• Journal of Chest Surgery
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• v.36 no.2
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• pp.73-78
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• 2003

The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. Material and Method: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8 :-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1 Result: There were 3 early and late deaths respectively Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1) In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. Conclusion: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However, more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class, Although the results were not satisfactory enough in all patients.

• Journal of Chest Surgery
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• v.32 no.7
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• pp.621-627
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• 1999

Background: Sixty five cases with congenitally corrected transposition of the great arteries (CCTGA) indicated for biventricular repair were operated on between 1984 and september 1998. Comparison between the results of the conventional(classic) connection(LV-PA) and the anatomic repair was done. Material and Method: Retrospective review was carried out based on the medical records of the patients. Operative procedures, complications and the long-term results accoding to the combining anomalies were analysed. Result: Mean age was 5.5$\pm$4.8 years(range, 2 months to 18years). Thirty nine were male and 26 were female. Situs solitus {S,L,L} was in 53 and situs inversus{I,D,D} in 12. There was no left ventricular outflow tract obstruction(LVOTO) in 13(20%) cases. The LVOTO was resulted from pulmonary stenosis(PS) in 26(40%)patients and from pulmonary atresia(PA) in 26(40%) patients. Twenty-five(38.5%) patients had tricuspid valve regurgitation(TR) greater than the mild degree that was present preoperatively. Twenty two patients previously underwent 24 systemic- pulmonary shunts previously. In the 13 patients without LVOTO, 7 simple closure of VSD or ASD, 3 tricuspid valve replacements(TVR), and 3 anatomic corrections(3 double switch operations: 1 Senning+ Rastelli, 1 Senning+REV-type, and 1 Senning+Arterial switch opera tion) were performed. As to the 26 patients with CCTGA+VSD or ASD+LVOTO(PS), 24 classic repairs and 2 double switch operations(1 Senning+Rastelli, 1 Mustard+REV-type) were done. In the 26 cases with CCTGA+VSD+LVOTO(PA), 19 classic repairs(18 Rastelli, 1 REV-type), and 7 double switch operations(7 Senning+Rastelli) were done. The degree of tricuspid regurgitation increased during the follow-up periods from 1.3$\pm$1.4 to 2.2$\pm$1.0 in the classic repair group(p<0.05), but not in the double switch group. Two patients had complete AV block preoperatively, and additional 7(10.8%) had newly developed complete AV block after the operation. Other complications were recurrent LVOTO(10), thromboembolism(4), persistent chest tube drainage over 2 weeks(4), chylothorax(3), bleeding(3), acute renal failure(2), and mediastinitis(2). Mean follow-up was 54$\pm$49 months(0-177 months). Thirteen patients died after the operation(operative mortality rate: 20.0%(13/65)), and there were 3 additional deaths during the follow up period(overall mortality: 24.6%(16/65)). The operative mortality in patients underwent anatomic repair was 33.3%(4/12). The actuarial survival rates at 1, 5, and 10 years were 75.0$\pm$5.6%, 75.0$\pm$5.6%, and 69.2$\pm$7.6%. Common causes of death were low cardiac output syndrome(8) and heart failure from TR(5). Conclusion: Although our study could not demonstrate the superiority of each classic or anatomic repair, we found that the anatomic repair has a merit of preventing the deterioration of tricuspid valve regurgitations. Meticulous selection of the patients and longer follow-up terms are mandatory to establish the selective advantages of both strategies.

• Journal of Chest Surgery
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• v.35 no.12
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• pp.876-881
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• 2002

Acute aortic dissection associated with high mortality rate has an extremely poor prognosis if early diagnosis and treatment are not received. Recently, with advanced computed tomography and echocardiography, diagnostic rate is higher and early operation is possible. Therefore preoperative medical therapy at ER(emergency room) lowered the mortality rate. This study was done to analyze the results with preoperative management at ER and operations, retrospectively. Material and Method: A series of 42 patients treated surgically for acute aortic dissections from 1991 to 2001 were included in this study. There were 18 males and 24 females. Mean age was 51.1 years. The admission course through emergency and outpatient department(OPD) was 34 and 8 respectively. Result: 26 patients underwent ascending aorta replacement-7 combined aortic valve replacements, 7 patients underwent descending aorta replacements and 9 patients received Bentall's operation. At emergency department, 20 patients received antihypertensive drugs and $\beta$-receptor blockers and 6 patients died. 22 patients did not receive antihypertensive and $\beta$-receptor block drugs and 10 patients died. There were 16(38%) overall deaths. Conclusion: Early diagnosis at ER or OPD is essential for acute aortic dissection, and it is important to select the most appropriate noninvasive interventions as possible. Therefore, preoperative drug therapy at ER is suggested according the patient conditions.

