Pulmonary artery atresia with major aortopulmonary collateral arteries
Presentation
Cyanosis and symptoms of congestive heart failure.
Patient Data
CT-scan scout view shows that the heart is enlarged with elevated cardiac apex suggestive of right ventricular enlargement. The main pulmonary artery segment is concave and the aortic arch is right-sided.
CT-scan images demonestrating that the right sided aortic arch gives three mirror image vessels with normal distribution. The desending aorta is seen running in front of right aspect of vertebral column. Pulmonary artery atresia is demonstrated. There are multiple major aorto-pulmonary collaterals (MAPCAs) supplying both lungs. A ventricular septal defect is seen underneath the aortic root.
Dilated SVC and retrograde flow of contrast to IVC and hepatic veins are seen.
Case Discussion
This is a case of pulmonary artery atresia with major aortopulmonary collaterals and ventricular septal defect.
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