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.
LucianiGBChangACStarnesVA. Surgical repair of transposition of the great arteries in neonates with persistent pulmonary hypertension. Ann Thorac Surg. 1996;61
(3):800–805.
2.
KumarATaylorGPSandorGGPattersonMW. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum. Br Heart J. 1993;69
(5):442–445.
3.
WernovskyGMayerJEJrJonasRA. Factors influencing early and late outcome of the arterial switch operation for transposition of the great arteries. J Thoracic Cardiovasc Surg. 1995;109
(2):289–301.
4.
RoofhoftMTBergmanKAWaterbolkTWEbelsTBarteldsBBergerRM. Persistent pulmonary hypertension of the newborn with transposition of the great arteries. Ann Thorac Surg. 2007;83
(4):1446–1450.
5.
Albert BrotonsDGirona ComasJCasaldàliga FerrerJRoqueta MasJPerapoch LópezJMurtra FerreM. Transposition of the great arteries and pulmonary hypertension: inhaled nitric oxide as a therapy and early surgical correction. An Esp Pediatr. 1997;47
(6):633–635.
6.
El-SegaierMHellström-WestasLWettrellG. Nitric oxide in neonatal transposition of the great arteries. Acta Paediatr. 2005;94
(7):912–916.
7.
Avila-AlvarezABravo-LagunaMCBronteLDDel CerroMJ. Inhaled iloprost as a rescue therapy for transposition of the great arteries with persistent pulmonary hypertension of the newborn. Pediatr Cardiol. 2013;34
(8):2027–2029.
8.
GoissenCGhyselenLTorneuxP. Persistent pulmonary hypertension of the newborn with transposition of the great arteries: successful treatment with bosentan. Eur J Pediatr. 2008;167
(4):437–440.
9.
McNamaraPJShivanandaSPSahniMFreemanDTaddioA. Pharmacology of milrinone in neonates with persistent pulmonary hypertension of the newborn and suboptimal response to inhaled nitric oxide. Pediatr Crit Care Med. 2013;14
(1):74–84.
10.
JaillardSBelliERakzaT. Preoperative ECMO in transposition of the great arteries with persistent pulmonary hypertension. Ann Thorac Surg. 2005;79
(6):2155–2158.
11.
CanakisAMCutzEMansonDO'BrodovichH. Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease. Am J Respir Crit Care Med. 2002;165
(11):1557–1565.
DeutschGHYoungLR. Histologic resolution of pulmonary interstitial glycogenosis. Pediatr Dev Pathol. 2009;12
(6):475–480.
14.
RadmanMRGoldhoffPJonesKD. Pulmonary interstitial glycogenosis: an unrecognized etiology of persistent pulmonary hypertension of the newborn in congenital heart disease. Pediatr Cardiol. 2013;34
(5):1254–1257.
15.
AlkhorayyefARyersonLChanAPhilliposELacsonAAdatiaI. Pulmonary interstitial glycogenosis associated with pulmonary hypertension and hypertrophic cardiomyopathy. Pediatr Cardiol. 2013;34
(2):462–466.
16.
TrittenweinGFürstGGolejJ. Preoperative ECMO in congenital cyanotic heart disease using the AREC system. Ann Thorac Surg. 1997;63
(5):1298–1302.
17.
MasutaniSSekiMTaketazuMSenzakiH. Successful management of the persistent pulmonary hypertension of the newborn with transposition of the great arteries by restricted patency of the ductus arteriosus: a simple and rational novel strategy. Pediatr Cardiol. 2009;30
(7):1003–1005.
18.
SharmaRChoudharySKBhanA. Left ventricle is better suited as pulmonary ventricle in simple transposition with severe pulmonary hypertension. Ann Thorac Surg. 2002;74
(5):1612–1615.
