• Journal of Chest Surgery
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• v.29 no.11
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• pp.1191-1196
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• 1996

Recently, if there is pulmonary hypertension in ventricular septal defect, early operation without pulmonary artery banding is recommended even though patient is under 1 year in age or has low body weight. We also had been performing operations under the above mentioned policy. From October 1986 to December 1995, eighty eight cases of ventricular septal defect under 10Kg in body weight were operated upon by open cardiac surgery. Mortality was compared by age, body weight and degree of pulmonary vascular hypertension. Total mortality was 10cases(11.4%); under 6months, the mortality was 5 in 14 cases(35.8%), from 6 months to 1year, 4 in 43 cases(9.3%) and over 1 year, one in 31 cases(3.2%), while there was no significant difference in mortality compared by degree of pulmonary vascular hypertension. And mortality under 6months in age was so high regardless of severity of pulmonary hypertension. After this exprience, too early operation,. especially under 6 months, should be considered very prudently, unless there were life threatning heart failure, impending irreversible pulmonary vascular bed change or failure to thrive.

• Journal of Chest Surgery
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• v.46 no.2
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• pp.98-103
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• 2013

Background: Atrial fibrillation (AF) is a common complication in elderly patients with atrial septal defect (ASD). The purpose of this study was to examine the efficacy of the maze procedure in these patients. Materials and Methods: Between February 2000 and May 2011, 46 patients underwent the maze procedure as a concomitant operation with ASD closure. Three patients who underwent a right-sided maze were excluded, and one patient was lost to follow-up. The mean follow-up duration was $3.2{\pm}2.5$ years. Electrocardiography was performed 1 month, 3 months, 6 months, and 1 year after surgery, and checked annually after that. Results: AF persisted in 4 patients after surgery. One year after surgery, among 38 patients, 55.3% remained in sinus rhythm without antiarrhythmic drugs. However, when including the patients who took antiarrhythmic drugs, 92.1% were in sinus rhythm. Freedom from AF recurrence at 3 months, 6 months, 1 year, 2 years, 3 years, and 5 years after surgery were $97.4{\pm}2.6$, $94.4{\pm}3.8$, $91.2{\pm}4.9$, $87.8{\pm}5.8$, $79.5{\pm}7.6$, and $68.2{\pm}12.4$, respectively. There was no early mortality after operation. Conclusion: Concomitant treatment with the maze procedure and ASD closure is safe and effective for restoring the sinus rhythm.

• Journal of Chest Surgery
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• v.43 no.1
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• pp.58-62
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• 2010

Rastelli repair has been considered the procedure of choice for surgically repairing transposition of the great arteries combined with ventricular septal defect and pulmonary stenosis. However, the long term results have been less than optimal and these patients who ungo this procedure can eventually display conduit obstruction, left ventricular outflow tract obstruction and arrhythmias. Many new procedures are now available and they are technically challenging for making a more normal anatomic repair. In our hospital, two patients who had a TGA with VSD and PS have been repaired with a Half Turned Truncal Switch Operation and a Lecompte maneuver in 2003 and 2006, respectively. We report on our two experiences with performing a Half Turned Truncal Switch Operation, and we discuss the changes of the biventricular outflow tract.

• Journal of Chest Surgery
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• v.25 no.1
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• pp.32-41
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• 1992

