Factors associated with mortality and reoperation in 377 children with total anomalous pulmonary venous connection
Tara Karamlou1, Rebecca Gurofsky, Eisar Al Sukhni
1Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8.
Insights
Mortality after total anomalous pulmonary venous connection repair has significantly decreased, but remains high for infants with cardiac types or venous obstruction. Improved perioperative care enhances survival, yet unfavorable anatomy is still a key factor.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Medical Outcomes Research
Background:
- Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart defect.
- Understanding historical trends in TAPVC outcomes is crucial for improving surgical strategies.
Purpose of the Study:
- To analyze era-specific changes in mortality and reoperation rates following surgical repair of TAPVC in children.
- To identify risk factors influencing survival and reoperation after TAPVC repair.
Main Methods:
- Retrospective review of 377 children with TAPVC treated between 1946 and 2005.
- Multivariable parametric regression models were used to assess incidence and risk factors for death and reoperation.
- Analysis included patient demographics, connection types, presence of obstruction, and perioperative management.
Main Results:
- Overall survival post-TAPVC repair improved significantly over time, reaching 97% in recent years.
- Risk factors for postrepair death included cardiac connection type, earlier repair year, younger age, epinephrine use, and postoperative pulmonary venous obstruction.
- Freedom from reoperation was 82% at 11 years, with increased risk associated with mixed connection type and postoperative pulmonary venous obstruction.
- Risk-adjusted survival for a neonate with unfavorable anatomy in 2005 was 37%, compared to 96% for a favorable case repaired at 1 year.
Conclusions:
- While mortality after TAPVC repair has decreased, it remains elevated in specific patient groups.
- Younger age at repair, cardiac connection type, and pulmonary venous obstruction are significant determinants of mortality.
- Despite advancements in perioperative care, unfavorable anatomical features continue to impact long-term survival after TAPVC repair.
Background:
We sought to determine era-specific changes in the incidence of mortality and reoperation in children with total anomalous pulmonary venous connection.
Methods And Results:
We reviewed the records of 377 children presenting from 1946 to 2005 with total anomalous pulmonary venous connection. Multivariable parametric regression models determined the incidence and risk factors for death and reoperation after repair. Pulmonary venous connection was supracardiac in 44%, infracardiac in 26%, cardiac in 21%, and mixed in 9%. Pulmonary venous obstruction was present in 48% at presentation, most frequently with infracardiac connection type (P<0.001). In total, 327 patients were repaired (median age, 1.7 months). Overall survival from repair was 65+/-6% at 14 years, with a current survival of 97%. Significant (P<0.01) incremental risk factors for postrepair death were cardiac connection type, earlier operation year, younger age at repair, use of epinephrine postoperatively, and postoperative pulmonary venous obstruction. More recent operation year was associated with younger age at repair (P<0.001), decreased use of deep hypothermic circulatory arrest (P<0.001), and use of specific drugs postoperatively (P<0.001). Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2005 is 37% (95% CI, 8 to 80) compared with 96% (95% CI, 91 to 99) for a patient with favorable morphology repaired at 1 year of age. Freedom from reoperation was 82+/-6% at 11 years after repair, with increased risk associated with mixed connection type (P=0.04) and postoperative pulmonary venous obstruction (P<0.001).
Conclusions:
Mortality after total anomalous pulmonary venous connection repair has decreased but remains highest in young patients and in those with cardiac connection type or pulmonary venous obstruction. Unfavorable anatomic characteristics remain important determinants of postrepair survival despite improved perioperative care.
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