• Journal of Chest Surgery
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• v.29 no.2
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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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• v.27 no.1
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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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• v.20 no.4
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• pp.695-705
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• 1987

This report provides follow - up data on 37 patients, aged 7 days to 25 years [median: 6.5 months], who underwent repair of total anomalous pulmonary venous connection at Seoul national University Hospital between May, 1978 and June, 1987. The patients were 22 males and 17 females and the sex ratio was 1.6 to 1, showing a male predominance. Sixteen patients had supracardiac, 13 cardiac, 3 infracardiac and 5 had a mixed type. The duration of follow up was from 1 month to 60 months [median: 14 months] There were eight early and one late deaths, and the overall mortality was 24%. The deaths during 1 year of life were eight [89%] and only one death [11%] occurred above 1 year of age. The mortality of cardiac type was unusually high, accounting for 56 percent of the total death, which was probably due to the preoperative poor clinical condition such as pulmonary edema and congestive heart failure. The major cause of death was the perioperative myocardial failure, and the survival was closely related to the preoperative clinical status, age and moderately elevated pulmonary arterial pressure, the sign of the elevated pulmonary vascular resistance and pulmonary venous obstruction. Early diagnosis and early application of surgical intervention is essential to the improved postoperative survival

• Journal of Chest Surgery
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• v.14 no.1
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• pp.40-48
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• 1981

Total anomalous venous return defines a group of congenital heart disease which have in common the entire pulmonary venous drainage returning directly or indirectly to the right atrium instead of to the left atrium. Despite of recent advance in treatment, this severe malformation in its various anatomical forms has a high surgical mortality during early infancy. Because of the high mortality in the untreated infant and the surgical risk in the first year of life, the timing of the operation remains important for optimal result. Three cases of T APV R, two supracardiac types and one mixed type, were treated with extracorporeal circulation during last three years in the Dept. of Thoracic and Cardiovascular Surgery, Seoul National University Hospital. The first one was 10 months old male with supracardiac type which drained through left innominate vein, and he was operated with profound hypothermia and total circulatory arrest but failed. The second case was 7 years old male with supracardiac type drained through left innominate vein, and he was well post operatively, and followed periodically for 12 months. The third case was 24 years old female with mixed type drainage (left upper pulmonary vein drained through left innominate vein, and the others through coronary sinus) was successfully corrected, and she was followed for 4 month without problem. All cases were diagnosed with cardiac catheterization and angiocardiogram, and also with echocardiogram in last two cases. In first two cases of supracardiac type, total circulatory arrest was used in brief period during anastomosis between common pulmonary venous trunk and left atrium. In the last case of mixed type, usual cardiopulmonary bypass with moderate hypothermia was used and total circulatory arrest was not needed.

• Journal of Chest Surgery
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• v.36 no.3
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• pp.136-141
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• 2003

We assessed the intermediate-term result of tricuspid annuloplasty (TAP) for tricuspid valve regurgitation (TR) associated with congenital heart disease in adults. Risk factors for residual TR were also analysed. Material and Method: From August 1989 to June 2001, seventy three adult patients, 51 females and 22 males, underwent TAP for TR associated with various congenital heart disease. Their age ranged from 46 years to 73 years (mean:43). Associated heart anomalies were atrial septal defect (55), ventricular septal defect (6), partial anomalous pulmonary venous return (4) and others (8). Preoperative and post-operative TR velocities were 3.25 m/sec and 2.56 m/sec respectively, and the types of TAP were De Vega in 43, Kay in 18 and Ring annuloplasty in 12. Postoperative follow-up duration was 2,347 patient-month (mean: 32.6 months), and 134 two-dimensional echocardiographic examinations were done during this period. Residual TR greater than III/IV was considered as TAP failure. Result: TAP failure was observed in 7 patients (9.6%), and one patient among them underwent tricuspid valve replacement. Risk factors for TAP failure were diagnosis other than atrial septal defect (p=0.001), preoperative (p=0.038) and postoperative (p=0.028) high TR velocity. There was no statistical significance in terms of TAP methods. Conclusion: Careful evaluation of valve morphology and aggressive surgical intervention are mandatory for the repair of TR with preoperative or residual RV pressure overload.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.430-438
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• 2002

