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Total anomalous pulmonary venous return. Review of 125 patients treated surgically
Insights
Surgical correction for total anomalous pulmonary venous return has a high mortality rate, especially in infants. Delaying surgery until at least six months of age improves survival for children with this congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Background:
- Total anomalous pulmonary venous return (TAPVR) is a severe congenital heart defect.
- Surgical correction is the primary treatment for TAPVR.
- Outcomes for TAPVR repair vary significantly based on patient factors.
Purpose of the Study:
- To analyze mortality rates in patients undergoing surgical correction of TAPVR.
- To identify factors influencing survival after TAPVR repair.
- To provide evidence-based recommendations for surgical timing.
Main Methods:
- Retrospective study of 125 patients with TAPVR.
- Analysis of overall mortality and mortality stratified by age at surgery.
- Evaluation of mortality based on lesion type and pulmonary vascular resistance.
Main Results:
- Overall mortality was 37%, with higher rates in younger patients (57% in infants <1 year).
- Infracardiac lesions had the highest mortality (62%), while cardiac defects had the lowest (30%).
- Pulmonary edema was the primary cause of death; survival correlated with pulmonary vascular resistance.
Conclusions:
- Surgical timing is critical for TAPVR repair outcomes.
- Delaying surgery until at least 6 months of age is recommended when feasible.
- Congestive heart failure may necessitate earlier surgical intervention.
Abstract:
One hundred twenty-five patients undergoing surgical correction of total anomalous pulmonary venous return were studied. The overall mortality was 37% and was related to age at the time operation was required. Mortality was 57% during the first year of life, 29% in patients between 13 and 24 months, and 15% in those between 2 and 10 years; no deaths occurred in those over 10 years. Mortality was highest in patients with infracardiac lesions (62%), and lowest in those with cardiac defects (30%). The major cause of death was pulmonary edema, and survival was closely related to the degree of increased pulmonary vascular resistance. Surgical treatment should be delayed until at least 6 months of age, but the development of congestive heart failure may necessitate earlier operation.