• Journal of Chest Surgery
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• v.17 no.1
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• pp.118-124
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• 1984

Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

• Journal of Chest Surgery
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• v.42 no.1
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• pp.87-91
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• 2009

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

• Journal of Chest Surgery
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• v.42 no.2
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• pp.244-247
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• 2009

A left atrial appendage aneurysm is a very rare medical condition which can develop by an inflammatory reaction or a degenerative change. If there is no accompanying anomaly, a left atrial appendage is considered a congenital disease. The majority of left atrial appendage aneurysms are detected incidentally because they usually do not cause any symptoms. Surgery is indicated, even for asymptomatic patients, because of the risk of life-threatening complications, such as atrial fibrillation, supraventricular tachycardia, systemic embolization, and cardiac arrest. Left atrial appendage aneurysms are usually treated by a median sternotomy with extracorporeal circulation, especially if the aneurysm has a broad base or contains a thrombus, but can treated by thoracotomy without extracorporeal circulation. We report a case of a successfully treated left atrial appendage aneurysm that was misdiagnosed as a partial pericardial defect without extracorporeal circulation in a 13-year old child.

• Journal of Chest Surgery
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• v.26 no.3
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• pp.163-169
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• 1993

This is the result of the annual statistic analysis of thoracic and cardiovascular surgical cases in 1992 Korea. Overall 17, 520 cases of surgery [11, 732 cases of thoracic surgery by 54 institutes / 5, 788 cases of cardiovascular surgery by 48 institutes] were done. 1. Tumor [N=2, 532] : Lung was the most frequently involved organ by tumor [54.9%],and the remainders were mediastinum [16.2%] / esophagus [14.8%] / chest wall [11.7%] / tracheobronchus [1.3%] / pleura [1.1%] in order. Of 1, 082 cases of primary lung cancer surgery,the frequency of cell type was squamous [62.6%] / adeno [21.6%] / small cell [7.1%] / large cell [2.7%]. Of 411 cases of mediastinal tumor surgery,the frequency of cell type was neurogenic [28.8%] / thymoma [27.6%] / teratoma [17.7%] / congenital cystic [17.2%]. Of 376 cases of esophageal tumor surgery,primary cancer were the most [85.4%]. 2. Infection [N=3, 157] : Pleura was the most frequently involved organ [59.0%],and the remainders were lung [31.3%] / chest wall [8.6%] / mediastinum [1.1%] in order. 3. Miscellaneous [N=6, 043] : Lung and pleural disease esp. pneumothorax [85.1%] was the most frequent surgical indication. The remainders were chest wall anomaly [3.4%] / benign esophageal disease [3.4%] / diaphragmatic pathology [2.4%] / myasthenia [1.4%] in order. Of 85 cases of thymectomy for myasthenia gravis,thymoma was noted in 58.8%. 1. Congenital heart disease [N=3, 363] : The ratio of noncyanotic to cyanotic heart disease was 3:1. Of 2, 516 cases of noncyanotic heart disease,the frequency of disease entity was VSD [44.1%] / ASD [26.0%] / PDA [19.4%] / PS [3.3%],and that of 847 cases of cyanotic heart disease was TOF [29.4%] / ECD [15.6%] / TGA [9.7%] / DORV [7.6%]. Overall mortalities were 2.1% in noncyanotic and 12.2% in cyanotic heart surgery. 2. Acquired heart disease [N=1, 929] : Of 1, 422 cases of valvular surgery,single mitral pathology was the most frequent candidate [48.0%],and total 1, 574 prosthetic valves which were mainly mechanical [95.6%] were used. Of 376 cases of coronary surgery,triple vessel was the most [35.9%],and the frequency of bypassing grafts was great saphenous vein [52.9%] / internal mammary artery [44.7%] / artificial vessel [2.4%]. Overall mortalities were 3.4% in valvular and 4.5% in coronary surgery. 3. Pericardium,Cardiac tumor,Arrhythmia,Aortic aneurysm,Assist device,and Pacemaker : There were no specific changes compared to previous survey1]. This nation-wide inquiry will be continued and reported annually by KTCS Society.