• Journal of Chest Surgery
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• v.37 no.10
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• pp.856-860
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• 2004

Interrupted aortic arch with concomitant intracardiac defects is a rare congenital anomaly that has an unfavorable natural course. We report a successful staged operation of interrupted aortic arch with apical muscular ventricular septal defect associating esophageal atresia with tracheoesophageal fistula in a 3-day-old neonate weighing 2.6 kg. We repaired esophageal atresia through the right thoracotomy and subsequently performed extended end-to-end anastomosis of the aortic arch with pulmonary artery banding through the left thoracotomy at same operation. The apical muscular VSD was repaired 87 day after first operation. The patient required multiple additional interventions before closure of the apical muscular ventricular septal defect, such as pyloromyotomy for idiopathic hypertrophic pyloric stenosis, anterior aortopexy for airway obstruction, and balloon aortoplasty for residual coarctation. She is now doing well.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.609-615
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• 2005

Background: Some controversy still exists concerning the operative indications of coronary fistulas. Nevertheless, a short-term and long-term outcomes are excellent with surgical interventions. In this study, we assessed our surgical results on this disease entity during the last 20 years. Anatomic diversity was described as well. Material and Method: From April 1986 to March 2005, 20 patients with coronary fistulas underwent surgical correction in Seoul National University Children's Hospital. Their medical records were reviewed retrospectively. Result: Twelve patients ($60\%$) were asymptomatic prior to surgery. All had electrocardiogram and echocardiogram and all but 3 had coro-nary angiogram preoperatively. Anatomically, none of them had two or more coronary fistulas. The sites of origin were left coronary system in 11 patients and right in 9. The draining sites were right ventricle in 11, right atrium in 3, left ventricle in 3, main pulmonary artery in 2, and superior vena cavae in 1. All of the involved, the coro-nary arteries were dilated or aneurismal. In 1 case, there was atherosclerotic change but no ischemic evidence in preoperative electrocardiogram. Operative techniques included external obliteration (13), internal obliteration (5), and both (2). External obliteration was done by ligation of the fistulous tract only in T patients, by fstula ligation plus plication in 3 and by plication or patch closure via fistulotomy in 3. There was no operative mortality. All of postoperative morbidities including transient sinus arrhythmia (2), complete atrioventricular block (1), decreased left ventricular function (2), ventricular tachycardia (1), pericarditis (1), and seizure (1) improved on discharge. The mean follow-up was 55.1$\pm$50.2 months (4.0 months${\~}$18.0 years) and there were no recurrences of fistula. There was 1 second operation for aortic root aneurysm, which developed after external patch closure of right coronary fistula. Conclusion: We demonstrated here that coronary fistulas can be cured with excellent clinical outcome and low operative risk under precise diagnosis. Understanding the anatomic diversity will help to construct surgical plans.

• Journal of the Korean Orthopaedic Association
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• v.54 no.6
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• pp.519-527
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• 2019

Purpose: To evaluate the treatment result in polyostotic fibrous dysplasia classified according to the involvement of the femoral head. Materials and Methods: Twenty-three patients from March 1987 to March 2014 were reviewed retrospectively. Patients with no involvement of the physeal scar in the femoral head were classified as Type I, and those with involvement of the physeal scar were classified as Type II. A plain radiograph was used to measure the femoral neck shaft angle, articulo-trochanteric distance (ATD), and anterior bowing through the lateral view. A teleoroentgenogram of the lower limb was used to measure the leg length discrepancy and lower extremity mechanical axis. The pre- and postoperative femoral neck-shaft angle and ATD were compared to assess the degree of correction of the deformity. Results: Among a total of 46 cases (23 patients), 28 cases (23 patients) had lesions in the proximal femur. Type I were 16/28 cases (15/23 patients) and Type II were 12/28 cases (9/23 patients). The preoperative proximal femoral neck-shaft angle was 116.8° in Type I and 95.3° in Type II. The ATD was 12.08 mm in Type I and -5.54 mm in Type II. The deformity correction showed significant improvement immediately after surgery, the deformity correction was lost in Type II (neck shaft angle Type I: 133.8°-130.8°, Type II: 128.6°-116.9°, and ATD Type I: 17.66-15.72 mm, Type II: 7.44-4.16 mm). The extent of anterior bowing was 12.74° in Type I and 20.19° in Type II. The mean differences of 12 mm between the 9 patients who showed a leg length discrepancy and the lower extremity mechanical axis showed 4 cases of lateral deviation and 7 cases of medial deviation. Conclusion: In polyostotic fibrous dysplasia, when the femur head is involved, the femur neck shaft angle, ATD, and anterior bowing of the femur had more deformity, and the postoperative correction of deformity was lost, suggesting that the involvement of the femoral head was an important factor in the prognosis of the disease.

