TY - JOUR
T1 - Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension
AU - Sanchez-de-Toledo, Joan
AU - González-Peris, Sebastià
AU - Gran, Ferran
AU - Gregoraci, Angela
AU - Ferreres, Joan C.arles
AU - Ruiz, Cèsar W.
AU - Balcells, Joan
AU - Abella, Raul F.
PY - 2015/7/1
Y1 - 2015/7/1
N2 - © The Author(s) 2015. 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.
AB - © The Author(s) 2015. 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.
KW - ECMO
KW - pulmonary hypertension
KW - pulmonary interstitial glycogenosis
KW - transposition of the great arteries
UR - https://www.scopus.com/pages/publications/84991259685
U2 - 10.1177/2150135115577433
DO - 10.1177/2150135115577433
M3 - Article
SN - 2150-136X
VL - 6
SP - 480
EP - 483
JO - World journal for pediatric & congenital heart surgery
JF - World journal for pediatric & congenital heart surgery
IS - 3
ER -