• 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.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.

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

• 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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• 1998.10a
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• pp.126-126
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• 1998

• Proceedings of the KTCVS Conference
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• 1995.10a
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• pp.70-70
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• 1995

• 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.21 no.4
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• pp.692-699
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• 1988

In 1987, we experienced 2 cases of T.A.P.V.D. corrected successfully under cardiopulmonary bypass. The first case was 28 years old male with supracardiac type drained through left innominate vein. He was oldest patient among T.A.P.V.D. which was reported in Korea. The other case was 14 years old male with cardiac type drained to right atrium directly without communication with coronary sinus. Two patients were well in postoperative 9 and 10 months and have NYHA functional class I.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.322-330
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• 1983

TAPVC represents 1 to 4% of all congenital cardiac defects. Generally severe and refractory cardiac failure develops in the majority of patients in the early infancy. In a small minority of patient, they are relatively asymptomatic in infancy, but symptoms will develop of necessity In the later life. Our three cases had dyspnea on exertion and cyanosis and had a history of frequent respiratory infections. All 3 cases had the typical showman configuration on roentgenograms of the chest. Current surgical therapy has greatly altered the unfavorable course of these patients after institution of extra-corporeal circulation. We have experienced three cases from June, 1973 to May, 1983, the patients were undergone complete repair with extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery in the Yonsei University. The ages ranged from 12 years to 14 years, all cases were supracardiac type of TAPVC. Only one case died due to bleeding from anastomosis site between LA and pulmonary venous trunk. In the remaining two patients with complete corrections of TAPVC, there was excellent relief of symptoms such as dyspnea and cyanosis. Their postoperative course have been good during follow-up.

• 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.