• Tuberculosis and Respiratory Diseases
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• v.43 no.1
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• pp.96-101
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• 1996

Hughes-Stovin Syndrome is an exceedingly rare combination of distal pulmonary arterial aneurysm and deep vein thrombosis, mostly found in young patients. There are striking similarities between the vascular manifestation of Behçet's disease and Hughes-Stovin Syndrome. It has been suggested that they may have a similar pathogenesis. Most patients died of massive hemoptysis due to rupture of aneurysm. Recently we have experienced the first case of Hughes-Stovin Syndrome in Korea. A 37 year old male patient was admitted because of recurrent hemoptysis and intermittent fever. He had a history of recurrent aphthous ulcers and erythema nodosum-like skin rash, But no other findings of Behcet's disease was found. Angiography showed multiple pulmonary arterial aneurysm and deep vein thrombosis in Right lower extremity. Histologic examination of specimens of open lung biopsy revealed leukocytoclastic angiitis. Pulmonary arterial aneurysms were successfully treated by coil embolization and he is in good condition with corticosteroid and cyclophosphamide therapy.

• Journal of Yeungnam Medical Science
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• v.34 no.2
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• pp.279-284
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• 2017

Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.

• Tuberculosis and Respiratory Diseases
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• v.61 no.1
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• pp.65-69
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• 2006

Behcet's disease is a systemic vasculitis of an unknown etiology involving the arteries and veins of all sizes. There are reports showing that a pulmonary artery aneurysm or thromboembolism and superior vena cava thrombosis are present in 5-10% of patients with Behcet's disease and that lung parenchymal lesions are mainly airway consolidations resulting from hemorrhage or infarction. We encountered a patient with increasing pulmonary cavitary changes and localized aspergilloma. The patient was a 43-year-old man diagnosed with Behcet's disease with a history of recurrent oro-genital ulceration and uveitis, and who was administered methotrexate, colchicines, prednisolone. During the follow up he developed progressive dyspnea upon exertion and finger clubbing. Therefore further evaluations were performed. Chest computed tomography showed more advanced consolidations and cavitations than the previous film with the previously known aspergilloma still observable. An open lung biopsy was carried out to determine the presence of malignant changes, which revealed nonspecific vasculitis. Azathioprine was added resultion in an improvement of symptoms.

• Journal of the Korean Society of Radiology
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• v.83 no.1
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• pp.70-83
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• 2022

Acute coronary syndrome (ACS) is mainly caused by atherosclerotic coronary artery disease (CAD); however, it can also occur in patients with non-atherosclerotic CAD. Conventional coronary angiography only shows the lumen of arteries, indicating the presence of stenosis or dilatation. Thus, it has limited value in evaluating the coronary artery wall and offers low specificity for diagnosing CAD. Coronary CT angiography provides additional information, including the depiction of the concerned vessel and the aorta, as well as the pulmonary artery, which permits the diagnosis of non-atherosclerotic CAD and the differentiation of various causes of the disease. In this review, we present the pathophysiology and CT imaging features of non-atherosclerotic CAD.

• Journal of Chest Surgery
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• v.31 no.2
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• pp.118-124
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• 1998

Minimally invasive coronary artery bypass grafting without using cardiopulmonary bypass (CPB) is a recently accepted modality of myocardial revascularization prcedures which is particularly suitable to the patients with lesions in the left anterior descending(LAD) and the right coronary arteries. Of the consecutive 35 patients of coronary artery bypass grafting performed at Sejong General Hospital from March to August 1996, six patients underwent minimally invasive coronary artery bypass grafting without CPB. All had stenotic lesions of the LAD more than 90%. Bypass grafting of the LAD was approached through midline sternotomy in one, through ministernotomy in two, and through limited left anterior thoracotomy in three patients, respectively. The internal mammary arteries were prepared without the use of thoracoscope. The mobilized mammary arteries were connected directly to the LAD in 5 patients, and the anastomosis required interposition of a segment of the radial artery in the remaining one. The diagonal branch was revascularized with the saphenous vein graft at the same time in one patient. No blood transfusion was necessary in 2 patients, and average blood required during surgery was 800ml in 4 patients. All patients were extubated from 4 to 14 hours(mean 9 hours) after operation. Early postoperative coronary angiography in 5 patients between 7 and 10 days after surgery has proved full patency of the grafts. With these limited clinical experiences, the clinical results demonstrated that minimally invasive coronary artery bypass grafting without CPB is an useful procedure especially in patients with isolated lesion in the proximal LAD.

