• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.919-924
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• 2007

Pulmonary venous stenosis may be congenital or acquired. Regardless of its origin, the prognosis for patients affected with PVS remains poor. There have been many attempts to palliate PVS with little success. This report describes two patients with PVS which became evident after repair of total anomalous pulmonary venous connection. Intravascular stents were successfully implanted, but progressive restenoses in the stents occurred and eventually both of the patients died. The pertinent literature is reviewed.

• Journal of Chest Surgery
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• v.39 no.5 s.262
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• pp.347-353
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• 2006

Background: Despite recent advances in surgical technique and perioperative care of total anomalous pulmonary venous return (TAPVR), post-repair pulmonary vein stenosis (PVS) remains as a serious complication. We thought that the most important factors of TAPVR repair to prevent PVS were good exposure, proper alignment, and sufficient stoma size. We analyzed our experience retrospectively. Material and Method: Between Jan. 1995 and Feb. 2005, we studied 74 patients diagnosed with TAPVR suitable for biventricular repair. Supra-cardiac type (n=41, 55.4%) was the most common. Mean CPB time, ACC time, and TCA (40.5%, 30/74) time were $92.1{\pm}25.9\;min,\;39.1{\pm}10.6\;min$, and $30.2{\pm}10.7\;min$, respectively. Mean follow-up duration was $41.4{\pm}29.1$ months and follow-up was possible in all patients. Result: The median age and body weight at operation were 28.5 days ($0{\sim}478$ days) and 3.4 kg $(1.4{\sim}9\;kg)$. Early mortality was 4.1% (3/74). Causes of death were pulmonary hypertensive crisis, sepsis, and sudden death. There was PR-PVS in 2 patients (early: 1, late: 1). Both patients were cardiac type TAPVR drained to coronary sinus. Re-operations were done but only one patient survived. Cumulative survival rate in 5 year and percent freedom from PVS were $94.5{\pm}2.7%\;and\;97.2{\pm}2.0%$, respectively. Conclusion: There was no PVS in patients who underwent extra-cardiac anatomosis between LA and CPVC. Therefore it could be said that our principle might be effective in preventing PR-PVS in patients suitable two-ventricle.

• Journal of Chest Surgery
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• v.37 no.4
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• pp.364-368
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• 2004

Congenital pulmonary vein stenosis is a rare anomaly and related to high mortality due to progressive pulmonary hypertension and heart failure in infancy. Aggressive anti-failure medication and surgical treatment is recommended. Surgical options are balloon dilatation, endovascular stent, pneumonectomy, lung transplantation, patch grafting, and sutureless repair. We report a case of congenital pulmonary vein stenosis with normal anatomical connection successfully treated with sutureless technique and using pulmonary vasodilators, such as Sildenafil, lloprost and iNO postoperatively.

• 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.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.194-198
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• 2009

Purpose : Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. Methods : Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. Results : Mean age at diagnosis was $28.1{\pm}33.4$ days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. Conclusion : In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.

• 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.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.510-517
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• 1999

Background: Total anomalous pulmonary venous connection (TAPVC) is still one of the more challenging congenital heart defects in newborns and young infants. The purpose of the study is to evaluate the early and midterm results of the surgical corrections for patients in early infancy with isolated TAPVC. Material and Method: Hospital records of 15 consecutive patients in early infancy (January 1993 to August 1998) were retrospectively reviewed. There were 8 boys and 7 girls whose ages ranged from 4 days to 3.5 months (median age 22 days). Their body weight ranged from 1.75 kg to 4.9 kg (mean 3.54 kg). The abnormal anatomical connections were supracardiac in 11, cardiac in 3, and infracardiac in 1. In 6 patients (40%), the pulmonary venous drainage was obstructive. Total circulatory arrest was used in 13 patients. Anastomosis between the common pulmonary vein and the left atrium was performed with a continuous suture technique using a fine nonabsorbable polypropylene suture through a lateral approach behind the right atrium. Result: There was one hospital death (6.5%) caused by a sepsis 17 days after the operation in a neonate who had supracardiac drainage and was dependent on a ventilator preoperatively. There were 2 late deaths. One died sudde`nly of an unknown cause at home 2.5 years after the operation and the other died of a recurrent pulmonary hypertension 3 months after the reoperation due to pulmonary venous obstruction (PVO). Two patients required reoperations because of PVO 5 months and 10 months respectively after the initial operation. Of these patients, one patient is alive at the present time with persistent pulmonary hypertension. All survivors without postoperative PVO (78.6%) were in NYHA functional class I at mean follow-up of 25.8 months (0.5∼67 months). Conclusion: Surgical correction of TAPVC in early infancy can be performed at low risk. However, there were 2 postoperative PVOs (14.3%) which had bad results. The survivors without postoperative PVO had excellent functional status.

