• Journal of Chest Surgery
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• v.28 no.6
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• pp.565-570
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• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

• Journal of Chest Surgery
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• v.18 no.3
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• pp.371-382
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• 1985

Over the past 4 decades after World War II a great deal of data and clinical experiences have been accumulated relating to the diagnosis and surgical treatment of congenital and acquired cardiovascular diseases in Korea. Clinical data on cardiovascular surgical cases performed in all 22 hospitals for open heart surgery in Korea was collected from it`s starting up to December 1984. The first recorded open heart surgery for a young adult atrial septal defect was performed by Young Kyoon Lee, M.D. on August 7, 1959, Seoul National University Hospital, Korea. And, some scattered reports on cardiovascular surgical cases in it`s early period have changed recently the number of hospitals for open heart surgery and clinical cases enormously in total amount of 13, 100 cardiovascular operations performed on 12, 990 cases up to December 1984. Of the total 13, 100 cardiovascular operations, congenital cardiovascular anomaly occupied 70%. Of the congenital cases, 6, 580 operations for acyanotic group [operative mortality 4.5%], and 2, 489 operations for cyanotic group [operative mortality 20%]. The incidence of congenital cardiovascular anomaly in order of frequency was ventricular septal defect [29%], patent ductus arteriosus [26%], Tetralogy of Fallot [22%], atrial septal defect [8, 6%], pulmonary valve stenosis [3.0%], and endocardial cushion defect [1.1%]. Of the 3, 412 valvular heart disease cases, which occupied 85% of total 4, 031 acquired cardiovascular disease, individual incidence was in mitral 2, 565 [63.5%], double valve 451 [11.1%], and simple aortic valve 351 [8.7%]. Total number of valve replaced, mechanical and tissue, was 2, 795, and 1, 884 for mitral, 808 for aortic, and 103 for tricuspid in position. Operative mortality for prosthetic valve replacement in total was 9.8%. Remained acquired cardiovascular cases chronic constrictive pericarditis [7.9%], cardiac tumor [1.9%], coronary artery bypass [1.8%], cardiac trauma [1.2%] and less than 1% of thoracic aortic aneurysm. Overall operative mortality for open and non-open cardiovascular surgical operation was 7.7% [congenital acyanotic group 3.2%, congenital cyanotic group 19.4%, and acquired group 7.8%]. In conclusion, present status of cardiovascular surgery in Korea is stabilized with accumulation of clinical cases and experiences, and the future in the field of cardiovascular surgery is promising, especially in the infant cardiac surgery and aortocoronary bypass surgery, with abrupt increase of specialized cardiac centers, trained specialists, and expanding social health insurance.

• Clinical and Experimental Pediatrics
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• v.58 no.10
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• pp.392-397
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• 2015

Purpose: Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis. Methods: We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013. Results: The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001). Conclusion: The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.

• Journal of Chest Surgery
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• v.36 no.6
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• pp.384-390
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• 2003

Background: Since Ross and Sormeville first reported the use of aortic homograft valve for correction of pulmonary atresia in 1966, homograft valves are widely used in the repair of congenital anomalies as conduits between the pulmonary ventricle and pulmonary arteries. On the basis of these results, we have used it actively. In this report, we describe our experience with the use of cryopreserved valved homograft conduits for infants and children requiring right ventricle to pulmonary artery connection in various congenital cardiac anomalies. Material and Method: Between January, 1996 and December 2001, 27 infants or children with a median age of 16 months(range 9days to 18years) underwent repair of RVOTO using homograft valved conduit by two surgeons. We studied 22 patients who have been followed up at least more than one year. The diagnosis at operation included pulmonary atresia with ventricular septal defect (n=13), truncus arteriosus (n=3), TGA or corrected TGA with RVOTO (n=6). Homograft valved conduits varied in size from 15 to 26 mm (mean, 183.82 mm). The follow-up period ranged from 12 to 80.4 months (median, 48.4 months). Result: There was no re-operation due to graft failure itself. However, early progressive pulmonary homograft valve insufficiency developed in one patient, that was caused by dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia after repair of pulmonary atresia with ventricular septal defect. This patient was required reoperation (conduit replacement). During follow-up period, there were significant pulmonary stenosis in one, and pulmonary regurgitation more than moderate degree in 3. And there were mild calcifications at distal anastomotic site in 2 patients. All the calcified homografts were aortic in origin. Conclusion: We observed that cryopreserved homograft conduits used in infant and children functioned satisfactorily in the pulmonic position at mid-term follow-up. To enhance the homograft function, ongoing investigation is required to re-establish the optimal strategy for the harvest, preservation and the use of it.

