic and pulmonary valves, an atrial septal defect (ASD), and pulmonary stenosis. We closed the ASD using a bovine patch and performed a commissurotomy of the pulmonary valve. Quadricuspid semilunar valves are very rare congenital abnormalities that are reported to occur nine times more frequently in the pulmonic valve than in the aortic valve. According to the Hurwitz Roberts classification, the aortic valve was type A, and the pulmonic valve was type B. The aortic valve normal function, but the pulmonic valve was stenotic and had abnormal function.

• Journal of Veterinary Clinics
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• v.26 no.2
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• pp.155-159
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• 2009

A 2-year-old female mixed dog(weighing 4.3 kg) was referred to the Veterinary Teaching Hospital, Kangwon National University, with primary complaints of exercise intolerance, nocturnal coughing and heart murmur. Diagnostic studies revealed bi-lateral holosystolic murmurs on phonocardiogram, over-riding aorta, pulmonic stenosis, ventricular septal defect, atrial septal defect, biventricular hypertrophy, and subaortic stenosis in diagnostic imaging studies. Based on the diagnostic findings, pentalogy of Fallot(POF) with subaortic stenosis(SAS) was tentatively diagnosed. This is the first case report of POF complicated with SAS in a dog in Korea.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.17-24
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• 2007

Background: The aim of this study is to assess the clinical results of various procedures in congenital aortic stenosis. Material and Method: From August 1987 to June 2004, 53 patients of congenital aortic stenosis underwent procedures such as percutaneous balloon valvuloplasty, aortic valvuloplasty, Ross procedure, and aortic valve replacement. The mean age of initial procedures was $8.2{\pm}6.0$ years. Percutaneous balloon valvuloplasty was peformed in sixteen patients, aortic valvulopiasty in thirty two patients, Ross procedure in nineteen patients, and aortic valve replacement in fourteen patients. The mean follow duration was $80.6{\pm}60\;(0{\sim}207)$ months. Result: There was 15.1% (8/53) of early mortality and no late mortality. The six patients with critical aortic stenosis were died of left ventricular dysfunction in early series (before 1 year; 4 cases) and two patients died after the Ross procedure and aortic valve replacement respectively. After percutaneous balloon valvuloplasty, most patients needed reoperations (14/16). Thirteen patients needed reoperation, after aortic valvuloplasty. After Ross procedure, two patients needed reoperation due to aortic regurgitation caused by progressive aortic root dilatation. The actuarial survival rate after Ross procedure at 7 years was 90.5%. Conclusion: In young children before the age of one, percutaneous balloon valvuloplasty was considered as :he safe initial palliative procedure. But children over one year-old, aortic valvuloplasty were the effective procedure. Ross procedure can be preformed safely with good results.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.88-91
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• 1997

Congenital long-segment tracheal stenosis with complex cardiac anomaly has generally been regarded as a fatal disease This report described the successful concomitant repair of unexpected congenital tracheal stenosis and complex cardiac anomaly with the use of edrdiopulmonary bypass. The patient was a 3-month-old girl with coarctation of aorta, V D, and PDA. The presence of tracheal stenosis was not discovered until when difficulty with endotracheal intubation was encountered at operating room. Thus, we decided concomitant repair of both lesions and performed anterior pericardial tracheoplasty combined with one stage repair of coarctation of aorta, VSD, and PDA under the cardiopulmonary bypass. The patient is doing well without any signs of complication at present, 2 years and 1 month after the operation.

• Journal of Chest Surgery
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• v.31 no.8
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• pp.763-769
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• 1998

Background: Supravalvular aortic stenosis is a rare form of congenital cardiac anomaly involving ascending aorta distal to coronary orifice. Materials and methods: We operated 12 cases of supravalvular aortic stenosis between July 1986 and March 1997. Age ranged from 4 to 17(mean 10.2) years and 11 of them were male. Nine patients had clinical features of Williams syndrome. We experienced two types of supravalvular aortic stenosis, including 10 hour glass type and 2 diffuse type. Results: Preoperative transaortic pressure gradient ranged from 40 to 180(mean 92) mmHg by cardiac catheterization. Pulmonary stenosis was associated in 5 and 2 of them required angioplasty. Operative techniques included 6 standard aortoplasty with elliptical patch, 4 extended aortoplasty with inverted Y shaped patch, and 2 modified Brom's repair. There were no operative deaths. Postoperative echocardiographic evaluation was done at a mean interval of 12 months. Grade I or II aortic regurgitation was found in 3 cases. Postoperative cardiac catheterization revealed a mean transaortic pressure gradient of 26 (range 0 to 75) mmHg. A mean pressure drop was 78(range 30 to 114) mmHg. All patients were followed up for a mean of 40(range 1 to 67) months with uneventful clinical course. Conclusions: Our data proved the low mortality and excellent hemodynamic improvement after surgical relief of supravalvular aortic stenosis in children.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.116-119
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• 2008

Transcatheter interventions are widely used for diagnosis and treatment of congenital heart disease. Complications associated with transcatheter interventions are uncommon. However, when they occur they are most often self-limited. Rarely, however, serious catheter related complications occur that may require emergent surgical intervention. In this case, the right common iliac artery was disrupted during transcatheter balloon valvuloplasty during the treatment of congenital aortic stenosis in a 2-week-old baby. After immediate surgical intervention with bleeding control using two balloon catheters the baby did well. Here we report this case and review the medical literature.

• 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.