• Journal of Chest Surgery
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• v.30 no.5
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• pp.493-500
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• 1997

We performed this study to evaluate hepatic venous drainage in atrial isomerism by MR and the clinical significance of anomalous hepatic venous return in total cavopulmonary shunt operation. Numbers and locations of hepatic veins in twenty-two patients with isomerism(thirteen with right isomerism and nine with left isom rism) were evaluated by MR. Operative procedure of hepatic veins and postoperative arterial oxygen saturation were compared with hepatic vein connection in six patients after total cavopulmonary shunt operation. Among nine patients with left isomerism, hepatic venous return was totally anomalous via a single opening in eight, and via two separate openings in one. Among thirteen patients with right isomerism, partial anomalous hepatic venous connection directly to the atrium was seen in four. One showed total anomalous hepatic venous connection to atrium through one opening. Total cavopulmonary shunt operation was performed in 6 patients. Hepatic veins were connected to pulmonary arteries in four patients who had one atrial opening of hepatic vein andlor IVC, or two ipsilateral atrial opening of hepatic veins and IVC. In conclusion, hepatic vein drainage to atrium is variable in atrial isomerism. MR is useful for evaluation of hepatic vein drainage in atrial isomerism and surgical pla ning.

• Journal of Chest Surgery
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• v.49 no.5
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• pp.379-382
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• 2016

With advancements in complex repairs in neonates with complicated congenital heart diseases, extracorporeal membrane oxygenation (ECMO) has been increasingly used as cardiac support. ECMO has also been increasingly used for low birth weight (LBW) or very low birth weight (VLBW) neonates. However, since prematurity and LBW are risk factors for ECMO, the appropriate indications for neonates with LBW, especially VLBW, are under dispute. We report a case of ECMO performed in a 1,360-g premature infant with VLBW due to cardiopulmonary bypass weaning failure after repairing infracardiac total anomalous pulmonary venous return.

• Journal of Genetic Medicine
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• v.3 no.1
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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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• 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.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.21 no.6
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• pp.1103-1110
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• 1988

We experienced 1 year old female patient with partial anomalous pulmonary venous return of the right lung into the inferior vena cava with atrial septal defect. Total anomalous venous drainage from the right lung is an uncommon form of congenital defect, and it may or may not be associated with an atrial septal defect. One patient having this venous anomaly is presented with a detailed description of the surgical reconstruction used. The postoperative result has been satisfactory.

• Journal of Chest Surgery
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• v.26 no.3
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• pp.180-185
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• 1993

From February 1982 to December 1991, 49 neonates and 105 infants in less than 3 months of age underwent open heart surgery in Seoul National University Hospital. There were 98 males and 56 females, and their mean ages were 16 days in neonatal group and 67 days in early infant group. Their body weight and height were less than 3 percentile of normal developmental pattern. In order of decreasing incidence, the corrected conditions included Transposition of great arteries with or without ventricular septal defect [43], isolated ventricular septal defect [34], Total anomalous pulmonary venous return [21], Pulmonary atresia with intact ventricular septum [9] and others [47]. Various corrective or palliative procedures were performed on these patients; Arterial switch operation [36], patch closure for ventricular septal defect [34], Repair of total anomalous pulmonary venous return [21], RVOT reconstruction for congenital anomalies with compromised right ventricular outflow tract [17]. Profound hypothermia and circulatory arrest were used in 94 patients [ 61% ]: 42 patients [ 85.7% ] for neonatal group and 52 patients [ 49.5% ] for early infant group. The durations of circulatory interruption were within the safe margin according to the corresponding body temperature in most cases [ 84% ]. The hospital mortality was 36.4% ; 44.9% in neonatal group and 32.4% in infant group 1 to 3 months of age. The mortality was higher in cyanotic patients [ 46.6% ], in those who underwent palliative procedures [ 57.8% ], in patients whose circulatory arrest time was longer than safe periods [ 60% 0] and in patients who had long periods of cardiopulmonary bypss and aortic crossclamping. In conclusion, there has been increasing incidence of open heart surgery in neonates and early infants in recent years and the technique of deep hypothermia and circulatory arrest was applied in most of these patients, and the mortality was higher in cyanotic neonates who underwent palliative procedures and who had long cardiopulmonary bypass , aortic cross-clamping and circulatory arrest.

• Journal of Chest Surgery
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• v.35 no.1
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• pp.52-55
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• 2002

We report one case of an 18-day-old female patient, weighing 3.4 kg, with severe cyanosis. The diagnosis was made with only transthoracic echocardiography, which revealed cor triatriatum with an atretic small opening of fibromuscular membrane, obstructive infracardiac total anomalous pulmonary venous drainage(TAPVD), severely restrictive interatrial communication, and scanty mitral inflow and aortic forward flow. The preoperative decision-making for biventricular repair was not easy due to collapsed left heart system caused by remarkably reduced blood flow An emergent operation was performed due to severe cyanosis. All left heart structures were somewhat hypoplastic but thought to be adequate for systemic circulation. Biventricular repair was done without specific intraoperative problems. The postoperative course was uneventful. The patient has been doing well with no evidence of pulmonary vein stenosis or mitral regurgitation for 4 months after operation.

• Journal of Chest Surgery
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• v.40 no.8
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• pp.569-573
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• 2007

A 3 month old female baby, who had been diagnosed with right atrial isomerism associated with total anomalous pulmonary venous return (TAPVR), a functional single ventricle and major aortopulmonary collateral arteries (MAPCA), underwent left MAPCA unifocalization and left Blalock-Taussig shunt (3.5 mm) at 3 months of age. The post-operative course was complicated by pulmonary venous congestion, and the drainage site of the TAPVR was found to be stenotic on echocardiography. We performed sutureless repair of the TAPVR along with unifocalization of the right MAPCA. She was put on an extracorporeal membrane oxygenator for 8 days after the 2nd operation, and she was able to come off the oxygenator with the placement of a central shunt (3 mm). She developed tracheal stenosis, which was presumably due to longstanding endotracheal intubation, and she then underwent tracheostomy. She was discharged to home on day 104 after the 1st operation, and she has been followed up for 2 months in a good clinical condition.

• Journal of Chest Surgery
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• v.28 no.8
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.