• Journal of Chest Surgery
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• 제11권4호
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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 Korean Neurosurgical Society
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• 제50권3호
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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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• 제59권sup1호
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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.

• Clinical and Experimental Pediatrics
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• 제60권12호
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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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• 제27권1호
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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.

• Journal of Chest Surgery
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• 제30권9호
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• pp.862-868
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• 1997

서울대학교병원 흉부외과에서는 1985년부터 1994년까지 총 9례의 숀 증후군 환자를 치험하였다. 남자 환 아가 떼이었고 여자 환아가 1례이었다. 첫 수술을 시행한 연령은 깨월에서 11세까지로 평균 연령은 33.0 $\pm$ 31.0 개월이었다. 선천성 승모판 협착증 및 대동맥 축착증은 모든 환아에서 존재하였고 승모판 상부 링 및 대동맥하 협착은 각각 4례의 환아에서 발견되었다. 2례의 환아에 있어서는 숀 증후군의 4가지 병변 모두를 가지고 있었다. 3례의 환아에 있어서는 한번에 완전 해부학적 교정술을 시행하였으며 나머지 2례의 환아에 있어서는 개흉술 하에 대동맥 축착증을 교정하고 나중에 정중 흉골 절개 하에 나머지 병변을 교정하는 단계적 수술을 시행하였다. 9례의 환아에 있어서 총 45개의 수술적 치료를 시행하여 1례당 평균 수술적 치료 는 5.0 $\pm$ 0.7개이었다 세번째 수술을 시행한 환아는 2례이었으며 각각 지속적인 승모판 협착으로 승모판 치 환술을 시행한 환아와 지속적인 대동맥 축착으로 대동맥 축착 성형술을 시행한 환아이었다. 한번에 완전 교정술을 시행한 환아와 1차 단계적 수술을 시행한 환아에 있어서 수술 사망률은 없었으나 2차 단계 \ulcorner수술을 시행한 6례중 2례가 사망하여 수술 사망률은 22.2%이었으며 사망한 2례 모두 1차 대동칵 축착증 교정술후 계속되는 심부전 및 인공 호흡기 이탈이 되지 않아 각각 1차 수술 후 9일과 15일 후에 완전 교정술을 시행했던 환아들로 모두 좌심실 형성부전에 의한 심부전으로 사망하였다. 세번째 수술시 사망한 환아는 없었다. 생존한 7례 모두 11개월에서 12년까지(평균 6.7 $\pm$ 3.6년)장기 추적 조사하였으며 모두 뉴욕 심장 협회 활 동도 1군에 속해 있었으며 판막 치환술로 인하여 쿠마딘을 복용하고 있는 환아와 마지막 추적조사시 흉부 X선 사진상 약간의 심비대가 있어 디곡신을 복용하고 있는 환아를 제외하고는 어떤 약도 복용하고 있지 않았 다 결론적으로 숀 증후군은 수술 사망률 및 이환율이 높은 질환이긴 하나 각각의 해부학적 병변의 특징을 잘 파악하여 적절한 치료를 한다면 좋은 장기성적을 기대할 수 있다는 것이다.

• Journal of Chest Surgery
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• 제39권4호
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• pp.275-280
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• 2006

배경: 대동맥폐동맥창은 매우 드문 질환이며 동반 심기형을 보이는 경우가 흔하다. 좌우 단락에 의한 과다한 폐 혈류량으로 조기 교정이 필수이나, 정확한 진단과 수술적 교정으로 좋은 결과를 얻을 수 있다. 본 연구에서는 과거 20년간의 경험한 대동맥폐동맥창을 정리하였다. 대상 및 방법: 1985년 3월부터 2005년 1월까지 16명(남 10, 여 6)의 환자가 대동맥폐동맥창으로 진단되어 수술적 교정을 시행하였고,수술당시 평균연령은 $157.8{\pm}245.3$ ($15.0{\sim}994.0$)일이었고, 평균 체중은 $4.8{\pm}±2.5$ ($1.7{\sim}10.7$) kg이었다. 동반 심기형은 동맥관개존증(8예), 심방중격결손증(7예), 대동맥궁단절증(5예), 심실중격결손증(4예), 난원공(3예), 삼첨판막역류증(3예), 승모판역류증(2예), 대동맥판역류증(1예), 대동맥축착증(1예), 좌측상대정맥(1예), 우심증(1예)이었다. 수술 방법은 대동맥폐동맥창의 분리 절단 후 대동맥 부위를 일차 봉합 또는 첩포 봉합하고 폐동맥 부위를 일차 봉합 또는 첩포 봉합하거나(11예), 대동맥폐동맥창의 분리 절단 없이 동맥 내에서 첩포 봉합술을 하거나(3예), 대동맥궁단절 및 대동맥축착을 동반한 환자에서 하대동맥을 직접 대동맥 창에 봉합하였다. (2예). 결과: 사망한 경우가 1예 있었다. 환자는 기관지 분지부에서 상방 2.5 cm정도 심한 협착과 기관유래기관지가 우상엽과 연결되어 있었던 경우로, 기관성형술(sliding tracheoplasty)시행 후 과다 출혈로 술 후 5일째 사망하였다. 복잡 대동맥폐동맥창 환자의 입원 기간 및 중환자실 체류 기간이 더 길었으며, 재수술(5예)과 합병증의 빈도도 더 높았다. 재수술은 좌폐동맥 협착(4예), 우폐동맥 협착(2예), 주폐동맥 협착(1예) 등이 원인이었다. 평균 추적 기간은 $6.8{\pm}5.6$ (57.0일$\sim$16.7년)년이었고, 생존 환자의 추적 기간 동안 NYHA 기능분류는 모두 I이었다. 결론: 연구자 등은 대동맥폐동맥창으로 진단된 16명의 환자에 대한 분석을 통해 조기 수술로 술 후 양호한 결과를 확보할 수 있음을 확인하였으며, 동반 심기형의 적절한 수술적 교정이 만기 예후를 좌우하는 것을 알 수 있었다. 수술 후 재협착의 빈도를 줄이기 위한 수술 전략의 재고가 요구된다.