From August 1982 to December 1991, 58 consecutive infants with tetralogy of Fallot underwent primary repair. Age ranged from 22 days to twelve months [n=58, 8.7$\pm$2.7 months] and body weight from 3.1 to 13 kilograms [n=58, 7.8$\pm$1.7 kilograms]. Qne infant had absence of the pulmonary valve; one had Ebstein`s anomaly and one had supramitral ring. Thirty-two patients [56%] experienced anoxic spell. Preoperative pulmonary artery indices were measured in 38 cases, ranging 126-552mm2/M2BSA[n=38, 251$\pm$79mm2/M2BSA]. All infants required a right ventricular outflow tract patch; in 41, the patch extended across the pulmonary valve annulus, in 13 of them, monocusps were constructed. All had patch closure of ventricular septal defect. Two infants had REV operation for avoiding injury to the canal branch of the right coronary artery which cross the right ventricular out flow tract. Post repair PRV/LV were measured at operating room in 40 cases, which revealed mean value of 0.49$\pm$0.12 [range: 0.25-0.74]. The hospital mortality was 10.3% [6 patients], and causes of deaths were right heart failure due to sustained right ventricular hypertension[4] and right ventricular outflow tract obstruction, intractablesuraventricular tachyarrhythmia[1], hypoxia[1] due to residual right to left shunt across the atrial septal defect in patient associated with Ebstein`s anomaly. All infants were doing well at follow-up from 1 to 101 months[20.6 months /patient, 1, 072 patient-month] Serial postoperative echocardiograms revealed no residual ventricular septal defects and estimated RVOT gradients between 0 and 40 mmHg except 3 cases [50, 50, 60 mmHg]. There were no late deaths and late ventricular arrhythmias or congestive heart failure. Redo operations were done in 2 cases because of residual right ventricular outflow tract obstruction. This experience with infants with tetralogy of Fallot suggests that, if mortality is tolerable, eletive repair of tetralogy of Fallot could be reasonably undertaken during the first year of life, and even better results could be anticipated along with improvement of methods of myocardial protection and postoperative care.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.627-632
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• 1986

This report describes our 17-years experience with intracardiac repair in 123 patients older than 15 years with tetralogy of Fallot. Major clinical manifestation was cyanosis and clubbing [102 Pts], but other minor associated manifestation were infective endocarditis, pulmonary tuberculosis, brain abscess, congestive heart failure, nephrotic syndrome, and tuberculous spondylitis. Prior palliative shunts had been performed in 10 patients. Preoperative hemoglobin ranged from 9.7 gm/dl to 25 gm/dl [mean 19 gm/dl]. The type of ventricular septal defect were typical perimembranous type, and total canal defect [13%]. The right ventricular outflow tract obstruction was due to combined [58.5%], infundibular [35%], and valvular stenosis [6.5%]. Transannular patch was used in 17% of patients. Hospital mortality was 9.8% in overall, but decreased to 1.7% since 1982. There was two late death [12 year actuarial survival [97%] due to fulminant hepatitis, residual abnormalities [PS, VSD]. Ninety two percent of survivors at follow-up are asymptomatic and leading an active normal life. Residual ventricular septal defect was detected with radionuclide single pass study in 15.3% of patients but almost cases were Qp/Qs less than 1.5, and only two patients had been candidates for reoperation.

• Journal of Chest Surgery
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• v.23 no.3
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• pp.430-437
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• 1990

We clinically evaluated 121 cases of ventricular septal defect which we operated from April, 1986 to December, 1989 at Inha General Hospital, Seong-Nam, Department of Thoracic and Cardiovascular Surgery, College of Medicine, Inha University. These patients were occupied 54.8% of all congenital heart diseases operated on its same period. Of the 121 patients, 63 patients were male[52.1%] and 58 patients were female[47.9i]. The two most common symptoms were frequent upper respiratory infection and dyspnea on exertion. By Kirklin s anatomical classification, type I constituted 34.7%, type II 61.98%, type III 0.03% and type IV not occupied. Associated cardiac anomalies were found in 34 cases, and PDA was most common associated anomaly, occupied in 22 cases. On the cardiac catheterization data, there were statistically significant correlation between VSD size[cm2 /BSAm2] and systolic pulmonary arterial pressure[sPAP], pulmonary to systemic flow ratio[Qp/Qs] & pulmonary to systemic pressure ratio[Pp/Ps] respectively, Type II [r=0.53, p<0.01] was more correlated than type I [r=0.49, p<0.05] between VSD size and Qp /Qs. We could not found the correlationship between age and Qp/Qs [Type I; r=0.16, Type II; r=-0.15] All cases were operated under cardiopulmonary bypass and 58 cases[46.3%] were operated through the right atrial approach, and 34 cases[28.1%] through the pulmonary arterial approach. Operative mortality rate was 4.13%[5 cases].

