• 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.

• Journal of Chest Surgery
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• v.23 no.4
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• pp.764-768
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• 1990

Congenitally corrected transposition of great arteries is a congenital cardiac anomaly with ventriculoarterial discordant connection and atrioventricular discordant connection. A 8-year-old girl had congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary valvular stenosis. By transaortic approach, ventricular septal defect was closed and pulmonary valvotomy was performed by transpulmonary approach. No heart block or aortic insufficiency developed postoperatively.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.569-573
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• 2007

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The post-operative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1069-1076
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• 1997

There remains controversy regarding the appropriate surgical treatment for coarctation of the aorta because of relatively high rate of recoartation and high mortality in the cases associated with complex anomalies. We evaluated 31 consecutive patients who underwent surgical repair of coarctation of the aorta from May 1992 through June 1996. Nineteen patients(61.3%) were neonates and 26(83.9%) were under three months. Nine patients did not have major associated anom lies(Group I), 15 patients had ventricular septal defect(Group II), and 7 patients had major complex anomalies(Group III). 35.5% of the patients had arch hypoplasia. Surgical procedures performed were as follows: extended end-to-end anastomosis in 17 patients, combined resection-flap procedure in 7 patients, and subclavian flap aortoplasty in 7 patients. Residual coarctation occurred in 7(25%) of 28 patients; 2 after subclavian (lap aortoplasty(2/6, 33.3%), none after combillrd resection-flap procedure(0/7, 0%), and 5 after extended end-to-end anastomosis(5/15, 33.3%). Higher incidence of residual coarctation was noticed in the group with arch hypoplasia. The incidence of postoperative coarctation at a mean follow-up of 20.5 months in survivals was 12.0%(3/25); 2 cases after subclavian flap aortoplasty(2/6, 33.3%), none after combined resection-flap procedure(017, 0%), and one after endtoend anastomosis(1/12, 8.3%). The mortality rate related to coarctation repair was 9.7%(3 patients all in Group III). This study revealed that isolated coarctation of aorta and coarctation with ventricular septal di3fect(groups I & ll) can be repaired with low mortality, but repair of coarctation with complex anomaly had a high operative mortality Also the patients with arch hypoplasia had higher incidence of post-operative residual coarctation.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.305-310
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• 2009

Background: The Ross procedure is known as a good surgical option for a young age group with aortic valve problems, but few reports on the Ross procedure are available in the Korean literature. This study is a review of our midterm results of 10 year experience with the pediatric Ross operation in Asan Medical Center. Material and Method: From March 1997 to October 2008, eighteen patients who were aged less than 16 years underwent the Ross procedure. There were 11 males and 7 females. The patients median age was 8.5 years (range: $0.5\sim14.0$). The aortic valve pathophysiology was 6 patients with aortic insufficiency, 4 patients with aortic stenosis, 7 patients with mixed aortic stenoinsufficiencey and 1 patient with infective endocarditis. The valve morphology was bicuspid in 11 and tricuspid in 7. All the patients were operated on with the root replacement technique. All the pumonic valves were replaced with an allograft except for one pericardial monocusp valve. The mean follow up duration was 52.8 months (range: 5.8$\sim$138.2 months). We reviewed the echocardiographic data with focusing on the, auto-graft dysfunction and reoperation. Result: There was no hospital mortality and late mortality. According to the last echocardiographic data, 2 autografts showed aortic regurgitation grade 2, 4 autografts showed aortic regurgitation grade 1 and the others were less than trivial. Reoperation of the pulmonic position conduit was performed 4 times in three patients. The rate of freedom from reoperation at 5 years was 72.2%. On the serial follow up, the Z-values of the aortic annulus/aortic sinus were changed from $1.6{\pm}1.7/0.9{\pm}1.7$ at preoperation to $1.8{\pm}1.6$(p=0.64)/$2.2{\pm}0.9$ (p=0.01) at the last follow-up. There was no significant relation between the growth of the neoaortic root and neoaortic insufficiency. Conclusion: Our midterm results of the Ross procedure in pediatric patients showed good autograft function and growth potential. Vet reoperation due to allograft dysfunction was a major concern.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.680-686
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• 1989

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origin of the coronary arteries. It may occur in several different anatomical form. Three type have been described; the hourglass, the hypoplastic and the membranous type, each term identifying the gross characteristic of the lesion causing by the aortic obstruction. Non cardiovascular condition commonly associated with supravalvular aortic stenosis are mental retardation, facial anomalies, hypercalcemia, etc. The diagnosis can be established preoperatively by left heart catheterization and selective angiography. Recently, we experienced a case of multiple localized supravalvular aortic stenosis involving, just above the sinus Valsalva and just proximal of the innominate artery. The surgical correction which was performed by a vertical incision across the each narrowing of aorta with replacement of diamond shaped double velour Woven Dacron patch under the CPB. He was discharged without any event.

