RT Journal Article
T1 Pulmonary arterial hypertension in children after neonatal arterial switch operation.
A1 Zijlstra, Willemijn Mh
A1 Elmasry, Ola
A1 Pepplinkhuizen, Shari
A1 Ivy, D Dunbar
A1 Bonnet, Damien
A1 Luijendijk, Paul
A1 Lévy, Marilyne
A1 Gavilan, Jose Luis
A1 Torrent-Vernetta, Alba
A1 Mendoza, Alberto
A1 Del Cerro, Maria Jesus
A1 Moledina, Shahin
A1 Berger, Rolf Mf
AB Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity with an estimated incidence of 0.6%-1.0%. Nevertheless, a clinical characterisation is lacking. We present an international cohort of children with PAH after neonatal ASO for TGA and describe epidemiology and clinical course. Data were collected of children with PAH after neonatal ASO (≤6 weeks after birth) for simple TGA without residual shunt defects, identified in four national paediatric PAH networks in Europe and one US referral centre. Twenty-five children were identified between 1989 and 2014. In 17 children (68%), PAH was detected  The occurrence of PAH after ASO for TGA represents a specific association. PAH onset may be early or late after ASO, with similar fatal course from first PAH detection. Mechanisms leading to PAH in this association are unknown, but may include abnormal prenatal pulmonary haemodynamics and/or genetic susceptibility. Routine, lifelong follow-up for children who undergo ASO for TGA should include screening for PAH.
YR 2017
FD 2017-01-23
LK http://hdl.handle.net/10668/10799
UL http://hdl.handle.net/10668/10799
LA en
DS RISalud
RD Apr 8, 2025