[Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries]
C Bonnet1, G Agnoletti, Y Boudjemline
1Service de cardiologie pédiatrique, groupe hospitalier Necker-Enfants malades, Paris. caroline.bonnet@chu-dijon.fr
Insights
Infants with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries can achieve positive outcomes despite small or absent central pulmonary arteries. This study highlights treatment strategies and outcome predictors in these complex congenital heart cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Outcomes
Context:
- Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) presents complex surgical challenges.
- The presence and size of central pulmonary arteries significantly influence treatment strategies and outcomes.
- Understanding predictors of success is crucial for optimizing care in affected infants.
Purpose:
- To identify treatment strategies and predictors of outcome in infants diagnosed with PA/VSD/MAPCAs.
- To analyze the impact of central pulmonary artery status on surgical repair and long-term results.
- To evaluate the effectiveness of staged versus one-stage complete repair approaches.
Summary:
- This review analyzed 47 infants with PA/VSD/MAPCAs, assessing outcomes based on central pulmonary artery presence (Type III confluent vs. Type IV absent) and repair strategies.
- Complete repair was achieved in 24 patients, with a favorable right/left ventricular pressure ratio (<0.5) in 79.3%. Staged repairs were more common (21/24 patients).
- Outcomes were positive even with small or absent central pulmonary arteries, suggesting that these factors do not preclude successful surgical management.
Impact:
- The findings suggest that surgical intervention can lead to positive outcomes in infants with PA/VSD/MAPCAs, irrespective of central pulmonary artery size or absence.
- This study provides valuable insights for surgical planning and risk stratification in complex congenital heart disease.
- Optimized treatment strategies can improve the quality of life for infants with these challenging conditions.
Introduction:
In order to establish the best strategy of treatment and predictors of outcome in infants with pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries, we reviewed our institutional experience concerning 47 infants.
Methods:
Inclusion criteria included an angiographic diagnosis of pulmonary atresia with ventricular septal defect and major aorto-pulmonary collateral arteries with or without central pulmonary arteries and a repair by the same surgeon. Thirty-one patients had confluent (type III) and 16 absent (type IV) central pulmonary arteries. Pulmonary arteries were considered to be adequate when they measured > or = 4 mm.
Results:
There were 8 deaths (17%). Complete repair was performed in 24 patients (18 in group III and 6 in group IV) with 79.3% had a right/left ventricular pressure <0.5, 16; 5%<1 and 4.2%>1. Eleven patients are waiting for complete repair and 4 will be operated on pulmonary arteries. One stage complete repair was performed in 3 patients, two and third stage repair (after unifocalisation or right ventricle to pulmonary arteries conduit) was attained in 21 patients. Among patients having had a complete repair and showing a right/left ventricular pressure <0.5, 88.9% had a pulmonary atresia with ventricular septal defect type III and 50% a pulmonary atresia with ventricular septal defect type IV. Only 56% of type III patients with a right/left ventricular pressure <0.5 had adequate central pulmonary arteries.
Conclusion:
In our study, the small size and the absence of central pulmonary arteries do not prevent a positive outcome.
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