• 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.31 no.2
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• pp.168-172
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• 1998

Inferior sinus venosus defect is a rare lesion in which there is a large interatrial communication adjacent to the atrial connection of the inferior caval vein. The defect is located posteriorly and inferiorly, outside the confines of the true atrial septum, and partial anomalous pulmonary venous connections are the rule. We underwent surgical repair in four patients with inferior sinus venosus defect and partial anomalous pulmonary venous return. There were three males and one female with an age range from four months to 25 years. A cross- sectional echocardiogram and cardiac catheterization had been performed preoperatively in all patients, but the correct diagnosis had been made in only one case. Surgical repair was indicated due to congestive heart failure, and one patient of 4-month-old needed urgent operation. The repair was accomplished by suturing a untreated autologous pericardial patch to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium. The preoperative knowledge of the unusual anatomy allows the surgeon to repair the anomaly without difficulties. For the patients in whom interatrial communication and anomalous pulmonary venous return are suggested, surgeon has to pay careful attention to the anatomical landmarks to avoid incorrect placement of the patch.

• 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.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.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.

• 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.

• Journal of Chest Surgery
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• v.29 no.3
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• pp.292-296
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• 1996

Total anomalous pulmonary venous return is a rare but serious cardiac malformation, accounting for only about 1.5~3% of congenital heart disease. Surgical results have been dramatically improved in the last two decades, largely owing to improved techniques of cardiopulmonary bypass and perloperative management. Seven patients ranging in age from 15 days to 11 years with total anomalous pulmonary venous return underwent repair between 1984 and 1995. The types of anomalous return were supracardiac in 5, and cardiac in 2. There were 5 boys and 2 girls. There were two hospital death, occurred in 15-day-old, and 40-day-old infants with supracardiac type. Follow-up periods have ranged from 3 months to 11 years, and all survivors have remained asymptomatic with normal growth and development.

• Proceedings of the KTCVS Conference
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• 1995.10a
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• pp.9-9
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• 1995

• Proceedings of the KTCVS Conference
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• 1998.10a
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• pp.126-126
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• 1998

• Journal of Chest Surgery
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• v.34 no.9
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• pp.672-679
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• 2001

Background: Surgical correction of partial anomalous pulmonary venous connection to the superior vena cava has been associated with postoperative venous obstruction and sinus node dysfunction. In this paper we describe our current approach and its short-term results. Material and Method: Between April 1999 and January 2000, 5 consecutive patients, ranging from 2 months to 66 years old, underwent corrective operation for partial anomalous pulmonary venous connection to the superior vena cava at Sejong General Hospital and Daegu Catholic University Medical Center. Surgical correction involved diversion of the pulmonary venous drainage to the left atrium using a right atrial flap(2 patients) or prosthetic patch(3 patients) with division of the superior vena cava superior to the restore site of the pulmonary veins and reimplantation on the right atrial appendage to restore systemic venous drainage. Result: All patients were discharged between postoperative day 9 and 15 without complications. One Russian boy returned to his country, therefore, he was lost to follow-up after discharge. Remaining 4 patients were asymptomatic and in normal regular sinus rhythm at a mean follow-up of 17.75$\pm$4.27 months. Follow-up echocardiographic study (range, 12 to 24 months) revealed no incidence of narrowing of the venous pathways or of residual shunt. Conclusion: Our current approach is relatively simple and reproducible in achieving unobstructive pulmonay venous and SVC pathways. By avoiding incision across the cavoatrial junction, surgical injury to the sinus node and its artery may be minimized. The presented surgical technique can be safely and effectively applied to the selected patients.