• Proceedings of the KOR-BRONCHOESO Conference
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• 2004.09a
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• pp.20-20
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• 2004

• Journal of Chest Surgery
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• v.37 no.4
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• pp.313-321
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• 2004

Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (＞30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.261-266
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• 2002

Vascular ring, originating from abnormal regression of the aortic arch during fetal life, can cause prolonged and recurrent respiratory symptoms and dysphagia when the diagnosis is delayed. We report a 4 month old girl with vascular ring, who had been treated for persistent respiratory symptoms including stridor, wheezing, and dyspnea soon after birth. Initially her respiratory symptoms were thought to be due to bronchiolitis, for which respiratory syncytial virus was confirmed by immunofluorescent staining. Her clinical course was again complicated with tracheitis and pneumonia due to Haemophilus influenzae type b. The possibility of anatomical anomaly was investigated when it was felt to be difficult to insert a suction catheter deep down through a endotracheal tube which was placed for adequate ventilatory management. A three-dimensional chest CT revealed a vascular ring consisting of a double aortic arch. For 5 months following surgery, her respiratory symptoms have slowly been improving. She developed another episode of pneumonia which was milder than the one which occurred before the surgery.

• Journal of Chest Surgery
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• v.27 no.11
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• pp.893-901
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• 1994

Forty nine patients [M: 31, F: 18], age from 2 months to 17 years [mean= 4.9 years], underwent operations, from April 1986 to December 1992, for the relief of subvalvular aortic stenosis in normal atrioventricular and ventriculoarterial connections.There were 4 anatomic types of subaortic stenosis : membranous in 29 cases [59.2%], fibromuscular in 11 [22.4%], diffuse tunnel type in 7 [14.3%], and miscellaneous in 2 cases. Thirty four patients [69.4%] had associated cardiac anomalies, of which ventricular septal defect was the most common [27 cases]. Other anomalies were patent ductus arteriosus, coarctation of the aorta, valvular aortic stenosis, double chambered right ventricle [DCRV], infundibular pulmonic stenosis, persistent left superior vena cava, and rigt aortic arch. Mean systolic pressure gradient between the left ventricle and ascending aorta was 26.4$\pm$17.6 mmHg : 13.1$\pm$17.6mmHg in the membranous type, 22.0$\pm$18.4mmHg in the fibromucular type, and 56.1$\pm$38.4mmHg in the diffuse tunnel type. Operative procedures were determined according to the type of subvalvular aortic stenosis : simple excision of subaortic membrane in the membranous type [29 cases], left ventricular myectomy with or without myotomy or fibrous tissue excision in the fibromuscular type [11 cases]. Among the 7 of diffuse tunnel type cases, ventricular myectomy was performed in 2 and a modified Konno operation was performed in 5 . Postoperative follow up was made with periodic echocardiography. The Mean postoperative follow up period was 33.8 months. There were 2 hospital mortalities [4.1%] and 2 late deaths. Residual stenosis remained in 3 cases and recurrence developed in 2 cases during the follow up period. 5 years actuarial survival rate was 91.8$\pm$3.9% and 5 year complication free rate was 72.3$\pm$10.4%. Conclusions : 1. Subvalvular aortic stenosis should be relieved completely as soon as possible when diagnosed, regardless of left ventricular outflow tract pressure gradient. 2. Good results were obtained using only simple excision of subaortic membrane in the membranous type of subaortic stenosis. However, aortoventriculoplasty [modified Konno prodedure] was necessary for good results in the diffuse tunnel type. 3. Periodic postoperative echocardiography was helpful in detecting the progression of residual stenosis and development of new stenosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Journal of Chest Surgery
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• v.23 no.3
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• pp.474-481
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• 1990