• Journal of Chest Surgery
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• v.37 no.7
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• pp.578-584
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• 2004

Sinus of Valsalva aneurysm is a rare cardiac anomaly and a long-term survival after surgical treatment has not been well established. This study was designed to evaluate the long-term surgical results after the repair of sinus Valsalva aneurysm. Material and Method: From April 1991 to November 2003, 35 patients (23 male, 12 female, mean age 35.2 years, range 11∼64) underwent operation for sinus of Valsalva aneurysm. Twenty six patients (74.3%) were in the New York Heart Association (NYHA) class III∼IV before surgery. In preoperative echocardiogram, mean EF was 63.32 $\pm$ 11.43% and nine patients (25.7%) were in AR grade III∼IV. Direct closure, patch closure of ruptured sinus Valsalva were performed in fourteen patients (46.7%), sixteen patients (53.3%) respectively. Aortic valve replacement, valvuloplasty were performed in five patients (14.3%), three patients (8.6%) respectively. Three patients (8.6%) underwent the Bentall procedure. Concomitant procedures were performed in 15 patients (42.9%), which were closure of VSD and ASD. Mean CPB time and ACC time were 116.79 $\pm$ 38.79 and 81.2 $\pm$ 28.97 minutes. Result: There was no operative mortality. One patient (2.9%) developed complete heart block that required a permanent pacemaker implantation. Three patients (8.6%) required reoperation due to a recurred rupture of the sinus Valsalva aneurysm and developed aortic insufficiency. Mean follow-up time was 58.55 $\pm$ 38.38 months. There was one late death. Actuarial 5 year freedom rate from reoperation was 87.1 $\pm$ 7%. Conclusion: Surgical treatment for sinus of Valsalva aneurysm is safe and has satisfactory long-term results.

• Tuberculosis and Respiratory Diseases
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• v.71 no.2
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• pp.126-133
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• 2011

Unilateral pulmonary artery hypoplasia (UPAH) is a rare disease in adults and is frequently accompanied by a congenital cardiac anomaly at a young age. The diagnosis is usually based on computed tomography (CT), angiography, and magnetic resonance imaging (MRI). However, no reports are available on retrograde flow in patients with UPAH. We describe a 68-year-old man with isolated UPAH and retrograde blood flow. He was admitted for dyspnea on exertion for the past 23 years. His diagnosis was delayed, as his symptoms and signs mimicked his underlying pulmonary diseases, such as emphysema and previous tuberculous pleurisy sequelae. A discrepancy was detected between the results of a ventilation-perfusion scan and the CT image. This was resolved by MRI, which showed retrograde blood flow from the right to the left pulmonary artery. Using MRI, we diagnosed this patient with isolated pulmonary artery hypoplasia and retrograde flow.

• Journal of Chest Surgery
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• v.31 no.2
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• pp.168-172
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• 1998

Inferior sinus venosus defect is a rare lesion in which there is a large interatrial communication adjacent to the atrial connection of the inferior caval vein. The defect is located posteriorly and inferiorly, outside the confines of the true atrial septum, and partial anomalous pulmonary venous connections are the rule. We underwent surgical repair in four patients with inferior sinus venosus defect and partial anomalous pulmonary venous return. There were three males and one female with an age range from four months to 25 years. A cross- sectional echocardiogram and cardiac catheterization had been performed preoperatively in all patients, but the correct diagnosis had been made in only one case. Surgical repair was indicated due to congestive heart failure, and one patient of 4-month-old needed urgent operation. The repair was accomplished by suturing a untreated autologous pericardial patch to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium. The preoperative knowledge of the unusual anatomy allows the surgeon to repair the anomaly without difficulties. For the patients in whom interatrial communication and anomalous pulmonary venous return are suggested, surgeon has to pay careful attention to the anatomical landmarks to avoid incorrect placement of the patch.

