• Journal of Chest Surgery
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• v.53 no.1
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• pp.16-21
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• 2020

Background: Patients undergoing cardiac surgery require postoperative chest drainage. However, the drain is difficult to keep in place in children with congenital heart disease. Since 2015, at Kyungpook National University Hospital, the chest tube is removed on postoperative day 1 in patients who have undergone simple congenital cardiac surgery (i.e., closure of an atrial or ventricular septal defect). In this study, we evaluated the relationship between the duration of drain placement and the likelihood of pericardial effusion after congenital cardiac surgery. Methods: The medical records of patients who underwent closure of an atrial or ventricular septal defect at our hospital between January 2014 and December 2016 were reviewed. In total, 162 patients who received follow-up echocardiography and had information available on postoperative pericardial effusion after the repair procedure were enrolled. Results: Echocardiography was performed at a median of 5 days (range, 4 to 6 days) postoperatively before discharge from the hospital. Pericardial effusion occurred in 21 patients (13.0%), of whom only 3 (1.9%) had moderate or greater pericardial effusion, regardless of the drain duration. All patients improved during outpatient follow-up without invasive management. No patient had severe complications because of pericardial effusion. The duration of drain placement did not affect the incidence of postoperative pericardial effusion (p=0.069). Operative survival was 100%. Conclusion: Based on our study, we recommend removing the drain as soon as its role is complete, generally on postoperative day 1, because early removal does not increase the incidence of pericardial effusion in patients undergoing simple congenital cardiac surgery.

• Journal of Chest Surgery
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• v.25 no.4
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• pp.383-390
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• 1992

From Oct. 1983 to Dec. 1991, 135 cases of atrial septal defect which were operated at the department of Thoracic and Cardiovascualr Surgery, Chonnam National University Hospital, were analysed retrospectively. They were 23.7% of all congenital heart diseases operated in the same period. Among the 135 cases, 62 cases were male and 73 cases were female. Their ages were ranged from 2 to 54 years and the mean was 18 years old. Main symptoms at admission were exertional dyspnea[70.37%], frequent URI[49.12%] and palpitation[32.59%], but 12 cases[8.88%] were asymptomatic. Electrocardiographic findings wer regular sinus rhythm in 96.99%, RVH in 64.66%, incomplete RBBB in 27.06%, complete RBBB in 42.10%, and first degree AV block in 9.02% All 135 cases were operated under the direct vision with cardiopulmonary bypass. Anatomically, most frequent type was fossa ovalis defect with complete septal rim[78.52%]. 117 of 135 ASD patients were repaired with pathch closure[86.66%] and 14 patients were repaired with direct closure[10.37%] and 4 patients in mutiple ASD were repaired with patch and direct closure[2.96%]. Postoperative complications were occured in 21 cases[15.56%], and they were wound infection, pleural effusion, postoperative bleeding, urinary tract infection, and heart failure mainly. One case died due to epidural hematoma and operative mortality was 0.74%.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.122-126
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• 2014

Coronary vasospasm is one of the fatal complications that may occur in patients undergoing open heart surgery. To date, however, there are not many cases in this series and no definite pathophysiology has been documented. We experienced a case of coronary artery vasospasm after atrial septal defect (ASD) surgery and then successfully treated it with both transbrachial intraaortic balloon pump and percutaneous cardiopulmonary support. Only several hours after ASD surgery, the patient exhibited the cardiovascular collapse, the ST-segment elevation, followed by ventricular fibrillation and normal coronary angiography findings. It is important to make a differential diagnosis of coronary artery vasospasm in patients presenting with ST-segment elevation who had no notable coronary artery diseases. This case indicates that clinicians should be aware of the possibility that the coronary artery vasospasm may also occur in patients undergoing ASD surgery.

• Journal of Genetic Medicine
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• v.15 no.1
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• pp.20-23
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• 2018

Point mutations in the human cardiac homeobox gene NKX2.5 are associated with familial atrial septal defect (ASD), atrioventricular (AV) conduction disturbance, as well as sudden cardiac death. To date, more than 60 NKX2.5 mutations have been documented, but there are no reports in Korea. We are reporting the first Korean family with ASD and AV block associated with a novel mutation in the NKX2.5 coding region. A 9-year-old boy presented with a slow and irregular pulse, and was diagnosed with secundum ASD and first degree AV block. The boy's father, who had a history of ASD correction surgery, presented with second degree AV block and atrial fibrillation. The boy's brother was also found to have secundum ASD and first degree AV block. There were two sudden deaths in the family. Genetic testing revealed a novel mutation of NKX2.5 in all affected members of the family.

