• 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.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.650-659
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• 1998

Between August 1996 and August 1997, 22 patients underwent extracardiac Fontan operations. The basic diagnoses included univentricular heart of the right ventricular type (n=12); univentricular heart of the left ventricular type (n=4); tricuspid atresia (n=4); left isomerism, transposition of great arteries, ventricular septal defect and pulmonary stenosis (n=1); and criss-cross heart with uneven ventricle (n=1). The median age of the 14 men and 8 women was 29 months (range from 21 months to 26 years). Previous procedures included bidirectional cavopulmonary shunt (n=15, interval=15.6$\pm$3.4 months), Kawashima operation (n=4, interval=37.5$\pm$20 months), and classic Glenn shunt (n=1, interval=14 years). In 2 patients, extracardiac Fontan operations were done without any previous procedures. A 16- to 22-mm flexible Gore-Tex tube graft (n=18), Hemashield graft (n=3), or, alternatively, a nonvalved aortic allograft (n=1) was cut and anastomosed end-to-end between inferior vena cava and undersurface of pulmonary artery using Gore-Tex or Prolene suture in a running fashion. In risk Fontan patients (n=12), a communication between the extracardiac conduit and the right atrium was constructed. In the most 13 recent patients, the procedures were done without cross-clamping of the aorta and with a beating heart. Operative mortality was 9.1% (n=2). Complications included persistent chest tube drainage for more than 7 days (n=5), chorea (n=2), and low cardiac output (n=1). There were no late deaths. Follow-up echocardiogram (mean: 6 months) demonstrated satisfactory hemodynamic results in the surviving 20 patients. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creationof a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function. Further investigations during a longer follow-up are needed to confirm the intermediate and long-term results, especially the reduction of late atrial arrhythmias.

• Journal of Chest Surgery
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• v.40 no.9
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• pp.624-628
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• 2007

Between 2001 and 2006, 3 neonates that had multilevel left ventricular outflow tract obstruction and a ventricular septal defect underwent the Norwood-Rastelli procedure. The body weights ranged from 2.9 to 3.1 kg. The patients had a near normal sized mitral valve and left ventricle. We simultaneously performed a modified Norwood procedure with native tissues-to-tissue anastomosis without circulatory arrest, and a Rastelli type procedure using a non-valved conduit from the right ventricle to the pulmonary artery and intracardiac patch baffling from the left ventricle to the pulmonary valve via the ventricular septal defect. The postoperative courses were uneventful. During follow-up, there was one late mortality caused by a cardiac catheterization related complication at 7 months after surgery, One patient required a Rastelli conduit change. Two patients are doing well during a follow-up period of 1 and 5 years, respectively.

• Journal of Chest Surgery
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• v.29 no.8
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• pp.828-835
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• 1996

From January 1993 to April 1995, 27 neonates (under age of 30 days underwent open heart surgery in the Department of Thoracic and Cardiovascular Surgery, Dong-A Medical Center. Mean age and weight were 12.1 days(2days∼306ays) and 3.29 kg(2.6kg∼4.1 kg) respectively. Cardiac anomalies were simple complete transposition of great arteries(TGA) in 11 neonates, TGA with coarctation of aorta(COA) in 1 , total anomalous pulmonary venous connection(TAPVC) in 5, double inlet right ventricle with TAPVC in 1, interrupted aortic arch(IAA) with ventricular septal defect(VSD) in 3, pulmonary atresia(PA) with intact ventricular septum(IVS) in 3, pulmonary stenosis with IVS in 1, Taussig-Bing anomaly with IAA in 1, and hypoplastic left heart syndrome(HLHS) in 1 . Postoperative complications were myocardial and/or pulmonary edema which caused open sternum in 13 patients(54.2%), acute renal failure( RF) in 10(37.0%), Intractable low cardiac output syndrome (LCOS) including weaning failure from cardiopulmonary bypass in 7(25.9%), bronchopulmonary dysplasia in 1, wound infection in 1, and paroxysmal supraventricular tachycardia in 1. Nine of 13 patients with postoperative open sternum were recovered with delayed sternal closure, and seven of 10 patients survived postoperative ARF with peritoneal dialysis. There were 8 operative deaths(29.6%): 3 in the patients with simple complete TGA, 1 In TCA with COA, 1 in PA with IVS, 1 in Taussig-Bing anomaly with IAA, 1 in DIRV with TAPVC, and 1 in HLHS. One late death occurred after arterial switch operation in simple TGA. The mosts common cause of death was low cardiac output syndrome. Our initial experience of open heart surgery in neonates showed high operative mortality and morbidity, especially in complex anomalies.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.554-560
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• 2003

Purpose : To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. Methods : 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. Results : The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. Conclusion : Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.

