• Journal of Chest Surgery
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• v.27 no.11
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• pp.902-906
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• 1994

Between May 1991 and August 1993, 16 patients underwent repair of complete atrioventricular septal defect without another major anomaly at Cardiovascular Center,Yonsei University College of Medicine. Ages of the patients ranged from 3 months to 38 years with a mean of 42 months. Among 16, 10 patients[63%] are associated with Down`s syndrome. All patients underwent primary repair except and one who received had been repaire of coactation of aorta and patent ductus arteriosus 2 month before. Preoperative mitral valve regurgitation [MR] was evaluated with Doppler echocardiography and angiography which revealed absent or grade I in 1, grade II in 8, grade III in 4, and grade IV in 3. Operative technique was performed under the moderate hypothermic cardiopulmonary bypass with crystalloid cardioplegia. Intraoperative echocardiography was performed epicardial approach [n=7] in the operative table or transthoracic approach [n=9] at intensive care unit. In all patients except 3, MR were improved. But in 3 patients, was not improved or exagerated comparing preoperative one. All of them were died.One patient was showed MR grade IV in intraoperative echocardiography, we re-repaired atriventricular valve with cardiopulmonary bypass. During follow-up period [at a mean of 11 months after repair], doppler echocardiography was performed in all patients. The follow up echocardiography revealed that the degree of MR in immediate postoperative period was not changed except in two patients in whom it was aggravated. Thus it seems that intraoperative and early postoperative echocardiography was employed important role of survival and can be predictable for long term results.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.381-388
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• 1984

Cor triatriatum is rare congenital cardiac anomaly first described by Church in 1868. The anomaly consists of an abnormal partitioning of the left atrium by a fibromuscular membrane that divides the atrium into an upper chamber, which receives the pulmonary veins, and a lower chamber, which contains the atrial appendage and the mitral valve. The upper and lower chambers communicate through a stenotic fenestration in the membrane, which has the hemodynamic consequence of pulmonary venous obstruction. Recently we experienced cot triatriatum associated with partial anomalous pulmonary drainage to right atrium. The upper chamber was connected to right atrium through a sinus venous type of ASD and received left superior and both inferior pulmonary vein, whereas the lower chamber so called true left atrium communicated with right atrium through foramen ovale type of ASD, left atrial appendage and mitral orifice. And the anomalous membrane has no fenestrations which permit blood flow. The operation was made right atrial approach under the CPB. We excised completely the anomalous septum and reconstructed atrial septal defect with pericardial patch to drain the right upper pulmonary vein to the left atrium. The postoperative course has been good during follow up.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.60-62
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• 2007

Pulmonary hypoplasia is an entity of pulmonary agenesis. Pulmonary agenesis is a rare congenital anomaly, usually diagnosed soon after birth. It is commonly associated with other anomalies, mainly of the cardiovascular systems. Although it may hasten the death of a child, sometimes it is compatible with normal growth. We report a right lateral thoracotomy approach for mitral valve repair in a young woman with pulmonary hypoplasia, in whom preoperative computed tomography showed severe right side shifting of the mediastinum and total collapse of the right lung.

• Journal of Chest Surgery
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• v.20 no.1
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• pp.218-222
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• 1987

Unroofed coronary sinus syndrome is an uncommon anomaly, Unroofed coronary sinus syndrome is caused by incomplete formation of the left atriovenous fold, and it usually is associated with Left SVC. If it is not diagnosed, a residual reversed or bidirectional shunt will result, and its complications will reduce life expectancy. We experienced 3 cases of unroofed coronary sinus syndrome which combines TOF with PLSVC, partial ECD, primum type ASD. In case of Unroofed coronary sinus syndrome which combines TIF with PLSVC, preoperative diagnosis was not made. In corrective operation for TOF of this case, pump weaning was failed due to hypoxia and cardiac arrest, and he expired at operation room. At autopsy of this case, complete unroofed coronary sinus was found. In the other 2 cases, partial unroofed coronary sinus syndrome was found in operation field and corrective operation was performed successfully. We report these 3 experiences with its review. ^u ++ Noninvasive Assessment of Pressure Gradients across Prosthetic Heart Valve by Doppler Ultrasound - A comparative study of the Duromedics Bileaflet Valves in mitral position and Normal Mitral Valves -with its review.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.158-161
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• 1990