It is known that low birth weight is a risk factor for poor outcome in cardiac surgery for many cardiac defects. We reviewed our recent surgical experiences on congenital heart defect (other than patent ductus arteriosus) in low birth weight babies. Material and Method: From September 1994 to February 2001, 31 consecutive infants weighing 2500 g or less underwent cardiac surgery with (OHS group n=12) or without cardiopulmonary bypass (CHS group n=19). A retrospective study was carried out to evaluate short-and intermediate-term outcome. Mean gestational age and age at operation were 36.9 weeks(range, 32.3-42weeks) and 32.1days (range, 0-87days) respectively. Mean body weight at birth and operation were 1972g (range, 1100-2500g) and 2105g (range, 1450-2500 g) respectively. There was no difference between the two groups in age and body weight. Defects included ventricular septal defect (VSD) (n=3), VSD with arch anomaly (n=2), total anomalous pulmonary venous return (n=2), transposition of the great arteries (TGA) (n=2), truncus arteriosus (n=2), and univentricular heart with cor triatriatum (n=1) in OHS group, and coarctation of aorta (n=7), tetralogy of Fallot (TOF) (n=3), TOF with pulmonary atresia (n=3), multiple muscular VSDs (n=1), double outlet right ventricle (n=1), pulmonary atresia with intact ventricular septum (n=2), tricuspid atresia (n=1), and TGA with multiple VSD (n=1) in CHS group. 13 patients (41.9%) were intubated pre-operatively. Result: There were 4 early deaths(＜30 days); 1 (8.3%) in OHS group and 3 (15.8%) in non-OHS group. All these early deaths were related to the pulmonary artery banding(PAB). There was no operative mortality in infants undergoing complete repair and palliative operations other than PAB. Delayed sternal closure was required in 3 patients. Prolonged postoperative mechanical ventilation (>7 days) was required in 7 patients(58.3%) in OHS and 7(38.8%) in CHS group. Late mortality occurred in 3 patients, two of which were non-cardiac. A patient in OHS group was documented to have neurologic sequelae. All the survivors except two are in NYHA class 1. Conclusion: Complete repair and palliative operations other than PAB can be performed in low birth weight infants with low operative mortality and an acceptable intermediate-term result. However, about a half of the patients required long-term postoperative mechanical ventilation.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.504-510
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• 2004

Background: Vasodilatory shock after cardiac surgery may result from the vasopressin deficiency following cardio-pulmonary bypass and sepsis, which did not respond to usual intravenous inotropes. In contrast to the adult patients, the effectiveness of vasopressin for vasodilatory shock in children has not been known well and so we reviewed our experience of vasopressin therapy in the small babies with a cardiac disease. Material and Method: Between February and August 2003, intravenous vasopressin was administrated in 6 patients for vasodilatory shock despite being supported on intravenous inotropes after cardiac surgery. Median age at operation was 25 days old (ranges; 2∼41 days) and median body weight was 2,870 grams (ranges; 900∼3,530 grams). Preoperative diag-noses were complete transposition of the great arteries in 2 patients, hypoplastic left heart syndrome in 1, Fallot type double-outlet right ventricle in 1, aortic coarctation with severe atrioventricular valve regurgitation in 1, and total anomalous pulmonary venous return in 1. Total repair and palliative repair were undertaken in each 3 patient. Result: Most patients showed vasodilatory shock not responding to the inotropes and required the vasopressin therapy within 24 hours after cardiac surgery and its readministration for septic shock. The dosing range for vasopressin was 0.0002∼0.008 unit/kg/minute with a median total time of its administration of 59 hours (ranges; 26∼140 hours). Systolic blood pressure before, 1 hour, and 6 hours after its administration were 42.7$\pm$7.4 mmHg, 53.7$\pm$11.4 mmHg, and 56.3$\pm$13.4 mmHg, respectively, which shows a significant increase in systolic blood pressure (systolic pressure 1hour and 6 hours after the administration compared to before the administration; p=0.042 in all). Inotropic indexes before, 6 hour, and 12 hours after its administration were 32.3$\pm$7.2, 21.0$\pm$8.4, and 21.2$\pm$8.9, respectively, which reveals a significant decrease in inotropic index (inotropic indexes 6 hour and 12 hours after the administration compared to before the administration; p=0.027 in all). Significant metabolic acidosis and decreased urine output related to systemic hypoperfusion were not found after vasopressin admin- istration. Conclusion: In young children suffering from vasodilatory shock not responding to common inotropes despite normal ventricular contractility, intravenous vasopressin reveals to be an effective vasoconstrictor to increase systolic blood pressure and to mitigate the complications related to higher doses of inotropes.