• Journal of Chest Surgery
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• v.39 no.6 s.263
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• pp.479-481
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• 2006

A newborn girl with a partial anomalous pulmonary venous connection, coarctation of the aorta, and ventricular and atrial septal defects underwent a complete repair successfully at 49 days of age. In this case, the left upper pulmonary vein was connected to the left innominate vein via an atypical vertical vein.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.12-17
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• 2006

Background: Circulatory arrest under deep hypothermia is an important auxiliary means for surgical correction of total anomalous pulmonary venous connection (TAPVC), However, cardiac operations under deep hypothermic circulatory arrest are associated with the risk of post-arrest neurologic abnormalities. The purpose of this study is to evaluate the results of the surgical correction of total anomalous pulmonary venous connection without the total circulatory arrest. Materiai and Method: Between April 2000 and October 2004, hospital records of 10 patients were reviewed retrospectively. Result: The locations for abnormal anatomical connections were supracardiac in 7 cases, cardiac in 1 case, and infracardiac in 2 cases. The mean cardiopulmonary bypass time and aorta cross clamp time were 116.8$\pm$40.7 and 69.5$\pm$24.1 minutes. There was no surgical mortality. Postoperative complications were post-repair pulmonary venous stenosis in 1 case, pneumonia in 1, pneumothorax in 1, wound infection in 1,and diaphragmatic paralysis in 1. All patients without pulmonary venous stenosis were in NYHA class I at mean follow-up of 16.6 months (3$\∼$49 months) Conclusion: We could obtain excellent results by repair without the total circulatory arrest for total anomalous pulmonary venous connection.

• Journal of Chest Surgery
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• v.34 no.4
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• pp.345-350
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• 2001

배경: 항부정맥 술식으로서의 메이스 술식은 우수한 동율동 전환율을 보고 있으나, 술식의 복잡성과 상대적으로 긴 수술 시간 등으로 모든 환자들에게 적용할 수는 없기에, 일부 환자군에서 보다 간단한 항부정맥술인 폐정맥 분리술을 시행 받은 환자들을 대상으로 동율동 전환율 등의 수술 성적과 위험 인자 등에 대해 알아보고자한다. 대상 및 방법: 1995년 10월부터 1999년 2월까지, 폐정맥 분리술을 시행받은 96명의 환자들을 대상으로 후향적 연구를 시행하였다. 남자가 37명(38.5%), 여자가 59명(61.5%) 이었고, 평균 연령은 46.9$\pm$11.6세 이었다. 승모판 협착 환자가 50명(52.1%), 폐쇄 부전이 24명(25%), 협착과 폐쇄부전이 같이 있었던 경우가 22명(22.9%)이었다. 판막 술식과 폐정맥 분리술을 시행하고 좌심방이 제거술을 시행하였다. 모든 환자들은 술 후 6개월 내지 1년 이내에 외래에서 심전도와 심초음파를 시행받았다. 평균 추적 관찰 기간은 25.9$\pm$11.5개월 이었다. 술 후 최근 추적일까지 동율동으로 전환된 군과 안된 군으로 나누어 분석을 하였다. 결과: 조기 성적으로는 사망이 3명(3.1%), 술 후 6개월 이내의 추적관찰 유실이 2명(2.1%) 이었다. 조기 합병증으로 재수술이 필요 했던 출혈이 4례, 심폐바이패스 이탈 실패오 심실보조장치 등의 삽입이필요했던 경우가 1례, 뇌혈관질환 1례, 심근경색이 1례 등이 있었다. 만기 합병증으로는 동기능 부전 증후군으로 영구 심박동기를 삽입한 경우가 1례, 일시적인 뇌경색 1례 있었고 다른 판막과 연관된 합병증은 없었다. 동율동 전환 환자 수는 75명(82%)이었다. 동율동으로의 전환이 어려운 위험인자로 50세로 이상의 연령(p=0.03), 65mm 이상의 좌심방 크기(p=0.03), 동반 우심 술식 시행(p=0.02)등이 있었다 결론: 승모판 질환을 동반한 심방 세동 환자에서 폐정맥 분리술을 시행하여 만족할 만한 동율동 전환율을 얻을 수 있었다.