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.147-151
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• 2021

The Say-Barber-Biesecker-Young-Simpson variant of Ohdo syndrome (SBBYSS) (Online Mendelian Inheritance in Man #603736) is a rare autosomal dominant disorder and clinically features blepharophimosis with ptosis, a mask-like facial appearance, cryptorchidism, congenital heart defect, long thumbs/great toes, and thyroid dysfunction. The etiology of SBBYSS has been shown to be due to heterozygous KAT6B gene mutation. Here we report a case of a neonate with SBBYSS identified a novel mutation in KAT6B gene. The patient showed typical dysmorphic facies, cryptorchidism with micropenis, overriding fingers, and long thumbs and toes at birth. He had also hypothyroidism, large atrial septal defect, and sensorineural hearing loss. The next generation sequencing identified a heterozygous novel variant, c.5206C>T (p.Gln1736Ter) in KAT6B gene. At the 9 months of age, he underwent patch closure for atrial septal defect. Until the 12-month follow-up, he was under-developed.

• Journal of Chest Surgery
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• v.39 no.12 s.269
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• pp.906-912
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• 2006

Background: Ventricular septal defect(VSD) is rare but feared complication after acute myocardial infarction. The patient could survive by surgery only, but the surgical mortality is still high. We investigate the surgical result and predictors for early surgical mortality. Material and Method: Between August 1993 and February 2006, 8 patients (male, 2: female, 6) with postinfarct VSD underwent surgical repair. Seven patients had one-vessel disease of left anterior descending artery, and 6 of them had wide extension of infarction to posterior septal wall as well as anterior septal wall of both ventricles. One patient had concomitant coronary bypass grafting to a coronary lesion unrelated to the infarction. Two patients had concomitant tricuspid annuloplasty and 1 patient mitral valvuloplasty. Result: Surgical mortality was 37.5%(3 patients). They all had only one-vessel disease of left anterior descending artery, which made extended posterior septal wall infarction as well as anterior septal wall infarction of both ventricles. In preoperative M-mode echocardiographic study of left ventricle, they had lower ejection fraction than survivors($34.9{\pm}4.2\;vs.\;54.8{\pm}12.3$; p=0.036). Conclusion: Most of patients with postinfarction VSD had one-vessel disease of left anterior descending artery. Early surgical mortality occurred in the patients with poor ejection fraction of left ventricle and the wide anterior septal wall infarction extending to the posterior septum.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.536-538
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• 2004

There are still some controversies regarding one stage repair of pectus deformity with congenital heart disease due to probable complications after extensive resection of deformed cartilages. We performed Nuss operation with simul-taneous intracardiac repair in 8 year old patient with pectus excavatum and partial atrioventricular septal defect. The result of operation was satisfactory without prolongation of operation time, bleeding or instability of chest wall.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.620-629
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• 1989

A clinical analysis of 138 cases of ventricular septal defect operated on from 1983 to June 1988 at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Chung-nam National University was done. The following results were obtained. 1. The VSD were 27.6 % and 41.0 % respectively of 500 open heart surgery cases and 334 congenital heart disease. 2. The range of age when the VSD was repaired was 3 months to 45 years, the mean age was 10.4 years and the 53.6 % of patients were under 7 years. The sex ratio was 6:4 in males favor. 3. The two common symptoms were frequent upper respiratory infection and dyspnea whose frequency was 62.5 % and 58.7% respectively. 4. 52% of the patients was below 25 percentile of the standard body weight. 5. The most common chest PA findings were cardiomegaly and increased pulmonary vascularity. 6. On anatomical classification, perimembranous type was most common [65.5%], subpulmonary arterial type was 27.6 %, perimembranous plus subpulmonary arterial type was 3.4 % multiple VSD was 0.8 % and LV-RA defect was 2.6 %. 7. Associated cardiac anomalies were founded in 39 cases [35.5 %] and PDA and aortic valve prolapse were most common associated anomalies and extracardiac anomalies were founded in 6.7 % of patients. 8. Mean extracorporeal circulation time was 68 min. and mean aortic cross-clamping time was 42 min. 9. The postoperative complications developed in 26 % of patients and the most common one was minor wound infection. 10. Right bundle branch block developed in 54.8% of the patients who had right ventriculotomy, 40.0% of the patients who had right atriotomy and 10.5% of the patients who had pulmonary arteriotomy. 11. The overall operative mortality was 5.1 % and the operative mortality of the patients who body weigh under 10 Kg was 23.8%.