• Journal of Chest Surgery
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• v.11 no.2
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• pp.194-198
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• 1978

Congenital aortic stenosis, a relatively uncommon congenital heart disease, may cause heart failure and may be fatal. In recent years, increased clinical awareness and improved diagnostic and operative technique has made accurate diagnosis and successful treatment possible. Recently we experienced 2 cases of congenital aortic stenosis, and which was corrected surgically. The first case was 9 years old boy, and second case was 16 years male. Preoperative diagnosis was entertained by angiography and cardiac catheterization in both cases. In each case, aortic valve opening was widened by incision along the fused commissure between the combined left and right coronary cusp on one side, and the noncoronary cusp on the other side. Post-op. pressure gradient between the aorta and left ventricle markedly reduced, in the first case, 50mmHg, and in the 2nd case, 55mmHg.Both patients discharged with good results 2 weeks after open heart surgery.

• Journal of Chest Surgery
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• v.26 no.2
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• pp.135-140
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• 1993

Supravalvular aortic stenosis is an uncommon, congenital narrowing of ascending aorta just above aortic valve. Eleven patients underwent an aortoplasty to relieve supravalvular aortic stenosis at Sejong General Hospital from July 1985 to December 1991. Age ranged from 5 to 14 years(median 9 years). There were 7 male and 4 female patients. Seven patients had characteristics of Williams' syndrome including elfin face and mental retardation. All patients had localized, hourglass type but 4 patients had atypical findings. Preoperative left ventricula-aortic pressure gradient ranged from 40 to 190 mmHg(mean 88 mmHg). To relieve severe supravalvular aortic stenosis, extended aortoplasty was used in 7 patients and standard aortoplasty in 4 patients. Postoperative pressure gradient ranged from 0 to 40 mmHg (mean 16.6 mmHg). Follow-up pressure gradient with Doppler ranged from 0 to 88mmHg(mean 32.5mmHg).

• Journal of Chest Surgery
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• v.41 no.5
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• pp.573-579
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• 2008

Background: Composite valve graft replacement is currently the treatment of choice for a wide variety of the lesions of aortic root disease. The purpose of this study was to explore the results of aortic root replacement after using the Cabrol technique over a 13-year period at our institution, and we analyzed the results to help surgeons make better decisions when repairing aortic root disease. Material and Method: Between January 1994 and December 2006, twenty-five patients underwent a Cabrol technique operation at our institution. The mean patient age was $43.7{\pm}14.1$ years old (range: $6{\sim}65$ years) and the male and female ratio was 21:4 (84% : 16%). The patients' follow-up was 100% complete, and the mean follow-up period was $60.7{\pm}50.4$ (range:$1{\sim}162$) months. Annuloaortic ectasia (n=18) was the most frequent cause of aortic disease in this series, followed by aortic dissection (n=7). The mean cardiopulmonary bypass time was $177.2{\pm}44.9$ minutes and the mean aortic cross clamping time was $123.4{\pm}34.1$ minutes. Nine patients were checked with MDCT (Multidetector computed tomography) for evaluating a well functioning secondary graft and the coronary anastomosis site. Result: The early mortality rate was 4% (1 of 25 patients). A significant stenosis, kinking or occlusion of the secondary graft was detected by MDCT in 4 patients. The overall survival rate was 88%. Conclusion: The Cabrol technique demonstrated a significant incidence of long-term complications such as secondary graft stenosis or obstruction. It could be used when the modified Bentall technique is not feasible.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.724-728
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• 1997

We experienced a case of thoracic aortic aneurysm combined with coronary artery disease. A 68-year-old man complained of anginal pain in the left anterior chest and nonspecific pain in the posterior chest. The aneurysm was extending from left subclavian artery to the diaphragm and sign of impending rupture was noted in the chest CT. Coronary angiograms r vealed significant obstruction of left circumflex coronary artery(>95%) and left anterior descending artery(>50%). Exposure was obtained through the left posterolateral thoracotomy incision in the 4th intercostal space and then partial femoro-femoral cardiopulmonary bypass was established. After aortic cross clamping, the aneurysmal sac was opened and repaired with interposition of 26 mm Hemashield graft. Under the beating heart with femoro-femoral cardiopulmonary bypass, aorto-left circumflex coronary bypass with autogenous saphenous vein used as conduit was performed. Postoperatively multiple cerebral infarction ensued due to intraoperative hypovolemic shock and hypoxic brain damage during cardiopulmonary bypass. Currently, the patient's mental status is drowsy and in an improving state.