• Journal of Chest Surgery
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• 제32권11호
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• pp.1004-1008
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• 1999

Background: Total anomalous pulmonary venous return is a relatively rare disease which has a very high mortality(80% within a year) if not properly corrected surgically. Material and Method: Twenty-six infants with total anomalous pulmonary venous return underwent repair between May, 1991 and February, 1996. Result: There were 19 boys and 7 girls. The mean age at operation was 2.6 months(range: 5 day to 11 month) and the mean body weight was 4.3kg(range:2.8 to 6.7 kg). Preoperative stabilization included ventilator for 5 patients and inotropic support for 6 patients. There were 6 hospital mortalities. Significant risk factors of operative mortality were preoperative ventilator care(p<0.03) and preoperative inotropic support(p<0.05). Age, body weight at operation, pulmonary venous obstruction, high pulmonary arterial pressure, spurasystemic right ventricular pressure or emergency operation did not affected the operative outcome. Postperative pulmonary venous obstruction occurred in three patients 2 or 3 months later, among them one patient was reoperated. The actuarial survival was 76% at 40 months. Conclusion: Although early mortality was high, repair of total anomalous pulmonary venous return should be attempted in early life, but the patients receiving ventilator care or inotropic support need special attention.

• Journal of Chest Surgery
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• 제27권1호
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• pp.52-56
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• 1994

This is case report of total anomalous pulmonary venous return with atrial septal defect which were corrected surgically by intracardiac procedure under total cardiopulmonary bypass.Two patients were supracardiac type,cardiac and mixed type was each one.The mixed type was three years old female patient.She was diagnosed as atrial septal defect with partial anomalous pulmonary venous return[right pulmonary vein drains into superior vena cava and right atrium] and corrected as usual.After operation,she underwent exertional dyspnea and frequent tachycardia.Chest x-ray film showed pulmonary congestion.Follow up cardiac cineangiogram revealed that left pulmonary vein also anomalously drained into left innominate vein through vertical vein.Through left thoracotomy,anastomosis was successfully carried between left atrium and vertical vein without cardiopulmonary bypass and there was no sign of pulmonary artery obstruction for two years follow up.The other three patient were corrected successfully without complication and got good result.

• Journal of Chest Surgery
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• 제29권2호
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• pp.213-218
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• 1996

Total anomalous pulmonary venous return (TAPVR) is very rare congenital heart disease. 25-year old male was admitted our hospital with dyspnea, headache and syncope as chief complaint. He was confirmed as mixed type TAPVR by echocardiography and cardiac catheterization. In this case, mixed type TAPVR was consisted with supracardiac type connection of left pulmonary vein and cardiac type of right pulmonary vein. Supracardiac type of left pulmonary common channel was anastomosed to the left auricular appendage during total cardiopulmonary bypass with fibrillating heart. Cardiac type of right pulmonary vein was operated during moderate hypothermia and aortic cross clamping. Coronary sinus septum was incised into ASD and closed with Gore-Tex patch so that right pulmonary blood flow directed to the left atrium. The patient's post-operative course was uneventful.

• Journal of Chest Surgery
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• 제29권3호
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• pp.292-296
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• 1996

총폐정맥환류이상증은 모든 폐정 맥혈이 좌심방 대신 직간접적으로 우심방으로 환류되는 선천성 심기 형으로 떼 내에 수술을 시 행치 않을 경우 80%의 높은 사망율을 나타내는 질환이다. 1984년 5월부터 1995년 5월까지 7례의 총폐정맥환류이상증에 대한 교정수술을 시행하였다. 환자의 연령분포는 15일부터 11세 까지로 평균 연령은 27.1개 월이 었다. 남녀 비는 남자가 5례, 여자가 2례 였다. 형태는 심장상부형이 5례, 심장형이 2례였다. 5례는 통상적인 체외순환하에서 수술하였고2례의 경우 초저 체온 및 완전혈류차단하에서 수술하였다 술후 합병증은 호흡부전 1례, 부정맥 2례, 창상감염이 1례 있었다. 수술사망은 2례 였으며 생존자들은 외래 추적 조사상 특이 증상없이 잘 지내고 있다.

• Journal of Chest Surgery
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• 제54권2호
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• pp.143-145
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• 2021

In a newborn in whom cor triatriatum was missed on echocardiography, infracardiac total anomalous pulmonary venous connection was successfully repaired with the aid of cardiac computed tomography (CT). In rare combinations, as in this case, an accurate diagnosis prior to surgery, which is of vital importance for successful repair, can be made through a high index of suspicion and the use of a supplemental imaging modality such as CT.

• Journal of Chest Surgery
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• 제28권6호
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• pp.565-570
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• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

• Journal of Chest Surgery
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• 제23권5호
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• pp.962-967
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• 1990

The anomalous pulmonary venous return of the entire left lung was an extremely rare congenital anomaly. The reported surgical experience with correction of this disorder was limited. The 3-year-old female patient underwent an operation upon the unilateral total anomalous pulmonary venous return from the left lung, in which the left superior pulmonary vein drained into innominate vein and the left inferior pulmonary vein into the coronary sinus, in Yeungnam University Hospital. The symptoms were nonspecific except frequent upper respiratory infection. Cyanosis was not seen. On auscultatory findings, a grade 2/6 systolic ejection murmur was audible over left second intercostal space of left sternal border and second heart sound had an increased pulmonary component which was widely splitted. The electrocardiogram demonstrated a right ventricular hypertrophy and right axis deviation and chest X-ray showed slightly increased pulmonary vascularity and bulged pulmonary conus. The echocardiogram demonstrated increased right atrial, ventricular, and pulmonary arterial dimension, and also secundum atrial septal defect and enlarged coronary sinus. The cardiac catheterization confirmed the left-to-right with a Qp/Qs of 2.0: 1 and oxygen step-up was seen in pulmonary artery, right ventricle, right atrium, and left innominate vein, and the catheter was not been introduced into the left pulmonary vein. A median sternotomy incision was done. Left superior pulmonary vein was drained to the innominate vein through anomalous vertical vein and the left inferior pulmonary vein drained to right atrium through the coronary sinus. The diversion of the left inferior pulmonary vein to posterior wall of left atrium was done after division in the proximity of coronary sinus. The anomalous vertical vein was diverted to base of left atrial auricle and then a atrial septal defect was sutured directly. The postoperative course was uneventful and she was discharged on the eleventh postoperative day. In the postoperative follow-up-2 months, she has been well without specific problems.

• 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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• 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.