• Journal of Chest Surgery
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• v.26 no.12
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• pp.955-958
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• 1993

Pseudocoarctation is extremely rare and is due to elongation and kinking of aortic arch which mimiks true coarctation but has no pressure gradient across it. This state is essentially benign entity and needs no surgical intervention, but it frequently tends to progress into the aortic aneurysm that results in compressive symptom due to mass effect and unawared rupture and death.We experienced a descending aortic aneurysm secondary to pseudocoarctation. The patient was 53 year-old female presented as easy f`atiguability and facial flushing. The aortogram revealed tortuous and enlarged aorta at the level of ligamentum arteriosum. The aneurysm was resected and was end-to-end anastomosed successfully under partial cardiopulmonary bypass. The postoperative course was uneventful and the patient was discharged on postoperative 10 day.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.862-868
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• 1997

Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.

• Journal of Chest Surgery
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• v.9 no.1
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• pp.10-19
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• 1976

One hundred and seventy-four patients were treated in this Department since 1956. One hundred and fifteen patients of them were surgically treated. They were classified on the basis of the disease entity as follows; 48 case of thrombo-angiitis obliterance, 8 cases of Leriche syndrome, 12 cases of arterial embolism, 36 arterial aneurysm, 5 arterio-venous fistula, 15 arterial and venous injuries, 8 pulseless diseases, 2 coarctation of aortas, 15 varicose veins, 12 thrombophlebitis, 9 superior venacaval syndromes, 2 inferior vena caval obstructions and Raynaud's diseases. All the cases of the Burger's diseases were males, and half of them were in the fourth decades, 39 cases underwent undergone unilateral or bilateral sympathectomies. All the Leriche syndromes were males aged over fifty. Three cases out of six were suffering from diabetes mellitus. 2 cases underwent aorto-femoral bypass graft with Y-shaped dacrons. And two embolectomies were performed in 2 cases. Eight cases of arterial embolisms among 12 had mitral valvular diseases with auricular fibrillation The most common site of lodgement of emboli was femoral artery. Nine out of 14 underwent embolectomies with Fogarty catheters. There were 14 peripheral arterial aneurysms, 16 thoracic and/or abdominal aortic aneurysms, and 4 dissecting aneurysms. Most frequent cause of peripheral arterial aneurysms were external trauma. Thoracic and abdominal aortic aneurysms were non-traumatic. And four cases of the dissecting aneurysms had significant hypertension and aged over fifty. Among 5 cases of arteriovenous fistulas, 2 cases hand typical Branham's sign, and they were normalized after operation. Eight cases of pulseless disease were females and aged from three to twenty-five. Three out of them were treated surgically using dacron prosthetic grafts, but the results of the surgery were variable and not satisfactory. A case of coarctation of aorta was treated surgically with an excellent result. Fourteen out of 15 varicose veins underwent ligation of the saphenous vein system, exstirpation of the varicose veins, stripping or some combination of these methods. Two cases of superior vena caval syndromes were operated by bypass graft between the left innominate vein and the right auricle. Two cases of inferior vena caval obstructions were operated upon through right atrial route using extracorporial circulation. All the four cases of vena caval obstructions showed excellent results postoperatively. Two cases out of 12 thrombophlebitis underwent thrombectomies. One of two Raynaud's diseases was surgically treated with an excellent result.

• Clinical and Experimental Pediatrics
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• v.60 no.12
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• pp.390-394
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• 2017

Purpose: Atrioventricular nodal reentry tachycardia (AVNRT) is less common in pediatric patients than in adult patients. Thus, data for pediatric AVNRT patients are insufficient. Hence, we aimed to analyze the patient characteristics, treatment, and any recurrences in pediatric AVNRT patients. Methods: We reviewed the records of 50 pediatric AVNRT patients who had undergone radiofrequency catheter ablation (RFCA) between January 1998 and December 2016 at a single regional center. The patients were aged ${\leq}18years$. Results: Among 190 pediatric patients who underwent RFCA for tachyarrhythmia, 50 (26.3%; mean age, $13.4{\pm}2.6years$) were diagnosed as having AVNRT by electrophysiological study. Twenty-five patients (25 of 50, 50%) were male. Twenty patients (20 of 50, 40%) used beta-blockers before RFCA. All patients had no structural heart disease except 1 patient with valvular aortic stenosis and coarctation of the aorta. RFCA was performed using the anatomic approach under fluoroscopic guidance. The most common successfully ablated region was the midseptal region (25 of 50, 50%). Slow pathway (SP) ablation and SP modulation were performed in 43 and 6 patients, respectively. Complication occurred in 1 patient with complete atrioventricular block. During follow-up, 6 patients had recurrence of supraventricular tachycardia, as confirmed by electrocardiography. Among them, 5 underwent successful ablation at the first procedure. In 1 patient, induction failed during the first procedure. Conclusion: RFCA is safe and effective in pediatric AVNRT patients. However, further research is needed for establishing the endpoints of ablation in pediatric AVNRT patients and for identifying risk factors by evaluating data on AVNRT recurrence after RFCA.