• Journal of Chest Surgery
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• 제9권1호
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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.

• Journal of Chest Surgery
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• 제21권3호
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• pp.510-517
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• 1988

With the ligation of a patent ductus arteriosus by Gross in 1938, surgeons first entered the field of congenital heart disease, and treatment of the patent ductus is representative of the rapid advance made in thoracic surgery in the last 40 years. We have had clinical experiences about 36 cases of this in the department of Thoracic & Cardiovascular surgery, Pusan Paik Hospital, Inje medical college from March 1891 to June 1987. And the results were summarized as follows. 1. There were 11 males, 25 females. The age range of the patients were from 8 months to 36 years with the mean age of 7.9 years. 2. The chief complaints of the patients on admission were frequent URI[50%], dyspnea on exertion[29.8%], chest pain[11.1% k 1%], growth retardation[2%], cough[2.8%], anorexia[2.8%]. But there were 11 patients[30.6%] having no subjective symptoms. 3. In auscultation, the usual continuous machinery murmur was noticed in 30 patients[83.3%], only systolic murmur in 6[16.7%]. 4. In the preoperative chest P-A views, there were noticed cardiomegaly in 20 cases, enlarged pulmonary conus and / or pulmonary plethora in 22 patients[61.1%]. 5. In the preoperative EGG findings, there were noticed pattern of LVH in 8 patients[22.2`], RVH in 2[5.6%], BVH in 4[11.5%] and normal in 19[52.89o]. 6. The size of PDA[mean] was 9.5 mm[length] and 8.8 mm[width], the range of length was from 4 to 29 mm and the range of width was from 4 to 18 mm. 7. There were noticed 6 cases which were combined with other anomalies[VSD in 2 cases, Coarctation of aorta in 2, Mitral regurgitation in 1, and AP window in 1]. 8. On operation, simple ligation of the ductus was performed in 30 cases[83.3%], division and suture-ligation in 5[13.9%]. 9. Postoperative complications were noticed in 4 cases[pneumonia in one case, wound infection or disruption in 3], but there were no mortality.

• The Korean Journal of Physiology
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• 제29권2호
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• pp.279-289
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• 1995

This study was designed 1) to develop a hypertensive animal model in which the blood pressures (BPs) of symmetric regions (right and left upper extremities) are significantly different and 2) to test the effect of BP per se on the contractility and endothelium-dependent relaxation of vascular smooth muscle. Rabbits were anesthetized with sodium pentobarbital and ventilated with room air via animal respirator. The transverse aorta was exposed through the left second intercostal space and the lumen of the aorta was narrowed partially by ligation using 3-0 silk and a probe at a point between the origins of the brachiocephalic trunk and the left subclavian artery. Four to eight weeks postoperatively, BPs were measured in the carotid artery as the high BP area (proximal to coactation site) and in the femoral artery as the low BP area (distal to coarctation site). In the animal model, pressure-overload hypertension was developed and the BP of the right subclavian artery was higher than that of the left subclavian artery. The concentrations of circulating epinephrine, norepinephrine, angiotensin I, and angiotensin II were measured. The right and left subclavian arteries and their branches were used for isometric tension recording in organ baths and their responsiveness to phenylephrine, serotonin, acetylcholine, and sodium nitroprusside were examined. The BPs of carotid and femoral artery in control animals were $116{\pm} 12/75{\pm}9\;mmHg (mean ${\pm}SEM$) and $130{\pm}16/68{\pm}9\;mmHg$ respectively, while those of carotid and femoral artery in the hypetensive animals were $172{\pm}6/111{\pm}10\;mmHg$ and 136{\pm} 4/100 {\pm}9\;mmHg$ respectively. There were no significant differences in the concentrations of circulating epinephrine, norepinephrine, angiotensin I, and angiotensin II between controls and the animal models. No significant differences were found in the vascular sensitivities to phenylephrine and serotonin between the high pressure-exposed vessels and the low pressure-exposed vessels. However, the endothelium-dependent relaxation to acetylcholine and nitroprusside-induced relaxation showed significant differences between the high pressure-exposed and the low pressure-exposed subclavian arteries. From the above results, we suggest that the contractility of vascular smooth muscle is unchanged by the elevated pressure per se. However, the endothelium-dependent relaxation to acetylcholine and the nitroprusside-induced relaxation are attenuated by pressure.