Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair

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.