• Journal of Chest Surgery
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• v.24 no.5
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• pp.475-479
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• 1991

Tetralogy of Fallot associated with atrioventricular canal defect is rare congenital anomaly. Because of complexity of the surgical corrections of two associated anomalies, the mortality of surgery has been high. We have experienced a case of the tetralogy of Fallot with atrioventricular canal defect in a 9-year-old boy of Down`s syndrome, and the anomalies were totally corrected with good result. Single Dacron patch was placed to close the ventricular septal defect and the pericardial patch for atrial septal defect. The right ventricular outflow tract was widened by infundibulectomy and pulmonary valvulotomy followed by Goretex patch in right ventricular outflow tract.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.917-929
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• 2006

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

• Journal of Chest Surgery
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• v.24 no.5
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• pp.445-450
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• 1991

Forty eight patients diagnosed as atrial septal defect, had been operated from April 1986 to December 1990 at the Department of Thoracic and Cardiovascular Surgery, Inha University Hospital, were analysed retrospectively. Of the 48 patients, 12 patients, were male and 36 patients were female, Their ranged from 8 months to 51 years old, and the mean was 18.0 years old. The proceeding two symptoms were exertional dyspnea and frequent upper respiratory infection. But 10 patients [20.8%] were asymptomatic. Electrocardiographic findings were regular sinus rhythm in 100.0%, RVH in 29.2%, incomplete RBBB in 27.1%, and first degree AV block in 2.1%. Hemodynamic studies were performed in 38 of 48 patients. Significant pulmonary hypertension[over 50mmHg in systolic pulmonary arterial pressure], which is the most serious risk factor, was developed in 4 patients[8.3%]. There was statistical significance between size of defect[cm2/BSA-M2] and systolic pulmonary arterial pressure[sPAP] retrospectively[p=0.036]. We could not find the correlationship between age and Qs % size. Anatomically, secundum type constituted 97.9%, in which 43 patients were PFO type[91.6%] and 3 patients were IVC type[6.3%]. 38 of 48 ASD patients were repaired with patch closure[72.9%] and remains were repaired with direct closure[27.1%]. The operative result was excellent except two cases of transient postoperative arrhythmia. There was no operative mortality.

• 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.31 no.12
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• pp.1230-1233
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• 1998

The Cox-Maze procedure was developed as a cure for atrial fibrillation. The recovery rate of both atrial contractility is reported low in the atrial fibrillation associated with mitral valvular heart disease than that of loan atrial fibrillation. We performed the Cox-Maze procedure (Maze III) in three cases who suffered from non-mitral heart diseases associated with atrial fibrillation: A ruptured sinus of Valsalva aneurysm, a ventricular septal defect, and an aortic stenoinsufficiency. The Cox-Maze procedure was performed concomitantly with correction of the underlying heart disease. Conversion to sinus rhythm was achieved in all three patients, and both right and left atrial mechanical activities could be identified echocardiographically after three postoperative months.

• Journal of Chest Surgery
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• v.17 no.2
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• pp.250-256
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• 1984

Eisenmenger syndrome is a condition which systemic arterial blood oxygen unsaturation occurs if obstruction in the pulmonary capillaries raises the pulmonary vascular resistance and pulmonary arterial pressure to or beyond systemic levels and then a significant right to left shunt develops across a preexisting cardiac septal defect or an aortopulmonary communication-We have experienced 3 cases of similar condition. Case I is 24 year old man who has had cyanosis and dyspnea on exertion since childhood. His pulmonary arterial pressure was 110/80mmHg. He was operated under diagnosis of the mitral stenosis and tetralogy of Fallot, but it was finally discovered that he had patent ductus arteriosus and ventricular defect was closed with perforated prosthetic patch, but the patient expired due to right heart failure low cardiac output. Case II was 16 year old female who had pulmonary hypertension of 110/85mmHg. She was diagnosed as Eisenmenger syndrome combining with atrial septal defect and patent ductus arteriosus. Case III was 20 year old male. His pulmonary arterial pressure was 110/70mmHg and the underlying defect was patent ductus arteriosus.