title: An Overview of Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.
abstract: 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.
authors.affiliation: Pediatric Cardiac Intensive Care Unit, Lucile Packard Children's Hospital, Palo Alto, CA, USA lpresnell@gmail.com.
authors.forename: Laura B
authors.initials: LB
authors.lastname: Presnell
authors.affiliation: The Heart Center, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Blankenship
authors.affiliation: The Heart Center, Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Sharon L
authors.initials: SL
authors.lastname: Cheatham
authors.affiliation: C.S. Mott Children's Hospital, University of Michigan Congenital Heart Center, Hospital Drive, Ann Arbor, MI, USA.
authors.forename: Gabe E
authors.initials: GE
authors.lastname: Owens
authors.affiliation: Research in Patient Services and Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Sandra L
authors.initials: SL
authors.lastname: Staveski
mesh_terms: D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D006348:Cardiac Surgical Procedures / Q000379:methods*; D003097:Collateral Circulation; D006343:Heart Septal Defects / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D018633:Pulmonary Atresia / Q000503:physiopathology / Q000601:surgery*
keywords: major aortopulmonary collaterals; pulmonary atresia; unifocalization; ventricular septal defect