• Journal of Chest Surgery
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• v.39 no.10 s.267
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• pp.775-778
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• 2006

Hypertrophic cardiomyopathy is characterized by inappropriate hypertrophy of the myocardium and is associated with various clinical presentations ranging from complete absence of symptoms to sudden, unexpected death. These are caused by dynamic obstruction of the left ventricular outflow tract and surgical approaches were initiated. But, the complete resection of hypertrophied midventricular septum is impossible by standard, transaortic approach, because of narrow vision and limited approach. And it leads to inadequate excision, will leave residual left ventricular out-flow tract obstruction or systolic anterior motion of mitral leaflet, and limit symptomatic improvement and patient's survival. We report a case of extended septal myectomy for hypertrophic cardiomyopathy of mid-septum in a child. The extended septal myectomy was performed by aortotomy and left ventricular apical incision, and made possible the complete resection of mid-ventricular septum, abnormal papillary muscles and chordae. The patient's symptom was improved and the postoperative course was uneventful.

• Journal of Chest Surgery
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• v.36 no.4
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• pp.277-279
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• 2003

A 15 year-old boy was referred to us because of mild dyspnea on exertion and incidentally found heart murmur. On echocardiography, a mass involving mainly interventricular septum and causing left ventricular outflow tract obstruction was detected. Cardiac catheterization demonstrated a transaortic pressure gradient of 20 mmHg. Partial excision of the septal mass was performed via aortotomy under cardiopulmonary bypass. The pathologic diagnosis revealed myocardial hamartoma. The lesion was mainly composed of mature, severely hypertrophic myocytes and intervening fibrosis. During the 5 year of follow-up after the surgery, no evidence of arrhythmia or tumor recurrence was documented.

• Journal of Chest Surgery
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• v.54 no.5
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• pp.389-392
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• 2021

Double outlet right ventricle (DORV) with pulmonary atresia (PA) is an uncommon congenital disease. Because of anatomical and physiological anomalies in the systemic and pulmonary circulation, corrective surgery may be challenging. We present the case of a patient with DORV and PA. This condition was successfully corrected using a modified Nikaidoh procedure, resulting in reduced obstruction of the left ventricular outflow tract.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.660-667
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• 1998

Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

• Journal of Chest Surgery
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• v.19 no.1
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• pp.165-173
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• 1986

During the 4 year period from 1982 through 1985, twelve patients have undergone operations for discrete subaortic stenosis with good short-term clinical result at Department of Thoracic and cardiovascular Surgery, S.N.U.H. According to the cineangiographic and operative findings, nine of the 12 patients were classified as Deutsch type I, the other 3 as type II, and eleven of the 12 had one or more associated anomalies of the cardiovascular system such as PDA[5], VSD[5], left SVC[2], MS[1], COA[1], supramitral membrane[1], DORY[1], right aortic arch[1], DCRV[1], and TOF[1] [one with Shone`s complex], and three of them had secondary cardiac disorders such as aortic regurgitation[3],mitral regurgitation[2], and tunnel shaped dynamic obstruction of left ventricular outflow tract[2]. We have performed membrane resection via oblique aortotomy with retraction of the aortic cusps in 7 cases and via VSD from right cardiac chamber in 5 cases with large VSD and have also performed the operations on the correctable associated anomalies. There was only one operative death in patient with associated TOF due to neurologic complication and no other postoperative difficulties except in one patient with transient heart block resolved spontaneously on postoperative 3rd day. To our knowledge, this article is the first report of operation for discrete subaortic stenosis in Korean literature.

• Journal of Veterinary Clinics
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• v.34 no.1
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• pp.54-57
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• 2017

A 1-year-old castrated male Schnauzer dog was presented with heart murmur. Auscultation revealed systolic murmur located at the left heart base (grade 5/6). There were no remarkable findings on thoracic radiographs. Two-dimensional echocardiography revealed subaortic tunnel-like obstruction at the entrance to the left ventricular outflow tract. Anterior mitral valve leaflet appeared to be tethered to septum with minimal motion. Chordae tendineae was abnormally thickened. Color Doppler analysis revealed turbulent flow starting below the aortic valve. Mitral regurgitation was presented during systole. Spectral Doppler recordings revealed high velocity flow through the aorta and mitral regurgitation. Based on echocardiographic examination, the dog was diagnosed with subaortic stenosis concurrent with mitral dysplasia. The patient was medicated with ${\beta}-blocker$ and diuretics. It has been doing well without apparent clinical signs at 2 year after the diagnosis.