• 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.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.457-462
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• 2003

Background: Surgical results of the Fontan procedures in patients with a single ventricle have improved. As the perioperative mortality continues to decline and late outcome is forthcoming, attention is now being directed toward late complications of the Fontan procedures. We retrospectively analyzed our experience with reoperations after Fontan procedures. Material and Method: Between January 1988 and December 2002, 24 patients underwent reoperations after Fontan procedures. The median age at Fontan procedures and reoperation was 3.3 years and 9.2 years, respectively. Types of initial Fontan procedures were atriopulmonary connection (n＝11), lateral tunnel Fontan (n＝11), and extracardiac conduit Fontan (n＝2). Indications for reoperation included atrioventricular valve regurgitation (n＝7), atrial arrhythmia (n＝8), Fontan pathway stenosis (n＝7), residual right-to-left shunt (n＝5), etc. Result: Procedures performed at reoperation included atrioventricular valve replacement (n＝6), conversion to lateral tunnel Fontan (n＝5), conversion to extracardiac conduit Fontan (n＝3), cryoablation of arrhythmia circuit (n＝7), etc. There was no operative mortality. There were 2 late deaths. Mean follow-up duration was 2.7$\pm$2.1 years. All patients except two were in NYHA class I at the latest follow-up. Among 8 patients with preoperative atrial arrhythmia, postoperative conversion to normal sinus rhythm was achieved in 7 patients. Conclusion: Reoperations after Fontan procedures could be achieved with low mortality and morbidity. Reoperation may lead to clinical improvement in patients with specific target conditions such as atrioventricular valve regurgitation, refractory atrial arrhythmia, or Fontan pathway stenosis, especially in patients with previous atriopulmonary connection.

• Clinical and Experimental Pediatrics
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• v.46 no.2
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• pp.154-161
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• 2003

Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, $12{\pm}9$ years; mean follow-up duration, $9{\pm}6.4$ years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. Results : Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P=0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. Conclusion : TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.305-310
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• 2009

Background: The Ross procedure is known as a good surgical option for a young age group with aortic valve problems, but few reports on the Ross procedure are available in the Korean literature. This study is a review of our midterm results of 10 year experience with the pediatric Ross operation in Asan Medical Center. Material and Method: From March 1997 to October 2008, eighteen patients who were aged less than 16 years underwent the Ross procedure. There were 11 males and 7 females. The patients median age was 8.5 years (range: $0.5\sim14.0$). The aortic valve pathophysiology was 6 patients with aortic insufficiency, 4 patients with aortic stenosis, 7 patients with mixed aortic stenoinsufficiencey and 1 patient with infective endocarditis. The valve morphology was bicuspid in 11 and tricuspid in 7. All the patients were operated on with the root replacement technique. All the pumonic valves were replaced with an allograft except for one pericardial monocusp valve. The mean follow up duration was 52.8 months (range: 5.8$\sim$138.2 months). We reviewed the echocardiographic data with focusing on the, auto-graft dysfunction and reoperation. Result: There was no hospital mortality and late mortality. According to the last echocardiographic data, 2 autografts showed aortic regurgitation grade 2, 4 autografts showed aortic regurgitation grade 1 and the others were less than trivial. Reoperation of the pulmonic position conduit was performed 4 times in three patients. The rate of freedom from reoperation at 5 years was 72.2%. On the serial follow up, the Z-values of the aortic annulus/aortic sinus were changed from $1.6{\pm}1.7/0.9{\pm}1.7$ at preoperation to $1.8{\pm}1.6$(p=0.64)/$2.2{\pm}0.9$ (p=0.01) at the last follow-up. There was no significant relation between the growth of the neoaortic root and neoaortic insufficiency. Conclusion: Our midterm results of the Ross procedure in pediatric patients showed good autograft function and growth potential. Vet reoperation due to allograft dysfunction was a major concern.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.622-628
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• 2002

Purpose : The purpose of this study is to ascertain the clinical manifestations and associated cardiac anomalies of dextrocardia. Methods : Twenty-four pediatric patients, who were admitted to, or visited, Dongsan Medical Center Keimyung University and were diagnosed with dextrocardia between January 1996 and July 2001, were enrolled in this study. All patients received echocardiogram and abdominal ultrasonogram to identify structural cardiac anomalies and visceral position. Results : Among 24 patients, 7 cases were situs solitus(29.2%), 10 cases(41.6%) were situs inversus and 7 cases(29.2%) were situs ambiguous. Most were diagnosed within a week of life(87.5%). They were most commonly cyanotic(45.8%), but eleven cases(45.8%) had no specific symptoms or signs. Nineteen cases(79.2%) had accompanied cardiac anomalies, and pulmonary stenosis or atresia were the most common among them. All the cases of situs solitus and ambiguous had associated cardiac anomalies, but half of the situs inversus had that. Eleven cases were dead by cardiac or extracardiac anomalies during follow up and the mortality rate was higher in situs solitus or ambiguous group than situs inversus group. Conclusion : Dextrocardia accompanies different cardiac and extracardiac anomalies. It's very important to diagnose dextrocardia and associated cardiac or extracardiac anomalies in the early stages of life to improve prognosis.