• 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.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.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.23 no.6
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• pp.1146-1151
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• 1990

Supravavular aortic stenosis is a congenital narrowing of the ascending aorta just distal to the level of the origins of the coronary arteries, that may be localized or diffuse. Five patients with supravalvular aortic stenosis were operated upon between July, 1986 arid June, 1990. Four of these patients were William`s syndrome [mental retardation, elfin face], and one was isolated supravalvular aortic stenosis. Preoperative diagnosis of the supravalvular aortic stenosis was made by left side cardiac catheterization and angiocardiography. There are three types of supravalvular aortic stenosis such as membranous, hourglass and hypoplastic. Four of our patients were of hourglass type, and one was hypoplastic type. Patch aortoplasty was performed in all cases. Preoperative systolic gradients ranged from 45 to 1SO mmHg [average 102.6 mmHg]: postoperative gradients ranged from 0 to 75 mmHg [average 39 mmHg]. The patient of hypoplastic type has been suffered from mild exercise intolerance even after the operation, and the postoperative echocardiography revealed the systolic gradient of 100 mmHg [preoperative 180 mmHg]. The results of surgery for hourglass type were excellent. But the patient with hypoplastic form would be benefited from some modifications of the operation.

• Journal of Chest Surgery
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• v.32 no.2
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• pp.108-118
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• 1999

Background: To evaluate the risk factor and long-term result of arterial switch operation , a retrospective study was done. Material and Method: A retrospective analysis was done to evaluate the early and long-term results on 58 patients who underwent an arterial switch operation(ASO) for transposition of the great arteries(TGA) with intact ventricular septum, between January 1988 and December 1996. Beforesurgery, 36 patients(62.1%) underwent balloon atrial septostomy, 32 patients(51.7%) received PGE1 infusion, and preparatory banding of pulmonary artery was performed on 6 patients(mean LV/RV pressure ratio 0.53$\pm$0.11). Result: The age at operation ranged from 1 to 137 days(mean 24$\pm$26 days) and the weights ranged from 1.8 to 6.8 kg (mean 3.5$\pm$0.8 kg). There were 14 early deaths(24.1%), but of the last 24 patients operated on since 1994, there were only 2 early deaths(8.3%). In the risk factor analysis, the date of operation was the only risk factor for early death(p-value < 0.01). Eight of the 14 early deaths were due to acute myocardial failure(mainly inadequate coronary blood flow). The length of follow-up ranged from 2 months to 8 years, average of 36$\pm$27 months. The follow-up included sequential noninvasive evaluations and 21 catheterizations and angiographic studies performed 5 to 32 months postoperatively with particular attention to the great vessel and coronary anastomosis, ventricular function, valvular competence, and cardiac rhythm. There were 5 late deaths(11.4%), one of thesedeaths was related to the late coronary problems, two to aspiration, one to uncontrolled chronic mediastinitis, and one to progressive aortic insufficiency and heart failure. The most frequent postoperative hemodynamic abnormality was supravalvular stenosis and the degree of pulmonary or aortic obstruction had slowly progressed in some cases, however there were no children who had to undergo a reoperation for supravalvular pulmonary or aortic stenosis. Aortic regurgitation was identified in 9 patients, which was mild in 7 and moderate in 2 and had progressed in some cases. Two patients who had an unremarkable perioperative course were identified as having coronary artery obstructions. The other late survivors were in good condition, were in sinus rhythm, and had normal LV functions. Actuarial survival rate at 8 years was 68.8%. Conclusion: We concluded that anatomic correction will be established as the optimal approach to the TGA with intact ventricular septum, though further long-term evaluations are needed.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.292-298
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• 2009

Background: Surgery for mitral valve disease in children carries both technical and clinical difficulties that are due to both the wide spectrum of morphologic abnormalities and the high incidence of associated cardiac anomalies. The purpose of this study is to assess the outcome of mitral valve surgery for treating congenital mitral regurgitation in children. Material and Method: From 1997 to 2007, 22 children (mean age: 5.4 years) who had congenital mitral regurgitation underwent mitral valve repair. The median age of the patients was 5.4 years old and four patients (18%) were under 12 months of age. 15 patients (68%) had cardiac anomalies. There were 13 cases of ventricular septal defect, 1 case of atrial septal defect and 1 case of supravalvar aortic stenosis. The grade of the preoperative mitral valve regurgitation was II in 4 patients, III in 15 patients and IV in 3. The regurgitation was due to leaflet prolapse in 12 patients, annular dilatation in 4 patients and restrictive leaflet motion in 5 patients. The preoperative MV Z-value and the regurgitation grade were compared with those obtained at follow-up. Result: MV repair was possible in all the patients. 19 patients required reduction annuloplasty and 18 patients required valvuloplasty that included shortening of the chordae, papillary muscle splitting, artificial chordae insertion and cleft closure. There were no early or late deaths. The mitral valve regurgitation after surgery was improved in all patients (absent=10, grade I=5, II=5, III=2). MV repair resulted in reduction of the mitral valve Z-value ($2.2{\pm}2.1$ vs. $0.7{\pm}2.3$, respectively, p<0.01). During the mid-term follow-up period of 3.68 years, reoperation was done in three patients (one with repair and two with replacement) and three patients showed mild progression of their mitral reguration. Conclusion: our experience indicates that mitral valve repair in children with congenital mitral valve regurgitation is an effective and reliable surgical method with a low reoperation rate. A good postoperative outcome can be obtained by preoperatively recognizing the intrinsic mitral valve pathophysiology detected on echocardiography and with the well-designed, aggressive application of the various reconstruction techniques.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.862-868
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• 1997

Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.