We have experienced 44 cases of coarctation of aorta in the age of infancy and children from April 1986 to September 1989 at Seoul National University Children`s hospital. Patients were thirty males and fourteen females, and their age ranged from one month to ten years[mean 23.84 $\pm$33.06 months] with thirty-two infant cases. In the infantile age, congestive heart failure was the most common chief complaint[18/32], and above that age, frequent upper respiratory infection was most common[8/12]. We experienced thirteen cases of isolated COA, twenty-two cases of COA with VSD, eight cases of COA with VSD, eight cases of COA with intracardiac complex anomalies and one case of COA with atrial septal defect. The associated intracardiac complex anomalies were three Taussig-Bing type double outlet right ventricle, one single ventricle, one transposition of great arteries, one atrioventricular septal defect, one hypoplastic aortic arch with left heart hypoplasia, and one Tetralogy of Fallot. Operative techniques of COA were twenty-three subclavian flap arterioplasty, 12 resection and end to end anastomosis, eight onlay patch angioplasty, and I direct angioplasty after resection of web. Among the cases with other cardiac anomalies, staged operation was done in twenty-nine patients, and single stage total correction was performed only in three patients. There were seven operative mortality[15.9%], all being in infantile age group, and among fourteen cases associated with large VSD[Qp/Qs>2.0, mean pulmonary arterial pressure>50mmHg], four patients were died, but there was no mortality in patients with small VSD. With above results, we are intended to discuss about the interval between staged operation, the fate of VSD after coarctoplasty in case of COA with VSD, causes of death, complications etc.

• Journal of Chest Surgery
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• v.44 no.2
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• pp.115-122
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• 2011

Background: The intramural coronary artery has been known as a risk factor for early death after an arterial switch operation (ASO). We reviewed the morphological characteristics and evaluated the early and mid-term results of ASO for patients with an intramural coronary artery. Materials and Methods: From March 1994 to September 15th 2010, 158 patients underwent ASO at Dong-A and Pusan National University Hospitals for repair of transposition of the great arteries and double outlet right ventricle. Among these patients, 14 patients (8.9%) had an intramural coronary artery. Mean age at operation was $13.4{\pm}10.2$ days (4 to 39 days) and mean body weight was $3.48{\pm}0.33$ kg (2.88 to 3.88 kg). All patients except one were male. Eight patients had TGA/IVS and 4 patients had an aortic arch anomaly. Two patients (14.3%) had side-by-side great artery relation, of whom one had an intramural right coronary artery and the other had an intramural left anterior descending coronary artery. Twelve patients had anterior-posterior relation, all of whom had an intramural left coronary artery (LCA). The aortocoronary flap technique was used in coronary transfer in 8 patients, of whom one patient required a switch to the individual coronary button technique 2 days after operation because of myocardial ischemia. An individual coronary button implantation technique was adopted in 6, of whom 2 patients required left subclavian artery free graft to LCA during the same operation due to LCA injury during coronary button mobilization and LCA torsion. Results: There was 1 operative death (7.1%), which occurred in the first patient in our series. This patient underwent an aortocoronary flap procedure for coronary transfer combining aortic arch repair. Overall operative mortality for 144 patients without an intramural coronary artery was 13.2% (19/144). There was no statistical difference in operative mortality between the patients with and without an intramural coronary artery (p>0.1). There was no late death. The mean follow-up duration was $52.1{\pm}43.0$ months (0.5 to 132 months). One patient who had a subclavian artery free graft required LCA stenting 6.5 years after surgery for LCA anastomotic site stenosis. No other surviving patient needed any intervention for coronary problems. All patients had normal ventricular function at latest echocardiography and were in NYHA class 1. Conclusion: The arterial switch operation in Transposition of Great Arteries or Double Outlet Right Ventricle patients with intramural coronary can be performed with low mortality; however, there is a high incidence of intraoperative or postoperative coronary problems, which can be managed with conversion to the individual coronary button technique and a bypass procedure using a left subclavian free graft. Both aortocoronary flap and individual coronary button implantation techniques for coronary transfer have excellent mid-term results.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.263-269
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• 1997

From April 1987 to May 1996, 13 infants underwent a Norwood operation for complex congenital heart diseases including hypoplastic left heart syndrome (n : 7), mitral stenosis with small VSD and subaortic stenosis (n : 1), mitral atresia with ventricular septal defect, coarctation of aorta, and subaortic stenosis (n = 1), interrupted aortic arch with ventricular septal defect and subaortic stenosis (n : 1), tricuspid atresia with transposition of the great arteries (n = 1), and complex double-inlet left ventricle (n : 2). All patients without hypoplastic left heart syndrome were associated wit hypoplasia of ascending aorta and arch. Age at operation ranged from 3 days to 8.7 months (mean 60.6 $\pm$ 71.6 days, median 39 days). The operative mortality( < 30 days) was 46% (6 patients). Late mortality was 15% (2 patients). All operative deaths occured during the Erst 24 hours after the operation as a result of cardiopulmonary bypass weaning failure (5 patients) and sudden hemodynamic instability postoperatively (1 patient). Late death was due to aspiration pneumonia in two cases. There are 5 long-term survivals (39%). Three of them have undergone a two-stage repair with a modified Fontan operation in two and total cavopulmonary shunt in one at 12, 17, 4.5 months after Norwood procedure with no mortality. Two patients have entered a three-stage repair strategy by undergoing a bidirectional cavopulmonary shunt at 3 and 5.5 months after initial operation with 1 operative death. The actuarial survival rate for all patients at the first-stage operation, including hospital deaths and ate death was 30.8% at 1 year. In conclusion, the operative mortality of Norwood operation was relatively high compared to other operation for major cardiac anomalies, continuing experience will lead to an improvement in result.

