• 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.41 no.3
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• pp.305-312
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• 2008

Background: Anatomic and functional abnormalities of the systemic atrioventricular (AV) valve are common in single ventricle. pathologies and continue to be associated with poor early and late outcomes in surgically palliated single. ventricle patients. We aggressively performed valvuloplasty for atrioventricular valve regurgitation (AVVR) during the course toward a Fontan operation. Material and Method: Between January 1995 and December 2004, 209 patients underwent a Fontan operation in our institution. We retrospectively evaluated the prevalence of AVVR and the influence of AV valve repair on outcome, and we analyzed the progression of AVVR after the Fontan operation for 168 patients where echocardiographic follow up results for more than 6 months after the Fontan operation were available. During the course toward a Fontan operation, 25 patients underwent 30 procedures for AVVR. These procedures. were. carried out during placement of a bidirectional cavopulmonary shunt (BCPS) for nine patients, between the time of placement of a BCPS and the Fontan operation for four patients, and during the Fontan operation for 17 patients. Five patients underwent procedures for AVVR twice. Result: The late mortality rate after the Fontan operation was 4.2% (n=7), with a median follow-up duration of 52 months (range, $6{\sim}123$ months). Seven patients (4%) had unfavorable outcomes such as significant (moderate or severe) AVVR in six patients, and significant AV valve stenosis in one patient was determined at the last follow up after the Fontan operation. Among the seven patients, four patients underwent AV valve repair after the Fontan operation, and one patient underwent subsequent AV valve replacement. Progression to AVVR of equal to or greater than grade 2 was noted in 30 patients (18%) at the last follow up after the Fontan operation, including 12 patients that underwent previous AV valve procedures. Initial grading of AVVR, a previous AV valve operation, and specific AV valve morphology such as a common AV valve or mitral atresia were significant risk factors for the progression of AVVR after the Fontan operation. Conclusion: In our surgical series, a small percentage of patients showed unfavorable outcomes. related to AVVR during the course toward a Fontan operation. However, a closer follow-up is required to evaluate the progression of the AVVR after a Fontan operation, especially for patients showing poor AV valve function at the first presentation and specific AV valve morphology.

• Journal of Chest Surgery
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• v.31 no.12
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• pp.1183-1194
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• 1998

Background: From Sept. 1985 to Sept. 1997, 2,000 cases of open heart surgery(OHS) were performed in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College of Medicine, Inje University. Material and Method: Among the total of 2,000 cases of OHS, 1532 cases were congenital heart disease(CHD) and 468 cases were acquired heart disease(AHD). The age distribution was 9 days(4.0kg) to 68 years in CHD and 11 to 66 years in AHD. In 1532 cases of CHD, there were 1403 acyanotic cases and 129 cyanotic cases. Result: The CHD cases consisted of 940 ventricular septal defects(61.4%), 324 atrial septal defects(21.1%), 112 tetralogy of Fallot(7.3%), 46 pulmonary stenosis(3%), 38 endocardial cushion defects(2.5%), 15 valsalva sinus ruptures(1%), 4 transposition of great arteries (0.3%), 4 double outlet right ventricles(0.3%), and etc. Corrective operations were applied for congenital heart disease with a result of 3.1% hospital mortality. Of 468 AHD, 381 cases were valvular heart diseases, 48 ischemic heart diseases, 12 cardiac tumors, 8 annuloaortic ectasias, 16 dissecting aortic aneurysms and etc. In the 381 valvular heart diseases, there were 226 single valve replacements(36 aortic valve replacements(AVR), 188 mitral valve replacements(MVR), and 2 tricuspid valve replacements(TVR), among these were 71 cases of double valve replacements(AVR & MVR), 54 cases of MVR with tricuspid valve annuloplasty(TVA), and 18 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 466. In MVR, 123 St. Jude Medical valves, 90 Carpentier-Edwards valves, 65 CarboMedics valves, 42 Sorin valves and 16 other valves were used. In AVR, 68 St. Jude Medical valves, 36 CarboMedics valves, 14 Carpentier-Edwards valves and 9 other valves were used. Coronary Artery Bypass Surgery(CABG) were performed in 48 cases. The patterns of bypass graft were 14 patients of single vessel graft, 21 patients of two vessels graft, 10 patients of three vessels graft and 3 patients of four vessels graft. Conclusion: The hospital operation mortality rate of congenital acyanotic, cyanotic and acquired heart diseases were 2.0%, 15.5%, and 5.1% respectively. The overall mortality rate was 3.6%(72/2,000).

• Journal of Chest Surgery
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• v.35 no.3
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• pp.209-216
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• 2002

Background: Valvular heart disease is still the most common health problem in Korea. By the end of the year 1999, there has been 94,586 cases of open heart surgery since the first case in 1958. Among them, 36,247 cases were acquired heart diseases and 20,704 of those had valvular heart disease. But there was no database system and every surgeon and physician had great difficulties in analysing and utilizing those tremendous medical resources. Therefore, we developed a valve registry database program and utilize it for risk factor analysis and so on. Material and Method: Personal computer-based multiuser database program was created using Microsoft AccessTM. That consisted of relational database structure with fine-tuned compact field variables and server-client architecture. Simple graphic user interface showed easy-to-use accessability and comprehensibility. User-oriented modular structure enabled easier modification through native AccessTM functions. Infinite application of query function aided users to extract, summarize, analyse and report the study result promptly. Result: About three-thousand cases of valve replacement procedure were performed in our hospital from 1968 to 1999. Total number of prosthesis replaced was 3,700. The numbers of cases for mitral, aortic and tricuspid valve replacement were 1600, 584, 76, respectively. Among them, 700 patients received prosthesis in more than two positions. Bioprosthesis or mechanical prosthesis were used in 1,280 and 1,500 patients respectively Redo valve replacements were performed in 460 patients totally and 40 patients annually Conclusion: Database program for registry of valvular heart disease was successfully developed and used in personal computer-based multiuser environment. This revealed promising results and perspectives in database management and utilization system.

