Paul M. Kirshbom, Richard J. Myung, J. William Gaynor, Richard F. Ittenbach, Stephen M. Paridon, William M. DeCampli, Tom R. Karl, Thomas L. Spray
Children’s Hospital of Philadelphia.
United States
Annals of Thoracic Surgery
Ann thorac Surg 2002; 74: 1616-1620
DOI: 10.1016/s0003-4975(02)03935-8
Abstract
Background: Early outcomes after repair of total anomalous pulmonary venous connection continue to improve; however, little information is available concerning long-term functional status and quality of life.
Methods: One hundred patients underwent isolated total anomalous pulmonary venous connection repair at The Children’s Hospital of Philadelphia between 1983 and 2001. Medical records were reviewed and a standardized questionnaire was administered to guardians of survivors.
Results: Median age at repair was 15.5 days (range, 1 to 563 days). Overall hospital mortality was 14%, decreasing from 19% before 1995 to 5% after 1995. At 15 years after repair, actuarial survival was 84% and freedom from late death or reintervention for hospital survivors was 85%. At a median follow-up of 5.9 years (range, 0 to 17.7 years) 64% of guardians described their child’s overall health as excellent, 27% good, 9% fair, and 0% poor. With regard to school performance, 40% of children were characterized as above average, 29% average, 4% below average, and 27% were in special education classes or had repeated grades. By multivariable logistic regression, the presence of associated chromosomal or noncardiac syndromes and pulmonary venous obstruction were found to be significant factors with regard to parental assessment of both overall health and school performance.
Conclusions: The majority of children who undergo isolated total anomalous pulmonary venous connection repair can expect an excellent long-term functional outcome. Factors present before operation, such as pulmonary venous obstruction and associated anomalies, can influence overall health and school performance in the long term.
Category
Segmental Pulmonary Venous Disease. Without a Focus on Pulmonary Hypertension
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes