Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension. (July 2015)

• Record Type: Journal Article
• Authors: Sanchez-de-Toledo, Joan; González-Peris, Sebastià; Gran, Ferran; Gregoraci, Angela; Ferreres, Joan Carles; Ruiz, Cèsar W.; Balcells, Joan; Abella, Raul F.
• Is Part Of: World journal for pediatric & congenital heart surgery. Volume 6:Number 3(2015:Jul.)
• Page Start: 480
• Page End: 483
• Publication Date: 2015-07
• Subjects: transposition of the great arteries -- pulmonary hypertension -- ECMO -- pulmonary interstitial glycogenosis; Pediatric cardiology -- Periodicals; Congenital heart disease in children -- Periodicals; Heart -- Abnormalities -- Surgery -- Periodicals; Heart -- Surgery -- Periodicals; Heart Defects, Congenital -- surgery -- Periodicals; Cardiac Surgical Procedures -- Periodicals; Child -- Periodicals; Adult -- Periodicals; 618.9212
• DOI: 10.1177/2150135115577433
• Languages: English
• ISSNs: 2150-1351

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.