An Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries

World J Pediatr Congenit Heart Surg. 2015 Oct;6(4):630-9. doi: 10.1177/2150135115598559.


Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is a rare and complex congenital cardiac lesion that has historically carried a poor prognosis. With advancements in surgical management, we have seen an improvement in the outcomes for children affected by this disease. However, this population continues to present challenges due to the complex anatomy and physiology associated with PA/VSD/MAPCA. This summary of material presented during one of the nursing sessions of the 2014 Meeting of the Pediatric Cardiac Intensive Care Society provides an overview for those in cardiac intensive care units who do not have a large experience with this lesion. We will review the anatomy, physiology, surgical approach, postoperative management strategies, and cardiac catheter intervention options for PA/VSD/MAPCAs. We will also discuss recent innovations that may lead to continued improvement in outcomes for this challenging patient population.

Keywords: major aortopulmonary collaterals; pulmonary atresia; unifocalization; ventricular septal defect.

Publication types

  • Review

MeSH terms

  • Aorta, Thoracic / abnormalities*
  • Aorta, Thoracic / physiopathology
  • Aorta, Thoracic / surgery
  • Cardiac Surgical Procedures / methods*
  • Collateral Circulation*
  • Heart Septal Defects / physiopathology
  • Heart Septal Defects / surgery*
  • Humans
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / physiopathology
  • Pulmonary Artery / surgery
  • Pulmonary Atresia / physiopathology
  • Pulmonary Atresia / surgery*

Supplementary concepts

  • Pulmonary Atresia With Ventricular Septal Defect