• Journal of Chest Surgery
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• 제21권1호
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• pp.148-151
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• 1988

We experienced 2 years and 5 months old male patient with partial anomalous pulmonary venous return of the left lung into the coronary sinus without atrial septal defect. After incising the atrial septum and the wall between the left atrium and the coronary sinus, we made the roof of the coronary sinus and closed the artificial atrial septal defect, with using patch, then we could change the direction of the blood flow from the coronary sinus into the left atrium. The patient was discharged on the 13th postoperative day after uneventful postoperative course.

• Journal of Chest Surgery
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• 제19권2호
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• pp.273-279
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• 1986

Total anomalous pulmonary venous return is a cardiac malformation in which there is no direct connection between any pulmonary vein and the left atrium but, rather all the pulmonary veins connect to the right atrium or one of its tributaries. TAPVC is a relatively uncommon anomaly, accounting for only about 1.5-3% of cases of congenital heart disease. Recently improvement in intraoperative techniques did eventually bring substantial improvements in the results in infants. 4 cases of TAPVC was successfully treated with one-stage operation, in the Dept. of Thoracic and Cardiovascular Surgery, National Medical Center in which 2 cases are supracardiac types and the other 2 cases are cardiac types. Sex ratio was 1:1, and the range of age was 2 years-18 years. The common pulmonary venous sinus was connected to the left vertical vein and innominate vein: in 2 supracardiac types and coronary sinus in 2 cardiac types. All cases are operated with standard cardiopulmonary bypass, and the hospital mortality was 0%.

• Journal of Chest Surgery
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• 제28권12호
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• pp.1174-1177
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• 1995

We experienced one case of TAPVC patient who was 39 years old man with mild dyspnea on exertion and easy fatigability. He was the oldest patient among TAPVC which was reported in Korea. Preoperative echocardiogram, cardiac catheterization and angiogram revealed supracardiac type TAPVC drained through left innominate vein. An anastomosis between common pulmonary venous trunk and left atrium, pericardial patch closure of ASD and ligation of left vertical vein were performed with extracorporeal circulation. The postoperative course was uneventful and discharged with excellent general condition and has been well during 8 months follow-up.

• Journal of Chest Surgery
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• 제12권1호
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• pp.30-32
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• 1979

Partial anomalous pulmonary drainage is frequently accompanied by an atrial septal defect. The anomaly is entirely compatible with life and the clinical findings are dependent primarily on the proportion of the pulmonary venous return to the right atrium. The anomaly is frequently missed even with complete diagnostic study. In this report we are presenting a case of anomalous pulmonary venous drainage combined with atrial septal defect and dextrocardia and the treatment of this condition.

• 대한영상의학회지
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• 제82권2호
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• pp.440-446
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• 2021

Levoatriocardinal 정맥은 폐정맥 기형의 드문 형태로, 좌심방 혹은 폐정맥과 체정맥을 연결해 주는 구조이다. 이는 폐정맥이 체정맥으로 연결되는 기형을 뜻하는 부분 폐정맥 환류 이상과 구분되는데, Levoatriocardinal 정맥은 정상 폐정맥을 통한 좌심방과의 연결성이 있다는 차이점이 있다. 저자들은 폐정맥정맥류가 흉부 엑스선 및 전산화단층촬영에서 동정맥기형으로 오인되었던 증례를 보고하고자 하며, 이는 혈관조영술을 통해 폐정맥정맥류와 동반된 Levoatriocardinal 정맥으로 진단된 경우이다. 이러한 폐정맥정맥류를 동반한 Levoatriocardinal 정맥을 보이는 증례는 영문 문헌상 보고된 바 없기에 이를 보고하고자 한다.

• Journal of Chest Surgery
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• 제49권5호
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• pp.337-343
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• 2016

Background: Recent developments in surgical techniques and hospital care have led to improved outcomes following total anomalous pulmonary venous return (TAPVR) repair. However, the surgical repair of TAPVR remains associated with a high risk of mortality and need for reoperation. We conducted this retrospective study to evaluate mid-term outcomes following in situ TAPVR repair without total circulatory arrest (TCA), and to identify the risk factors associated with surgical outcomes. Methods: We retrospectively reviewed 29 cases of surgical intervention for TAPVR conducted between April 2000 and July 2015. All patients were newborns or infants who underwent in situ TAPVR repair without TCA. Results: Four anatomic subtypes of TAPVR were included in this study: supracardiac (20 cases, 69.0%), cardiac (4 cases, 13.8%), infracardiac (4 cases, 13.8%), and mixed (1 case, 3.4%). The median follow-up period for all patients was 42.9 months. Two (6.9%) early mortalities occurred, as well as 2 (6.9%) cases of postoperative pulmonary venous obstruction (PVO). Preoperative ventilator care (p=0.027) and preoperative PVO (p=0.002) were found to be independent risk factors for mortality. Conclusion: In situ repair of TAPVR without TCA was associated with encouraging mid-term outcomes. Preoperative ventilator care and preoperative PVO were found to be independent risk factors for mortality associated with TAPVR repair.