• Journal of Chest Surgery
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• v.39 no.8 s.265
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• pp.626-632
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• 2006

Background: Pulmonary hypertension caused by chronic pulmonary embolism is underrecognized and carries a poor prognosis. Medical therapy is generally unsatisfactory and palliative. With the improvement of operative technique and postoperative management, pulmonary endarterectomy has been the treatment of choice for this condition. Material and Method: Between January 2001 and December 2005, eleven patients were received pulmonary endarterectomy. All patients had chronic dyspnea and exercise intolerance. Diagnosis was made with cardiac echocardiography, lung perfusion scan and computed tomography. Before the operation, Greenfield vena cava filter were placed in all patient except one. Deep hypothermic circulatory arrest was used for the distal-most portion of the endarterectomy procedure. More than moderate degree of tricuspid reguirgitation was repaired during operation. Result: There was no early and late death. Right ventricular systolic pressure was reduced significantly after operation from $91{\pm}21$ mmHg to $40{\pm}17$ mmHg on echocardiography (p=0.001). NYHA class and tricuspid reguirgitaion were improved postoperatively. Although mild reperfusion injury in three case and postoperative delirium in one case were observed, all of them recovered without complication. Conclusion: Pulmonary thromboendarterctomy offers to patient an acceptable morbidity rate and anticipation of clinical improvement. This method is safe and effective operation for pulmonary hypertension caused by chronic pulmonary thromboembolism.

• Journal of Chest Surgery
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• v.29 no.10
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• pp.1081-1089
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• 1996

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.

• The korean journal of orthodontics
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• v.29 no.4 s.75
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• pp.467-481
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• 1999

Cleft lip and/or palate is one of the most common congenital craniofacial anomalies. According to previous epidemiologic studies, incidence of cleft lip and/or palate has been increasing nowadays. However, there is no report about epidemiologic study of cleft lip and/or palate patients who visited dept. of orthodontics in Korea. So the purpose of this study was to provide the epidemiological characteristics and important basic clinical data for the diagnosis and the treatment of the cleft lip and/or palate patients. With the orthodontic and cleft charts, diagnostic models and X-ray films from 250 patients with cleft lip and/or palate who visited Dept. of Orthodontics, Seoul National University Dental Hospital during the last 11 years, the authors investigated patient's visiting yew, types of cleft, patient's gender, and Angle's classification of malocclusion, and surgery timing. The results were as follows ; 1. The number of cleft patients who visited Dept. of Orthodontics, SNUDH increased during 1988-1990 and then it declined until 1992. From 1993 to 1996, it showed a stationary trend. After 1997 it showed an overwhelmingly increasing trend. 2. In the cleft type, the ratio of cleft lip cleft lip and alveolus cleft palate : cleft lip and palate was 7.6:19.2:9.6:63.6. In cleft position, unilateral clefts were more than bilateral ones (cleft lip 79:21, cleft lip and alveolus 77:23, cleft lip and palate 75.5:24.5). In cleft side, left clefts were mote than right clefts (cleft lip 53.3:46.7 cleft lip and alveolus 59.5:40.5, cleft lip and palate 59.2:40.8). 3. In gender ratio, males were more than females in cleft lip (57.9:42.1), cleft lip and alveolus (68.8:31.2) and cleft lip and palate (76.1:23.9). But in cleft Palate females were more than males as 41.7: 58.3. 4. In the age groups, 7-12 year group was the most abundant as $52\%$, and then 0-6 year group ($20.4\%$), 13-18 year group ($17.2\%$), more than 18 yew group ($10.4\%$) were followed as descending order. 5. Most of the cleft lip repair surgeries were operated in 0-3 month ($60.3\%$) and 4-6 month ($17.9\%$). 6. The cleft palate repair surgeries were done in 1-2 year ($31.7\%$), 0-1 year ($25.6\%$), 2-3 year ($12.1\%$), more than 5 year ($11.6\%$) as descending order. 7. The lip scar revision surgeries were done before admission at elementary school in $60\%$. (4-6 you ($27.5\%$), 6-8 year ($19.6\%$), more than 10 year ($19.6\%$), 2-4 year ($13.7\%$) as descending order) 8. The rhinoplasties were done before admission at elementary school in $51.7\%$. (0-2 year ($7.1\%$), 2-4 year ($14.3\%$), 4-6 year ($21.4\%$), 6-8 year ($14.3\%$)). 9. The pharyngeal flap were done at 6 Y (72.5 months) after birth on average and there was even distribution of surgery timing. 10. In relationship between Angle's classification of malocclusion and cleft types, Class I was most abundant and Class III, Class II were followed as descending order in cleft lip group. But Class III was most abundant and Class I, Class II were followed as descending order in cleft lip and alveolus group, cleft palate group, and cleft lip and Palate group. The percentage of frequency in Class III malocclusion was overwhelmingly higher in cleft lip and palate group than any other groups. 11. Because the frequency of class III malocclusion was most prevalent in all age groups, anterior crossbite was the most common chief complaint of cleft patients.