A congenital coronary artery fistula is an uncommon anomaly which has a direct communication between a coronary artery and the lumen of any one of the four cardiac chambers, or the coronary sinus, or its tributary veins or the superior vena cava. The right coronary artery is involved most frequently, and the abnormal communication in most often is to the right ventricle followed in incidence by drainage into the right atrium and the pulmonary artery. Recently. we experienced a case of congenital coronary artery fistula associated with valvular heart disease. The fistulous communication was noted between the left circumflex artery and the left atrial appendage. Under the cardiopulmonary bypass, the internal obliteration of the left atrial appendage, mitral valve replacement, and aortic valve exploration were accomplished. Postoperative hospital course was uneventful and the patient was discharged without any problems.

• Journal of Chest Surgery
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• v.15 no.2
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• pp.250-253
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• 1982

A persistent left superior vena cava draining into the left atrium associated with atresia of the coronary sinus-ostium, ASD, and PDA is a rare congenital anomaly. The patient was a 4 year-old female whose complaints were frequent URI and exertional dyspnea. The congenital heart anomaly was suspected at 2 months of her age. Chest films showed cardiomegaly [C-T ratio, 75%]. EKG, Echocardiography, cardiac catheterization and angiocardiography were performed. Open heart surgery was done under impression of LV-RA shunt, bilateral superior vena cavae, and ASD. At the time of operation, huge LA and RA, inferior vena caval defect of a secundum type ASD [1.5 x 3cm in diameter], absence of innominate vein, atresia of the coronary sinus-ostium, and persistent LSVC draining into LA were noted. Direct suture closure of ASD and ligation of LSVC were done. The patient`s postoperative course was somewhat eventful: systolic murmur at apex remained. Four months after the operation, congestive heart failure attacked a few times. PDA that was overlooked at the time of open heart surgery was detected through postoperative cardiac catheterization in.4 months later. Emergent operation for closure of PDA was performed on the day of recatheterization. After that, patient`s heart failure was easily controlled without any notable problem.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.289-296
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of Chest Surgery
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• v.34 no.9
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• pp.680-685
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• 2001

Background: Tricuspid valve replacement is very rarely performed procedure and its long-term result is not yet satisfactory. Moreover, it is not well known whether bioprosthesis or mechanical prosthesis is the best selection for artificial valve. We reviewed 72 cases of tricuspid valve replacements in 71 patients between January 1989 and December 1998, trying to analyze the overall results and risk factors for mortality and morbidity. Material and Method: Average age of the patients at the time of operation was 42$\pm$13 years(range 16 to 65 years) and the sex ratio of male versus female was 32/39. Primary diagnosis consisted of 50 cases of aquired valvular heart disease and 18 cases of congenital heart disease, such as Ebstein’s anomaly. 4 cases had isolated tricuspid valve regurgitation. Implanted valves were 69 mechanical prosthesis and 3 bioprosthesis. Concomitant mitral or aortic valve replacements were performed in 50 cases. One patient received concomittant pulmonary valve replacement. Result: There were 7(9.72%) operative deaths and 7(13.0%) late deaths. Actuarial survival at 10 years was 59.2$\pm$7.2%. Prosthetic tricuspid valve thrombosis occurred 11 times in 5 patients. Reoperation for prosthetic tricuspid valve failure was performed in 1 patient. In this case, examination of the explanted prostheses showed that the tricuspid stenosis was the result of valve thrombosis. Among the 47 survivors, 46 patients(98%) were in functional class I or II. Conclusion: In our ten-year experience of tricuspid valve replacement, mortality and morbidity were satisfactory. Mechanical prosthesis in tricuspid position showed comparable clinical results as bioprosthesis.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.523-528
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• 2005

Background: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. Material and Method: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. Result: There were 2 hospital death $(16.7\%)$. The mean follow-up period was $7.1\pm4.1$ years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for $2.2\pm1.1$ days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening $(33.4\pm9.1\%\;vs\;17.7\pm9.6\%,\;n=10,\;p<0.05);$ left ventricular end diastolic dimension $(33.4\pm7.3\;mm\;vs\;44.8\pm7.0\;mm,\;n=10,\;p<0.05)$ and systolic dimension $(22.2\pm7.5\;mm\;vs\;33.4\pm7.9\;mm,\;n=10,\;p<0.05)$. Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). Conclusion: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

• 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.