Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension

World J Pediatr Congenit Heart Surg. 2015 Jul;6(3):480-3. doi: 10.1177/2150135115577433.

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.

Keywords: ECMO; pulmonary hypertension; pulmonary interstitial glycogenosis; transposition of the great arteries.

Publication types

  • Case Reports

MeSH terms

  • Cardiac Surgical Procedures / methods*
  • Ductus Arteriosus, Patent / surgery*
  • Extracorporeal Membrane Oxygenation / methods*
  • Glycogen Storage Disease / etiology*
  • Glycogen Storage Disease / therapy
  • Humans
  • Infant, Newborn
  • Persistent Fetal Circulation Syndrome / surgery*
  • Transposition of Great Vessels / surgery*