Related Experiment Videos
Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era
1Division of Cardiopulmonary Surgery, Oregon Health Sciences University, Portland 97201-3098.
Insights
Total anomalous pulmonary venous connection (TAPVC) repair in infants has improved outcomes. Early surgical intervention with specific techniques leads to low mortality and excellent long-term survival rates for this challenging congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Neonatal Critical Care
Background:
- Total anomalous pulmonary venous connection (TAPVC) is a complex congenital heart defect in newborns and infants.
- Despite advancements, surgical correction remains challenging.
Purpose of the Study:
- To evaluate the outcomes of primary total correction for TAPVC in neonates and young infants.
- To assess early and late mortality, reoperation rates, and long-term survival.
Main Methods:
- Retrospective review of 30 patients undergoing primary total correction of TAPVC since 1981.
- Surgical approach included early intervention, profound hypothermia with circulatory arrest, cardioplegic preservation, and wide anastomosis.
- Mean follow-up of 47 months.
Main Results:
- Operative mortality was 13%, with deaths occurring in critically ill, preoperatively ventilated patients.
- The 7-year survival rate was 79% +/- 8%.
- 91% +/- 6% of patients were reoperation-free at 7 years, with only one symptomatic survivor.
Conclusions:
- A consistent surgical approach utilizing early intervention and specific techniques has led to improved outcomes for TAPVC.
- Low early mortality, low attrition, and excellent late results are achievable.
- This strategy offers a favorable prognosis for infants with TAPVC.
Abstract:
Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results.