• Journal of Chest Surgery
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• v.32 no.5
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• pp.471-479
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• 1999

Background: Patients with tetralogy of Fallot(TOF), pulmonary atresia(PA) and major aortopulmonary collateral arteries(MAPCAs) have been managed by heterogenous surgical strategies. We have taken 5 different surgical approaches to this lesion. In this study, relative advantages and disadvantages of each strategy were discussed by analyzing our surgical results. Material and Method: Between January 1986 and June 1998, 50 patients aged 1 to 177(39${\pm}$31) months at a various morphologic spectrum of this lesion were treated at our institution. The groups, which were classified according to the initial approaches, consisted of one-stage unifocalization(A-1, n=9), staged unifocalization(A-2, n=11), right ventricle to pulmonary artery connection(RV-PA)(B-1, n=11), one-stage RV-PA plus unifocalization (B-2, n=11), and one-stage definitive repair(C, n=8). Morphologic charateristics, operative mortalities, and probabilities of definitive repair were compared between the groups and the causes of death, complications and the follow-up results were described. Result: Mean ages at the first operation were 57${\pm}$18(A-1), 42${\pm}$48(A-2), 18${\pm}$14(B-1), 52${\pm}$55(B-2), and 32${\pm}$34(C) months (p<0.05). Mean numbers of MAPCAs were least in group C (A-1=4.3${\pm}$1.0, A-2=4.5${\pm}$1.3, B-1=4.1${\pm}$1.9, B-2=4.1${\pm}$1.6, C=3.4${\pm}$1.8 : p<0.05). The ratios of the direct and the indirect MAPCAs in each group were not different between the grousps (A-1=91%/9%, A-2=78%/22%, B-1=80%/20%, B-2=80%/20%, C=81%/19% : p>0.05). Nineteen patients had more than 1 dependent MAPCAs. True pulmonary arteries were not present 13 patients and they were confluent in 29. A total of 101 operations were performed. Operative mortalities of initial procedures were 33%(3/9, A-1), 18%(2/11, A-2), 0%(0/11, B-1), 36%(5/11, B-2) and 13%(1/8, C) (p<0.05). Percentages of the definitive repair among the patients of each group were 22%(2/9, A-1), 18%(2/11, A-2). 45%(5/11, B-1), 27%(4/11, B-2), and 100%(8/8, C)(p<0.05). Hypoxic respiratory and cardiac failure(6), hypoxic encephalopathy(2) and sepsis(4) were the major causes of death. Phrenic nerve palsy occured in 5 patients. Thirty-one patients among the survivals have been followed up for a mean duration of 74${\pm}$42(3-145) months. One and five year actuarial survival rates were 73% and 73 %. Conclusion: In conclusion, Right ventricle to pulmonary artery connection used in patients with TOF/PA/MAPCA as an inital procedure appeared to be highly successful in enhancing the chance of satisfactory definitive repair without the significant surgical risks. One-stage total repair at an ealier age group could be performed safely with the resonable outcomes. Unifocalization approach, whether it was performed in a single stage or in the multiple stages, resulted in the high operative mortality and the lowest chance of definitive repair, however more tailored selection of the patients and the long follow-up is mandatory to prove the usefulness of this approach.