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KMID : 1130320090520020194
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Korean Journal of Pediatrics
2009 Volume.52 No. 2 p.194 ~ p.198
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Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection)
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Chu Mi-Ae
Cho Joon-Yong
Kim Yeo-Hyang
Hyun Myung-Chul
Lee Sang-Bum
Choe Byung-Ho
Choi Hee-Joung
Kim Gun-Jik
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Abstract
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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 1 9 9 4 to February 2 0 0 8 were included.
Results : Mean age at diagnosis was 2 8 .1±3 3 .4 days (1 -1 2 6 days). Sites of drainage were supracardiac type (1 5 ), 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 1 4 patients (5 8 .3%). The operative mortality was 1 6 .7% (4 of 2 4 ) and overall hospital mortality (including deaths without operation) was 2 2 .2% (6 of 2 7 ). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (6 0%) patients, and ostial pulmonary vein in the other 2 (4 0%) 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 periand post-operative echocardiographic follow-up examinations.
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KEYWORD
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Heart defects, Congenital, Pulmonary veins’Reoperation, Mortality
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