title: Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension.
abstract: Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that underwent successful arterial switch procedure. A lung biopsy obtained during the surgical procedure showed pulmonary interstitial glycogenosis, a reversible condition. Concerns over left ventricle deconditioning after ECMO could be minimized with appropriate management and monitoring of the ductus arteriosus and appropriate timing of surgery.
authors.affiliation: Department of Critical Care Medicine, Cardiac Intensive Care Division, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA joansdt@gmail.com.
authors.forename: Joan
authors.initials: J
authors.lastname: Sanchez-de-Toledo
authors.affiliation: Department of Pediatric Intensive Care Medicine, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Sebastià
authors.initials: S
authors.lastname: González-Peris
authors.affiliation: Department of Pediatric Cardiology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Ferran
authors.initials: F
authors.lastname: Gran
authors.affiliation: Department of Neonatology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Angela
authors.initials: A
authors.lastname: Gregoraci
authors.affiliation: Department of Pathology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Joan Carles
authors.initials: JC
authors.lastname: Ferreres
authors.affiliation: Department of Neonatology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Cèsar W
authors.initials: CW
authors.lastname: Ruiz
authors.affiliation: Department of Pediatric Intensive Care Medicine, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Joan
authors.initials: J
authors.lastname: Balcells
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Raul F
authors.initials: RF
authors.lastname: Abella
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods*; D004374:Ductus Arteriosus, Patent / Q000601:surgery*; D015199:Extracorporeal Membrane Oxygenation / Q000379:methods*; D006008:Glycogen Storage Disease / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007231:Infant, Newborn; D010547:Persistent Fetal Circulation Syndrome / Q000601:surgery*; D014188:Transposition of Great Vessels / Q000601:surgery*
keywords: ECMO; pulmonary hypertension; pulmonary interstitial glycogenosis; transposition of the great arteries