• Journal of Chest Surgery
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• 제32권10호
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• pp.939-942
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• 1999

Partial anomalous pulmonary venous connection is frequently found in any ASD(atrial septal defect) patients. These patients are usually symptomatic, therefore, easily diagnosed as just simple ASD. We experienced a case of a 37-year-old female patient with ASD in which the left upper pulmonary vein was connected to SVC by the left inominate vein. The patient was diagnosed as simple ASD previously. During cardiac catheterization, we found a meaningful oxygen saturation step up between the SVC and its upper portion. Angiogram confirmed PAPVC. The surgical correction of anastomosis of PAPVC with left atrial appendage and direct closure of ASD were done. The patient was discharged 15 days later.

• Journal of Genetic Medicine
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• 제3권1호
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• pp.1-4
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• 1999

Scimitar syndrome is a rare congenital anomaly that is characterized by hypoplasia of the right lung and the right pulmonary artery with anomalous pulmonary venous drainage to the inferior vena cava. The scimitar vein is usually visible on chest radiographs, but may be obscured by the heart. It is essential for surgical correction to establish the point of drainage of the anomalous vein and associated anomalies. There are recent reports of familial total anomalous pulmonary venous return suggesting heritable forms of this anomaly. Although genetic factors are believed to have important roles in congenital heart disease, few genes involved in heart development have been located. We report a case of familial chromosome 9 inversion with Scimitar syndrome in an offspring who presented with dextrocardia. Evaluation with magnetic resonance cineangiograph imaging demonstrated an anomalous pulmonary vein draining into the inferior vena cava above the diaphragm and hypoplasia of the right lung and the right pulmonary artery. Chromsome analysis showed pericentric inversion of chromosome 9, inv 9 (p13, q21), in the patient and his mother as well. A brief review of the related literature is also included.

• Journal of Chest Surgery
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• 제31권2호
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• pp.168-172
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• 1998

하정맥동 결손(inferior sinus venosus defect)은 하대정맥과 우심방의 경계 부위에 발생하는 심방중격결손의 한 형태로 매우 드문 선천성 심장질환이며, 심방중격의 하부 뒤쪽에 위치하고 한 개 이상의 우 폐정맥의 환류 이상을 동반하고 있어 개심술시 정확한 교정을 요하므로 수술 전 또는 수술 중 정확한 해부학적 진단이 선행되어야 한다. 저자들은 부분 폐정맥 환류이상을 동반한 하정맥동형 심방중격결손 4예를 치험하였다. 1예는 25세 성인이었고 3예는 14개월 미만의 영아 및 소아였다. 4예 모두 심부전 때문에 수술했으며 이중 4개월의 영아는 긴급 수술이 필요했다. 진단을 위해 심초음파 및 도플러 검사와 심도자법을 시행하였다. 수술전 확진은 1예에서 가능했고 3예는 수술전 병변을 의심하고 수술시 확진되었다. 수술방법으로 이상 환류되는 폐정맥이 좌심방으로 환류 되도록 자가심막을 이용하여 심방중격결손을 폐쇄하였다. 4예 모두 수술 결과는 양호하였으며 초음파 검사의 추적에서 하대정맥과 폐정맥의 환류장애 소견은 없었다. 정확한 수술 교정을 위해 수술전 정확한 진단이 필수적이며, 수술전 폐정맥의 부분 환류이상과 심방중격 결손으로 진단된 환자에서는 수술중 하정맥동 결손의 여부를 확인하여 적절한 수술적 교정을 시행해야 할 것으로 사료된다.

• Journal of Chest Surgery
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• 제39권1호
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• pp.12-17
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• 2006

배경: 초 저체온 하 순환 정지는 총 폐정맥 환류 이상을 수술하는데 있어 중요한 보조 수단이다. 그러나 초 저체온 하 순환 정지 하에 심장 수술을 하는 것은 순환 정지 후 신경계 이상의 위험을 동반하고 있다. 이에 완전 순환 정지 없이 총 폐정맥 환류 이상을 수술하여 그 결과를 평가하고자 한다. 대상 및 방법: 2000년 4월에서 2004년 10월까지 10명의 환자의 의무 기록을 후향적으로 분석하였다. 결과: 해부학적 연결 이상의 위치는 심장 상부형이 7예,심장형이 1예, 심장 하부형이 2예였다. 평균 심폐기 가동 시간과 대동맥 차단 시간은 각각 116.8 $\pm$40.7분, 69.5$\pm$24.1분이었다. 수술로 인한 사망 예는 없었고, 합병증은 술 후 폐정맥 협착이 1예, 폐렴, 기흉, 창상 감염, 횡격막 마비가 각각 1예씩 있었다. 평균 16.6개월의 추적 관찰 기간동안 페동맥 협착이 없었던 모든 환아는 NYHA class I으로 지내고 있다. 결론: 총 폐정맥 환류 이상을 완전 순환 정지 없이 수술하여 아주 만족할 만한 결과를 얻을 수 있었다.