• Journal of Chest Surgery
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• v.34 no.8
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• pp.597-603
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• 2001

Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.630-634
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• 2009

According to the 2007 International Society for Heart and Lung Transplantation (ISHLT) report, a congenital diagnosis, infantile transplantation and being on extracorporeal membrane oxygenation (ECMO) at the time of transplant are risk factors for mortality for the patients who undergo a heart transplant, and a large body weight ratio also increases the risk of mortality. The patient of this case underwent a Ross operation and mitral valve repair due to left ventricle outflow track obstruction and mitral regurgitation. But the baby was treated with ECMO due to heart failure after the operation. When he was 3-months-old and had been. on 30 days of ECMO, he underwent a heart transplant with a heart that had a high donor-recipient weight ratio (4.42). We present this case from a technical standpoint and we include a review of the relevant literature.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.675-679
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• 2005

Background: Clinical reports on replacement of all three (AV + MV + TV) valves are rare. Material and Method: From January 1992 to December 2003, 38 patients received triple valve replacement (aortic, mitral, tricuspid) at Yonsei Cardiovascular Center. Mean age of patients was $49.5\pm10.7 (28\~69)$ years, and 24 patients $(63.1\%)$ were female. Rheumatic valve disease was the most common cause of operation (n=37). Preoperative New York Heart Association functional class were II in 4, III in 24 and IV in 10. Fifteen patients (group 1) received triple valve replacement at their first operation. Twenty three patients (group 2) received one or more operations before tricuspid valve replacement (TVR). Seven patients received tricuspid valve annuloplasty at first operation and received TVR later. Result: Six patients died at hospital after operation $(15.8\%)$ and all these patients were in group 2. All patients in group I survived and were discharged. Three patients $(9.4\%)$ died during follow up periods. Most of the survivors had improved functional class (I in 22, II in 8, III in 1, IV in 1). During follow up period, there were 4 valve related complications. The 10-year survival rate was $68.8\%$ and survival rate for free from valve related event at 10 years was $85.5\%$. Conclusion: After triple valve replacement, most patients showed improvement of symptoms. And during follow up period, valve related complications and survival were acceptable. Therefore, if indicated, triple valve replacement is recommended before the patients' conditions get worse.

• Journal of Chest Surgery
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• v.14 no.3
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• pp.260-267
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• 1981

Left atrial myxoma is a rare disease and its recurrence is reported to be always possible whenever primary excision is incomplete. Cardiac Myxoma is rare disease of the heart, and it has a little chance of recurrence. We experienced a patient of recurrent left atrial myxoma who was 31 years old, had been gotten excision of Lt. atrial myxoma when she was 28 years of her age. She was gotten mitral valve replacement simultaneously during 2nd operation with difficulty. So we report this case with the review of the literatures. [KTCS 1981;3:260-267]Surgical Treatment of Acute Pyogenic Pericarditis followed the Sepsis Ki Woo Shin, M.D.,* Ho Wan Lee, M.D.* and Dong Jun Lee, M.D.* Two cases of acute pyogenic pericarditis are, one case, 12-year-old male patient, followed the bacteremia of pneumonia and other case, 9 year old female patient, followed the bacteremia of osteomyelitis. After the confirmed diagnosis by pericardial aspiration, the emergency pericardial window was made to relief the severe cardiogenic symptoms. The general symptoms were improved immediately, but 40 days and 15 days after pericardiostomy, in each case, the sign and symptoms of cardiac compression were seen with recurrent cardiac tamponade. Pericardiectomy with median sternotomy was performed in each case and thereafter the patients were discharged without any problems.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.156-162
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• 2014

A 25-month-old boy was referred to the hospital due to large head detected on routine physical examination. At visit, dysmorphic facial appearances, including broad nose, prominent forehead, and coarse face, were noted. Nasal obstruction with nasal voice, prominent adenoids, and bilateral middle ear effusions were detected. His abdomen was distended, and liver and spleen were palpated about 3 finger and 2 finger breadths, respectively. He was operated for bilateral inguinal hernias. The motion of both elbow joints was mildly limited on supination and pronation. Urinary level of glycosaminoglycan was elevated and the enzyme activity of iduronate sulfatase in leukocytes was decreased. The mutational analysis of the gene iduronate 2-sulfatase (IDS) revealed c.263G>A (p.Arg88His) mutation. His developmental scale showed delayed development and there was cardiac valvular involvement (tricuspid regurgitation and mitral valve prolapse). After the diagnosis of Hunter syndrome, enzyme replacement therapy started on a weekly basis without progression of any clinical features. Here we report a case of early diagnosed Hunter syndrome detected by large head on routine examination. Thus, it is important to associate Hunter syndrome in the patient with large head especially, if there is the history of bilateral inguinal hernia and prominent adenoids to increase the possibility of early diagnosis and treatment.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.187-192
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• 2023

Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.