• Korean Journal of Veterinary Research
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• v.57 no.1
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• pp.63-66
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• 2017

A 6-month-old mature intact female Siamese cat presented with exertional dyspnea. Diagnostic studies revealed pleural effusion, grade 4/6 left basal systolic murmur, deep S-wave in electrocardiograph leads I, II, and III, cardiomegaly with pleural effusion on radiography, pulmonic systolic (~5.8 m/sec) and tricuspid (3.6 m/sec) regurgitant jets, atrial septal defect, and a hypoplastic right outflow tract. Based on these results, the case was diagnosed as pulmonic stenosis with atrial septal defect. To the best of our knowledge, this is the first case report describing pulmonic stenosis with atrial septal defect in a cat in Korea.

• Journal of Korean Neurosurgical Society
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• v.57 no.1
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• pp.68-71
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• 2015

We report a case of non communicating hydrocephalus due to membranous obstruction of Magendie's foramen. A 37-year-old woman presented with intracranial hypertension symptoms caused by the occlusion of Magendie's foramen by a membrane probably due to arachnoiditis. As far as the patient's past medical history is concerned, an Epstein-Barr virus infectious mononucleosis was described. Fundoscopic examination revealed bilateral papilledema. Brain magnetic resonance imaging demonstrated a significant ventricular dilatation of all ventricles and turbulent flow of cerebelospinal fluid (CSF) in the fourth ventricle as well as back flow of CSF through the Monro's foramen to the lateral ventricles. The patient underwent a suboccipital craniotomy with C1 laminectomy. An occlusion of Magendie's foramen by a thickened membrane was recognized and it was incised and removed. We confirm the existence of hydrocephalus caused by fourth ventricle outflow obstruction by a membrane. The nature of this rare entity is difficult to demonstrate because of the complex morphology of the fourth ventricle. Treatment with surgical exploration and incision of the thickened membrane proved to be a reliable method of treatment without the necessity of endoscopic third ventriculostomy or catheter placement.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.362-370
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• 1984

The term corrected transposition of great arteries [hereafter referred to as corrected TGA] of the heart in which there is both a discordant atrio-ventricular relationship and transposition of the great vessels. Usually situs solitus is present, while the ventricles are inverted showing an l -loop. The great vessels are transposed and in the l-position so that the pulmonary artery arises from the right-sided morphological left ventricle and the anteriorly l- transposed aorta arises from the left-sided morphological right ventricle yielding an SLL pattern. In the majority of cases, associated lesions are common. The most frequent are ventricular septal defect, obstruction to the pulmonary outflow tract, tricuspid valve incompetence and atrio-ventricular conduction abnormalities. In the rare cases, no associated conditions are present and hemodynamic pathways are normal. In the report, we present one case of a 20 year-old male having corrected TGA associated with severe tricuspid valve incompetence, was corrected by tricuspid valve replacement, directly developed a supra-ventricular tachycardia but was controlled by calcium-entry blocker, verapamil, successfully.

• Journal of Chest Surgery
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• v.39 no.11 s.268
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• pp.850-853
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• 2006

The VSD in TOF is usually large and unrestrictive with an equal to or greater than that of the aortic annulus. Typically shunting through the VSD is bidirectional or right-to-left component. Restrictive VSD in TOF caused by ingrowing fibrotic tissue is very rare. We report a case of restrictive VSD and LVOTO in TOF caused by ingrowing fibrotic tissue with the review of literature.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.660-667
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• 1998

Background: This study is to evaluate the effectiveness and application of Lecompte procedure as a treatment for various complex cardiac anomalies with pulmonary outflow tract obstruction. Methods: Between July 1988 and December 1997, 44 patients underwent Lecompte procedure in Seoul National University Children's Hospital. The male to female ratio was 24 to 20 and the mean age was 29.2 months(range, 3 to 83). Of these patients, 28(63.6%) had transposition of great arteries with ventricular septal defect and pulmonary stenosis(or pulmonary atresia), 14(31.8%) had double outlet right ventricle with pulmonary stenosis(or pulmonary atresia), and so on. The principles of the technique are 1) extension of the ventricular septal defect or conal resection, 2) construction of a intracardiac tunnel connecting the left ventricle to the aorta, and 3) direct connection, without a prosthetic conduit, of the pulmonary trunk to the right ventricle. Results: There were 3 in-hospital deaths and their causes were sustained hypoxia, myocardial failure, and sepsis, respectively. There was 1 late death due to sepsis. Reoperations were performed in 6 patients who had pulmonary outflow tract obstructions(4 cases), residual muscular ventricular septal defect(1 case), and recurrent septic vegetation(1 case). The cumulative survival rates by the Kaplan-Meier method were 92.7%, 92.7%, and 92.7% at 1, 2, and over 4 years. The reoperation free survival rates were 92.7%, 92.7%, and 70.2% at 1, 3, and over 5 years. Among the risk factors for the operative death, aortic cross clamping time had statistical significance(p<0.05) and all the risk factors for the recurrent pulmonary stenosis such as age, pulmonary artery index, and materials used for the pulmonary outflow tract reconstruction had no statistical significance(p>0.05). Conclusions: Our review suggests that Lecompte procedure is an effective treatment modality for various complex cardiac anomalies with pulmonary outflow tract obstruction. Repair in early age is possible and the rates of mortality and morbidity are also acceptable.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.649-655
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• 1988