• Journal of Chest Surgery
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• v.23 no.2
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.18-29
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• 1983

Thirty-two patients with a cyanotic cardiac malformations having more complex intracardiac defects than ones in a tetralogy of Fallot underwent complete intracardiac repairs in a-full-year period from July 1981 to June 1982. Twenty-two patients [68.8%] died within 30 days after surgery: Transposition of the great arteries, seven of 10 patients; Double-outlet right ventricle, four of 6 patients; Tricuspid atresia, four of 6 patients; Single ventricle, all of 4 patients; Pulmonary atresia, two of 3 patients; Double-outlet left ventricle, none of 2 patients; and Truncus arteriosus, one of a single patient. All deaths occurred with a low cardiac output syndrome or a failed off-bypass, and they were almost always accompanied with other grave postoperative complications. The complex intracardiac anatomy itself was one of the risk factors by making a complete intracardiac repair of the defects difficult in a small heart. The reconstruction of the right ventricular outflow carried a difficulty in balancing an adequate relief of the obstruction with an avoidance of making too much pulmonary valvular insufficiency as well. On the other hand, the presence of an elevated pulmonary arterial pressure and a high pulmonary vascular resistance was also the factors affecting the postoperative surviv als. The importance of detailed knowledge of intracardiac anatomy and hemodynamics from the careful preoperative evaluation of the patient was discussed along with the necessity of technical refinement of the correction.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.83-87
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• 1997

Taussig-Bing anomaly is infrequently associated with interrupted aortic arch and size discrepancy of great arteries makes it difficult to undergo arch reconstruction and arterial switch operation. A 20-day old male infant was admitted with the diagnosis of Taussig-Bing anomaly with type B Interrupted aortic arch. Multi-organ failure, due to the diminution of ductal flow, was stabilized after 3 weeks of prostaglandin El and controlled ventilatory support. The surgical correction consisted of VSD closure, arterial swtich and extended aortic arch reconstruction. The marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery was overcome by constructing distal neoaorta using both native ascending and descending aortic tissue. The patient was extubated on postoperative 2nd day Postoperative catheterization showed no left ventricular outflow obstruction, no intracardiac shunt, and no incompetence of neoaortic valve.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.545-550
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• 2005

Background: Discrete subaortic stenosis is known to recur frequently even after surgical resection. We retrospectively reviewed the preoperative and postoperative changes in pressure gradient through left ventricular outflow tract, and the recurrence rate. Material and Method: Between September 1984 and December 2004, 34 patients underwent surgical treatment. Mean age of patients was $17.1\pm15.2$ years and 19 patients $(55.9\%)$ were male, 16 patients $(47.1\%)$ had previous operations and associated diseases were aortic regurgitation (11), coarctation of aorta (3), and others. Result: Immediate postoperative peak pressure gradient was significantly lower than preoperative peak pressure gradient (21.8 mmHg vs 75.8 mmHg, p<0.04). Peak pressure gradient measured after 50.3 months of follow up was 20.2 mmHg which was also significantly lower than that of preoperative value but not significantly different from that of immediate postoperative value. There was no surgical mortality but one patient developed cerebral infarction. Mean follow up duration was $69.8\pm54.6\;months$. During this period, 5 patients $(14.7\%)$ had reoperation, 3 $(8.8\%)$ of whom were due to recurred subaortic stenosis. We found no risk factors for recurrence and survival for free from reoperation was $76.4\%$. Conclusion: Excision of subaortic membrane combined with or without myectomy in discrete subaortic stenosis showed sufficient relief of left ventricular outflow tract obstruction with low mortality and morbidity, but careful long term follow up is necessary for recurrence, since it is not predictable.