• Journal of Chest Surgery
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• v.35 no.12
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• pp.862-870
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• 2002

The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

• Journal of Chest Surgery
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• v.39 no.9 s.266
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• pp.659-667
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• 2006

Background: Experimental studies of vascular remodeling in the pulmonary arteries have been performed actively. These models required a persistent vascular insult for intimal injury induced by chronic hypoxia, monocrotaline intoxication or chronic air embolism and characterized medial hypertrophy and neointimal formation by active synthesis of the extracellular matrix protein. The purpose of this study was to determine the pattern of pulmonary vascular remodeling after obstruction of the pulmonary vein. Material and Method: Obstruction of the right pulmonary vein with a metal clip was performed in Sprague-Dawley rats $(352{\pm}18g,\;n=10)$ to cause pulmonary vascular disease. Fifteen days later, experimental studies were done and finally the both lungs and hearts were extirpated for experimental measurement. Pulmonary arterial pressure, weight ratio of right ventricle (RV) to left ventricle (LV) and ventricular septum (S) (RV/LV +S weight ratio), and pulmonary artery morphology (percent wall thickness, %WT) were evaluated and compared with normal control groups. Result: Pulmonary hypertension $(38{\pm}12mmHg\;vs\;13{\pm}4mmHg;\;p<0.05)$ and right ventricular hypertrophy (right ventricular/left ventricular and septal weight ratio, $0.52{\pm}0.07\;vs\;0.35{\pm}0.04;\;p<0.05$) with hypertrophy of the muscular layer of the pulmonary arterial wall (percent wall thickness, $22.4{\pm}6.7%\;vs\;6.7{\pm}3.4%;\;p<0.05$) were developed by 15 days after obstruction of the pulmonary vein. Conclusion: Obstruction of the pulmonary vein developed elevation of pulmonary blood pressure and medial hypertrophy of the pulmonary artery. These results are a part of the characteristic vascular remodeling. Theses results demonstrate that obstruction of the pulmonary vein can develope not only high pulmoanry blood flow of contralateral lung but also intima injury inducing vascular remodeling.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.95-101
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• 1992

A 27 years old male developed right-sided massive, recurrent, pleural transudate. EKG and echocardiogram showed right ventricular hypertropy. Chest X-ray and concurrent perfusion lung scan, performed after enough expansion of the right lung by drainage of the effusion through small cathter, showed that perfusion defect mismatched with the roentgenographic defect, which was likely to be a high probability of pulmonary thromboembolism. By cardiac catherization and pulmonary angiography the occlusion of pulmonary veins drained from the upper and middle lobe of the right lung could be revealed. More precise cause of occlusion couldn't be clear up because thoracotomy had to have been dangerous due to severe pulmonary hypertension. So the massive reurrent effusion was treated by repeated tetracycline instilations through chest tube and he was discharged. After following up 14 months at out-patient clinic, he expired because of sudden massive hemoptysis.

• Tuberculosis and Respiratory Diseases
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• v.43 no.2
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• pp.274-279
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• 1996

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in which the primary abnormality is obliterative obstruction of pulmonary veins, especially venules. Clinicaly, we should suspect this disease in the case of congestive cardiac failure with pulmonary hypertension, chronic interstitial pulmonary edema, and normal or elevated wedge pressure on cardiac catheterization. We experience a case of pulmonary hypertension due to pulmonary veno-occlusive disease. A 55-years -old woman developed progressive dry cough and dyspnea for 3 months. Physical examination showed normal heart sounds, diffuse crackles in the whole lung fields. The liver was not palpable and pitting edema was absent. The diagnosis was made by chest HRCT, 2-D echocardiography, normal pulmonary capillary wedge pressure on cardiac catheterization, and confirmed by thoracoscopic lung biopsy. This patient was treated with vasodilator(calcium antagonist) and with mild symptomatic improvement. We reported a case of pulmonary veno-occlusive disease with review of literatures.

• Journal of Chest Surgery
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• v.38 no.10 s.255
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• pp.725-728
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• 2005

A case is described in a girl who presented with recurrent life-threatening hemoptysis at the age of 18 months, and had been diagnosed as atrial septal defect with severe cardiomegaly which was presumed to result in pulmonary vein stenosis at the age of 6 months. Closure of atrial septal defect was associated with decreased heart size and improved pulmonary venous flow. However, recurrent life-threatening hemoptysis occurred during follow-up, Computed tomography scan demonstrated left pulmonary vein stenosis and extrinsic compression of the left bronchus by multiple soft tissue density-masses. Exploratory thoracotomy revealed single stenotic left pulmonary vein, and flat left main bronchus compressed by multiple hypertrophied lymph nodes, Unexpected endotrachial tube bleeding during left hilar dissection mandated to proceed to left pneumonectomy, The patient's postoperative course was uneventful. Follow-up chest roentgenography revealed acceptable left hydrothorax without mediastinal shifting, Nevertheless, a long-term follow-up is necessary.

• Neonatal Medicine
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• v.18 no.2
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• pp.404-408
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• 2011

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.52-55
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• 2002

We report one case of an 18-day-old female patient, weighing 3.4 kg, with severe cyanosis. The diagnosis was made with only transthoracic echocardiography, which revealed cor triatriatum with an atretic small opening of fibromuscular membrane, obstructive infracardiac total anomalous pulmonary venous drainage(TAPVD), severely restrictive interatrial communication, and scanty mitral inflow and aortic forward flow. The preoperative decision-making for biventricular repair was not easy due to collapsed left heart system caused by remarkably reduced blood flow An emergent operation was performed due to severe cyanosis. All left heart structures were somewhat hypoplastic but thought to be adequate for systemic circulation. Biventricular repair was done without specific intraoperative problems. The postoperative course was uneventful. The patient has been doing well with no evidence of pulmonary vein stenosis or mitral regurgitation for 4 months after operation.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.610-614
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• 2003

Pulmonary hypertension may be associated with variable conditions such as the hyperkinetic state or pulmonary vascular obstruction. In these, stenosis of the individual pulmonary veins without any cardiac or vascular malformation is very rare. We experienced stenosis of individual pulmonary veins in a 10 months old boy who was admitted with recurrent dyspnea and cyanosis and then underwent angiogram and a lung perfusion scan.

• Tuberculosis and Respiratory Diseases
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• v.58 no.2
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• pp.174-178
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• 2005

The bronchial varices in mitral stenosis are uncommon and incidentally discovered during bronchoscopy. Although bronchial varices are primarily associated with bronchial or pulmonary disease, the bronchial vein can be dilated with increased pulmonary venous pressure secondary to mitral stenosis. The bronchial varices may present massive hemoptysis. The hemoptysis can be controlled by mitral commissurotomy or mitral valve replacement in case of mitral stenosis. We report a case of bronchial varies in a patient with severe mitral stenosis. The bronchial varices were found incidentally during bronchoscopy and they were nearly disappeared by mitral valve replacement.

• Journal of Chest Surgery
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• v.36 no.2
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• pp.73-78
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• 2003

The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. Material and Method: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8 :-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1 Result: There were 3 early and late deaths respectively Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1) In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. Conclusion: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However, more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class, Although the results were not satisfactory enough in all patients.