One hundred and one patients with tetralogy of Fallot who were older than 16 years of age underwent a total correction of the anomaly between May, 1964 and July, 1987. This group comprised 14.9% of the 679 consecutive patients who had repair of the tetralogy at our institution during the same period. Of the 101 patients, 8 had a previous shunt procedure for palliation. The preoperative mean hemoglobin value was 16.9*1.0% and the mean systemic oxygen saturation, 84.4*0.9%. In 76 patients[75.2%], a type II ventricular septal defect was seen whereas in 14 patients[13.9%], the defect was type I. In 72 patients[71.3%], other cardiac anomalies were present which included patent foramen ovale in 37.6%, atrial septal defect in 8.99b, vegetations in 6.9%, right sided aortic arch in 5.9% and coronary artery anomaly in 5.0%. The right ventricular outflow obstruction was caused most commonly by combination of infundibular and valvular stenosis[74.3%], followed by isolated infundibular stenosis[19.8%] and valvular stenosis [5.9%] alone in order. The preoperative mean diameter of the pulmonary valve ring size was 10.2*0.5 mm in diameter. A transannular patch enlargement of the right ventricular outflow tract was performed in 28 patients and, in 12 a pericardial monocusp was utilized. Major anomalous aorto-pulmonary vessels were encountered in 5 patients which were detected before or during the operation. In 3 patients, they were ligated beforehand to control the flooding of the operative field. Postoperatively, the mean systolic pressure gradient between the right ventricle and the main pulmonary artery was 16.2*2.3 mmHg and the mean systolic pressure- ratio between the right and the left ventricle was 45.3*2.0%. Perioperative complications including bleeding in 8.9%, pleural effusion in 7.9%, dysrrhythmia in 4.9%, and residual VSD in 4.0%. Operative mortality was 8.9%. There has been no operative death in the recent 65 cases since 1981. There were 2 late deaths, 68 and 113 months after surgery. There were 2 late detachment of the VSD patch during the follow-up period. Of the 6 patients with patch detachment found during the postoperative period, 3 had subacute bacterial endocarditis before or after the operation indicating The serious nature of this complication. Two of these patients subsequently underwent a successful reoperation.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.153-160
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• 1991

Tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 91 of 250 patients treated surgically [including redo operations] between April 1986 and December 1989. Their age ranged from 6 months to 14 years [mean 39.7 months]. Associated cardiovascular anomalies were right aortic arch [n=22], ASD [n=12], PDA [n=5], persistent left SVC [n=5], and others [n=6]. PA index was measured pre-operatively since 1987 to estimate pulmonary artery size and safe total correction[mean 289$\pm$110mm2/BSA]. Eight patients received previous shunt take down procedure concomitantly. Pulmonary arteriotomy was extended through small pulmonary annulus to a minimal distance upon the right ventricular infundibulum and transannular patch was applied in 38 patients [41.3%], in 31 of them monocuspid patch was utilized. pRV/LV was measured at operation room in 77 patients [mean 0.58$\pm$0.36]. Operative mortality was 6.6% [6/91]. The causes of death were low cardiac output [n=5], arrhythmia[n=1] and respiratory failure [n=1]. At follow-up between 12 months and 57 months [mean 30.8 months] most patients were in New York Heart Association class I without cardiac medication. There was no late death, but reoperations were required in 3 patients to relieve residual right ventricular outflow obstruction. Thus successful repair of tetralogy of Fallot can be accomplished in most patients including infants by transatrial-transpulmonary approach and the better result can be anticipated with respect to postoperative right ventricular function and arrhythmia than the conventional transventricular approach.

• Journal of Chest Surgery
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• v.16 no.1
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• pp.40-48
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• 1983

Tetralogy of Fallot is characterized by the interventricular septal defect associated with obstruction of the right ventricular outflow. The importance of the latter anatomic malformation was clinically evaluated according to the angiographic measurements of the pulmonary arteries along with the pulmonary valve annulus. Seventy of a total 76 patients operated on during a whole year of 1981 were the patients for clinical evaluation. Fifteen patients died within 1 month after operation with the operative mortality of 21.4%. The young age and the severity of pulmonary arterial hypoplasia were ones among the surgical risk factors at a total corrective surgery of tetralogy. Preoperative angiographic measurements of the pulmonary arteries to speculate the expected postoperative ratio between the left ventricular and the right ventricular pressures were retrospectively calculated according to the formula. The predicted values of P RV/LV greater than 0.5 carried apparenliy higher complication and mortality rates than the group of P RV/LV less then 0.5. The selection of the candidates for either a total correction or the staged operation In tetralogy of Fallot can be evaluated on the preoperative angiographic measurements and the expected Improvements of the clinical results were discussed.

• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.101-106
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• 2013

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

• Clinical and Experimental Pediatrics
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• v.57 no.1
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• pp.19-25
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• 2014

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.