• Journal of Chest Surgery
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• v.40 no.3 s.272
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• pp.193-199
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• 2007

Background: Reoperation is usually required for a right ventricle to pulmonary artery conduit obstruction caused by valve degeneration, conduit peel formation or somatic growth of the patient. An autologous tissue reconstruction (peel operation), where a prosthetic roof is placed over the fibrotic tissue bed of the explanted conduit, has been used to manage conduit obstructions at our institute since May 2002. Herein, the early and midterm results are evaluated. Material and Method: Between May 2002 and July 2006, 9 patients underwent obstructed extracardiac conduit replacement with an autologous tissue reconstruction, at a mean of 5.1 years after a Rastelli operation. The mean age at reoperation was $7.5{\pm}2.4$ years, ranging from 2.9 to 10.1 years. The diagnoses included 6 pulmonary atresia with VSD, 2 truncus arteriosus and 1 transposition of the great arteries. The preoperative mean systolic gradient was $88.3{\pm}22.2mmHg$, ranging from 58 to 125 mmHg. The explanted conduits were all Polystan valved pulmonary conduit (Polystan, Denmark). A bioprosthetic valve was inserted in 8 patients, and a monocusp ventricular outflow patch (MVOP) was used in 1 patient. The anterior wall was constructed with a Gore-Tex patch (n=7), MVOP (n=1) and bovine pericardium (n=1). Pulmonary artery angioplasty was required in 5 patients and anterior aortopexy in 2. The mean cardiopulmonary bypass time . was 154 minutes, ranging from 133 to 181 minutes; an aortic crossclamp was not performed in all patients. The mean follow-up duration was 20 months, ranging from 1 to 51 months. All patients were evaluated for their right ventricular outflow pathway using a 3-D CT scan. Resuit: There was no operative mortality or late death. The mean pressure gradient, assessed by echocardiography through the right ventricular outflow tract, was 20.4 mmHg, ranging from 0 to 29.6 mmMg, at discharge and 26 mmHg, ranging from 13 to 36 mmHg, at the latest follow-up (n=7, follow-up duration >1 year). There were no pseudoaneurysms, strictures or thrombotic occlusions. Conclusion: A peel operation was concluded to be a safe and effective re-operative option for an obstructed extracardiac conduit following a Rastelli operation.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.737-744
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• 2005

Purpose : Infants and children with cardiovascular diseases often present with respiratory symptoms. However, missed or delayed evaluation for potential airway problem may complicate overall prognosis. The aim of this study is to determine the clinical characteristics of these patients and explore the cause of airway problem. Methods : We reviewed the medical records of 64 patients(M : F=33:31, mean age : $6.3{\pm}7.5$ months) whose airway problems were proven by computed tomography or bronchoscopy in perioperative periods at the Asan Medical Center from January 1997 to June 2004. Patients were divided into two groups based on the duration of ventilator care : ${\leq}7$ days(group 1 : 23 cases, M : F=10 : 13) and >7 days(group 2 : 41 cases, M : F=23 : 18). Results : The patients in group 2 significantly developed more post-operative respiratory symptoms than group 1(P<0.001) and had more airway problems including extrinsic obstruction, intrinsic anomaly, and combined problem than group 1 although not significantly different(P=0.082). Among underlying diseases, the most common diseases were vascular anomaly(26.2 percent) and aortic arch anomaly(26.2 percent) in group 1 and pulmonary atresia with ventricular septal defect(22.4 percent) in group 2. The most frequent respiratory symptoms were recurrent wheezing pre-operatively and failure of ventilator weaning post-operatively. The major types of airway anomaly were tracheomalacia and tracheal stenosis(in each case 18.2 percent). Nineteen patients with persistent airway problems underwent aortopexy or other vascular correction. Of the 19 patients, 13(68.4 percent) were improved, but 2 failed in weaning ventilator and 4 died of non-airway problems. Conclusion : Early evaluation and treatment for potential airway problems may affect natural or surgical prognosis in patients with cardiovascular diseases presenting with respiratory symptoms.