• Journal of Chest Surgery
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• v.33 no.6
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• pp.476-486
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• 2000

Background: Pulmonary atresia with intact ventricular septum(PA/IVS) is an anatomically heterogenous anomaly with a variety of surgical strategies possible. The purpose of the study is to evaluate the influence of right ventricular size on the early and midterm results of surgical repair of PA/IVS. Material and method: Medical records of 20 consecutive patients with PA/IVS operated on between January 1993 and August 1999 were retrospectively reviewed. There were 12 boys and 8 girls whose ages ranged from 2 days to 14.5 months (median 6 days). Their body weight ranged from 2.52kg to 9.35 kg(median 3.18kg). The preoperative Z-value of the diameter of the tricuspid valve(T-valve) was less than or -4 in 5 patients, between -4 and -2 in 1, between -2 and 0 in 7, between 0 and 2 in 6, and greater than or 2 in 1. All patients who had z-value of tricuspid valve greater than -2.05 were attempted biventricular repair(n=15) and all patients who had it smaller than -4.4 underwent systemic-pulmonary shunt operation only(n=3) or bidirectinal cavopulmonary shunt with right ventricular reconstruction(n=2). Result: Two early deaths(2/20, 10%) occurred. Both were infants who underwent transannular patch with shunt. One of these two had huge right ventricle(Z-value of tricuspid valve = 5). There were 2 late non-cardiac deaths 3 and 7 months after operations respectively. Follow-up was completed in all children at a mean of 35.3 months(range, 5 to 54 months). 10 of 11 survivors who underwent transannular patch or valvotomy with or without shunt procedure were in NYHA functional class I even though some of them had small interatrial communication or patent shunt. All three patients who had shunt procedure only at initial palliation completed Fontan procedures with no death. Two patients who underwent right ventricular outflow reconstruction with bidirectional cavopulmonary shunt were also in good condition. Conclusion: The transanular RVOT patch or valvotomy with or without systemic-pumonary shunt as an initial palliative procedure to achieve biventricular repair for the patients who had neither too small nor too large right ventricle(-2.05$\leq$Z-value of T-value of T-valve$\leq$2) could be performed at low operative risk(1/14 7.1%). Systemic-pulmonary shunt procedure and bidirectional cavopulmonary shunt procedure for the patients who had small right ventricle(Z-value of T-valve$\leq$4.4) could be also performed with low risk. But a patient with huge right atrium and ventricle(Z-value of t-valve=5) had poor operative result.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.569-577
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• 1986

Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.

• Journal of Chest Surgery
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• v.33 no.4
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• pp.290-300
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• 2000

Background: To evaluate the diagnostic accuracy of EBT(Electron Beam Tomography) in the diagnosis of conotruncal anomaly and to determine whether it can be used as a substitute for cardiac angiography. Material and Method: 20 patients(11M & 9F) with TOF(n=7, pulmonary atresia 2), DORV(n=7), complete TGV(n=4), & corrected TGV(n=2) were included. The age ranged from 7 days to 26 years(median 60 days). We analyzed the sequential chamber localization, the main surgical concenrn in each disease category (PA size, LVED volume and coronary artery pattern for TOF & pulmonary atresia, the LV mass, LVOT obstruction, coronary artery pattern for complete TGV, and type of VSD and TV-PV distance for DORV, etc) and other associated anomalies(e.g., VSD, arch anomalies, tracheal stenosis, etc). Those were compared with the results of echocardiography(n=19), angiography (n=9), and surgery(n=11). The interval between EBT and echocardiography/angiography was within 20/11 days, respectively except for an angiography in a patient with corrected TGV (48 days). Result: EBT correctly diagnosed the basic components of conotruncal anomalies in all subjects, compared to echocardiography, angiography or surgery. These included the presence, type and size of VSD(n=20), pulmonic/LV outflow tract stenosis(n=15/2), relation of great arteries and the pattern of the proximal epicardial coronary arteries(16 out of 20). EBT proved to be accurate in quantitation of the intrapericardial and hilar pulmonary arterial dimension and showed high correlation and no difference compared with echocardiography, angiography, or surgery(p>0.05) except for left pulmonary arterial & ascending arterial dimension by echocardiography. LVED volume in seven TOF(no difference: p>0.05 & high correlation: r=0.996 with echocardiography), and LV mass in 4 complete TGV were obtained. Additionally, EBT enabled the cdiagnosis of subjlottic tracheal stenosis and tracheal bronchus in 1 respectively. Some peripheral PA stenosis were not detected by echocardiography, while echocardiography appeared to be slightly more accurate than EBT in detecing ASD or PDA. Conclusion: EBT can be a non-invasive and accurate modality of for the evaluation of most anatomical alteration including peripheral PS or interruption in patients with conotruncal anomalies. Combined with echocardiography, EBT study provides sufficient information for the palliative or total repair of anomalies.