Korean J Pediatr.  2009 Feb;52(2):194-198. 10.3345/kjp.2009.52.2.194.

Clinical features and results of recent total anomalous pulmonary venous connection : Experience in a university hospital (Clinical study of total anomalous pulmonary venous connection)

• Chu MA
• Choi BH
• Choi HJ
• Kim YH
• Kim GJ
• Cho JY
• Hyun MC
• Lee SB

• KMID: 2281675
• DOI: http://doi.org/10.3345/kjp.2009.52.2.194

Abstract

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+/-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.

Keyword

Heart defects; Congenital; Pulmonary veins; Reoperation; Mortality

MeSH Terms

Drainage
Early Diagnosis
Follow-Up Studies
Heart
Hospital Mortality
Humans
Prognosis
Pulmonary Veins
Reoperation