• Journal of Chest Surgery
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• v.15 no.1
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• pp.90-97
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• 1982

Two hundred one patients of ventricular septal defect, which were operated at Seoul National University Hospital, were analysed on clinical background during the period from January, 1975 to December, 1980. The results were as follows: 1. Of the 201 patients, 118 patients were male [58.7%] and 83 patients were female [41.3%]. Their age ranged from 15 months to 40 years, and the mean age was 8.7 years. 40% of the patients were between 4 and 8 years. 2. The most common symptoms showed frequent U RI and exertional dyspnea. 3. On Kirklin`s anatomical classification, type I constituted 26.9%, type II 58.2%, type III 12.4%, and type IV 1.0%. We showed marked increased incidence of type I VSD as compared to Caucasians* 4. 46 cases were associated with other congenital cardiac diseases. They were PDA [13 cases], AI[11 ], ASD[6], PS[10], MI[4], and Double aortic arch [1]. 5. In 128 patients, who had complete hemodynamic data and were not associated with other congenital cardiac diseases, an attempt was made to correlate the EKG findings with the hemodynamic data, and defect size with the hemodynamic data. The children had variable distribution of PA syst. pr. and Rp/Rs. But most of adults had $R_P$/$R_S$of 0.15 or less. As $P_P$/$P_S$increased, the rate of operative complication increased also. 6. When a normal EKG pattern was present, $Q_P$/$Q_S$and $R_P$/$R_S$and $P_P$/$P_S$were relatively low. When EKG findings were LVH pattern, there was diastolic volume overload to left ventricle. As RVH, there was systolic pressure overload to right ventricle. And as BVH, there was mixed pattern of diastolic volume overload to left ventricle and systolic pressure overload to right ventricle. 7. Among patients in defect was less than 1 $cm^2$ per $M^2$ of BSA, $Q_P$/$Q_S$was less than 2:1, and $R_P$/$R_S$less than 0.25, and PAsyst. pr. less than 50 mmHg, and $P_P$/$P_S$was less than 0.5. But patients with the defect greater than 1 $cm^2$ per $M^2$ of BSA had no correlationship between $Q_P$/$Q_S$, $R_P$/$R_S$, PAsyst. pr. and defect size in each other. Most of patients with the defect greater than 2 $cm^2$/$M^2$ BSA, $R_P$/$R_S$was greater than 0.5. 8. Operative mortality rate was 9.5% [19 cases] among 201 patients. And complication rate including mortality rate was 22.9% [46 cases].

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.99-103
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• 2013

Purpose: This study was designed to determine the frequency and echocardiographic findings of 22q11.2 deletions in fetuses with cardiac defects on fetal ultrasound or familial backgrounds of 22q11.2 deletions. Materials and methods: We retrospectively reviewed the medical and ultrasonographic records of 170 fetuses that underwent fluorescence in situ hybridization (FISH) analysis for chromosome 22q11.2 deletions between February 2001 and April 2013. Results: Among 145 fetuses with cardiac defects, six (4.1%) had 22q11.2 deletions. Deletions of 22q11.2 were detected in 6 (5%) of the 120 fetuses with conotruncal defects: 5 (8.9%) of 56 with tetralogy of Fallot (TOF) and 1 (5.9%) of 17 with double outlet right ventricle (DORV). No deletions were found in cases of pulmonary atresia, truncus arteriosus, right aortic arch, or transposition of the great arteries. No 22q11.2 deletions were found in non-conotruncal cardiac malformations. Among 25 fetuses with familial backgrounds of 22q11.2 deletions, one (4%) had a maternally inherited 22q11.2 deletion with no cardiac findings. Conclusion: Knowledge of the frequency and echocardiographic findings of 22q11.2 deletions might be helpful for prenatal genetic counseling. It is advisable to perform FISH analysis for 22q11.2 deletions in pregnancies exhibiting conotruncal cardiac defects such as TOF or DORV.