• Journal of Chest Surgery
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• v.26 no.8
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• pp.604-608
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• 1993

Coarctation of aorta is rather common congenital cardiovascular disease in the western contries, but it is known to be less than 2 % in Korea. From June 1986 to December 1992, seven patients of surgically treated coarctation of aorta who were less than 2 years old, were experienced at Department of Thoracic and Cardiovascular Surgery, Yeungnam University Hospital. The patients included six male and one female, with ages in the range of one month and 24 months. Four patients were preductal type and three juxtaductal. Associated cardiac anomalies were present in all patients and they were PDA[6 cases], ASD[3], VSD[2], bicuspid aortic valve[2], aortic stenosis[1], mitral regurgitation[1], and tricuspid regurgitation[1]. The operative procedures were four end to end anastomosis and three subclavian flap aortoplasty. Mean aortic cross clamping times were 37.3 minutes in patients with end to end anastomosis and 30.3 minutes in patients with subclavian flap aortoplasty. There were two operative deaths in patients who were treated with subclavian flap aortoplasty and pulmonary artery banding. One patient who had been treated with subclavian flap aortoplasty was complicated with postoperative mild paraplegia in lower limb. Pulmonary artery banding has been disappointing in our patients, and the data was suggestive that earlier total repair of complicated coarctation might improve survival.

• Journal of Yeungnam Medical Science
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• v.10 no.1
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• pp.237-244
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• 1993

A free-floating ball thrombus in the left atrium is a rare complication of the mitral valvular disease. A 53-year-old man was admitted for pain and paresthesia on both legs. On admission he had auscultatory sign of mitral stenosis and mitral regurgitation, and the roentgenogram of his chest revealed a slight pulmonary venous congestion, enlargement of the pulmonary conus and cardiomegaly. Laboratory findings including complete blood counts, coagulation studies and blood chemistry were normal. An echocardiographic examination revealed a mitral stenosis and a free-floating ball thrombus in the left atrium. We performed the emergent open heart surgery for removal of the ball thrombus and mitral replacement successfully with Duromedics 29 mm valve. The size of thrombus was $39{\times}32{\times}30$ mm.

• 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.53 no.5
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• pp.306-309
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• 2020

A 59-year-old man presented for possible durable ventricular assist device (VAD) implantation. He had previously been diagnosed with congenitally corrected transposition of the great arteries, a ventricular septal defect, an atrial septal defect, pulmonary valve stenosis, and aortic valve regurgitation. In the previous 22 years, he had undergone palliative cardiac surgery 3 times. VAD implantation as a bridge to transplantation was planned. Owing to severe adhesions, mesocardia, a left ascending aorta, and moderate aortic regurgitation, we performed VAD implantation and aortic valve closure via a dual left thoracotomy and partial sternotomy.

• Journal of Yeungnam Medical Science
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• v.5 no.2
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• pp.101-110
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• 1988

Between April, 1984 and September, 1988, 459 patients underwent cardiovascular surgery at the Yeungnam University Hospital. Of these, 355 cases were open heart surgeries and 104 cases were non-open heart surgeries. There were 237 patients of acyanotic congenital cardiac anomalies, 40 patients of cyanotic congenital cardiac anomalies, and 85 patients of acquired heart diseases. The sex ratio of cardiovascular diseases was represented as 1:1.3 in male and female. The age distribution was ranged from 1 day to 65 years old. The common congenital cardiovascular anomalies were ventricular septal defect(38.7%), patent ductus arteriosus(25.5%), atrial septal defect(20.7%), Tetralogy of Fallot(8.3%), and pulmonary stenosis(2.4%) in order of frequency. Among 87 acquired cardiovascular diseases, 81 patients underwent operation for cardiac valvular lesions. 51 patients had mitral valve replacement and 13 patients had aortic valve replacement and 17 patients had double valve replacement. The overall mortality of cardiovascular surgery was 3.3% and mortality of open heart surgery was 3.9%.

• Journal of Chest Surgery
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• v.49 no.5
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• pp.344-349
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• 2016

Background: Monocusp reconstruction with a transannular patch (TAP) results in early improvement because it relieves residual volume hypertension during the immediate postoperative period. However, few reports have assessed the long-term surgical outcomes of this procedure. The purpose of the present study was to evaluate the mid-term surgical outcomes of tetralogy of Fallot (TOF) repair using monocusp reconstruction with a TAP. Methods: Between March 2000 and March 2009, 36 patients with a TOF received a TAP. A TAP with monocusp reconstruction (group I) was used in 25 patients and a TAP without monocusp reconstruction (group II) was used in 11 patients. We evaluated hemodynamic parameters using echocardiography during the follow-up period in both groups. Results: At the most recent follow-up echocardiography (mean follow-up, 8.2 years), the mean pulmonary valve velocities of the patients in group I and group II were $2.1{\pm}1.0m/sec$ and $0.9{\pm}0.9m/sec$, respectively (p=0.001). Although the incidence of grade 3-4 pulmonary regurgitation (PR) was not significantly different between the two groups (group I: 16 patients, 64.0%; group II: 7 patients, 70.0%; p=0.735) during the follow-up period, the interval between the treatment and the incidence of PR aggravation was longer in group I than in group II (group I: $6.5{\pm}3.4years$; group II: $3.8{\pm}2.2years$; p=0.037). Conclusion: Monocusp reconstruction with a TAP prolonged the interval between the initial treatment and grade 3-4 PR aggravation. Patients who received a TAP with monocusp reconstruction to repair TOF were not to progress to pulmonary stenosis during the follow-up period as those who received a TAP without monocusp reconstruction.