• Journal of Chest Surgery
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• v.17 no.4
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• pp.657-665
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• 1984

Thirty-seven patients had undergone repair of a endocardial cushion defect between 1977 and Aug. 1983 in Seoul National University Hospital. Twenty eight had a partial defect, one intermediate defect and eight complete endocardial cushion defect. Tricuspid cleft was found in 4 cases and mitral cleft was in all p-ECD. Seven patients were of type C anatomy in c-ECD. Four patients had associated major anomalies, including three TOF in c-ECD, one coarctation in p- ECD. In p-ECD patients, the septal defect was closed with patch in all cases and the atrioventricular valvular insufficiency was corrected with MVR in 4 cases, TVR in 1 case and simple interrupted sutures in remainders. In c-ECD patients the septal defect was closed with single patch except one case. The atrioventricular valve was repaired with simple interrupted sutures except one MVR and TVR case. The operative mortality was 14.2% in p-ECD, 44.4% in c-ECD, but recent 3 years [1980-1983] mortality was 8.7% in p-ECD, 20% in c-ECD. More than grade III systolic regurgitant murmur was oted postoperatively in 4 cases of c-ECD and 3 cases of p-ECD. The operative risk factors were preoperative NYHA classification, cyanosis, Rp/Rs, systolic pressure of main pulmonary artery and the degree of regurgitation of atrioventricular valves. The causes of death were low cardiac output syndromes, pulmonary complications and arrhythmias.

• Journal of Chest Surgery
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• v.20 no.2
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• pp.404-410
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• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Journal of Korean Neurosurgical Society
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• v.50 no.3
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• pp.256-259
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• 2011

We report a rare case of Williams syndrome accompanying moyamoya disease in whom postoperative global cerebral infarction occurred unpredictably. Williams syndrome is an uncommon hereditary disorder associated with the connective tissue abnormalities and cardiovascular disease. To our knowledge, our case report is the second case of Williams syndrome accompanying moyamoya disease. A 9-year-old boy was presented with right hemiparesis after second operation for coarctation of aorta. He was diagnosed as having Williams syndrome at the age of 1 year. Brain MRI showed left cerebral cortical infarction, and angiography showed severe stenosis of bilateral internal carotid arteries and moyamoya vessels. To reduce the risk of furthermore cerebral infarction, we performed indirect anastomosis successfully. Postoperatively, the patient recovered well, but at postoperative third day, without any unusual predictive abnormal findings the patient's pupils were suddenly dilated. Brain CT showed the global cerebral infarction. Despite of vigorous treatment, the patient was not recovered and fell in brain death one week later. We suggest that in this kind of labile patient with Williams syndrome accompanying moyamoya disease, postoperative sedation should be done with more thorough strict patient monitoring than usual moyamoya patients. Also, we should decide the revascularization surgery more cautiously than usual moyamoya disease. The possibility of unpredictable postoperative ischemic complication should be kept in mind.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.84-87
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• 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

• Journal of Chest Surgery
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• v.11 no.4
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• pp.529-534
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• 1978

Recent advances in the surgical treatment of congenital disorders of the heart have necessitated an accurate preoperative diagnosis. Right heart catheterization has become widely accepted as a research tool and diagnostic test to detect the heart diseases, especially in the congenital heart anomalies. Right heart catheterizations were carried out in 50 cases of congenital heart diseases at Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital, during the period of June, 1975 through September 1978. In age distribution, 27cases were below 10 years of age, 18 cases between 11 and 20, and 5 cases above 20 male to female ratio was 2.8:1. The distribution of congenital heart diseases was VSD [42%], TOF [36%], PDA [10%], ASD [8%], and PS[4%]. Of these, 44 cases [88%], were compatible with the clinical impressions that were made preliminarily before cardiac catheterization, and all the cases except 1 case of VSD was correlated well with the postoperative diagnosis. The right heart catheterization is considered to be reliable and accurate toll in the preoperative diagnosis of congenital heart diseases. These procedures caused complications such as left side hemiplegia [lcase], occlusion of the femoral artery [lcase], and transient ventricular tachycardia [1case], and so the complication rate of right heart catheterization was 6% [3 cases]. None of patients who have undergone right heart catheterization was died.

• Journal of Chest Surgery
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• v.27 no.1
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• pp.24-30
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• 1994

Eighteen infants with a large ventricular septal defect[VSD] underwent primary surgical repair from January 1986 to December 1992. Operation was done because of failure to thrive, medically intractable heart failure, recurrent pneumonia, increased pulmonary vascular resistance[PVR]. Four patients[22.2%] died in the early postoperative period. Relief of heart failure and normalization of growth and weight gain was evident in all survivor. There was no late postoperative death. The results of primary surgical repair of VSD in infancy are compared with those of palliative pulmonary artery banding[PAB] and of VSD closure after PAB. Twenty-seven patients with isolated VSD or with VSD associated with atrial septal defect, patent ductus arteriosus, or coarctation of the aorta underwent initial palliative PAB. There were 3 early postoperative deaths[11.1%]. Severe elevation of PVR persisted in two patients. Closure of VSD and pulmonary artery debanding was done in twenty patients, with 2 early postoperative deaths[10.0%]. Placement of the PAB too close to the pulmonary annulus necessitated trasannular patching in one patient, but any problem caused by migration of the band was not developed. It is concluded that primary surgical repair of VSD in infancy is reasonable and that PAB is indicated only for those patients less than 6 months old with a complicated defect or in an emergency situation.