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Pulmonary arterial hypertension in children after neonatal arterial switch operation.

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2017-01-23

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Zijlstra, Willemijn Mh
Elmasry, Ola
Pepplinkhuizen, Shari
Ivy, D Dunbar
Bonnet, Damien
Luijendijk, Paul
Lévy, Marilyne
Gavilan, Jose Luis
Torrent-Vernetta, Alba
Mendoza, Alberto

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Abstract

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.

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MeSH Terms

Arterial Switch Operation
Child, Preschool
Europe
Female
Follow-Up Studies
Humans
Hypertension, Pulmonary
Incidence
Infant
Male
Retrospective Studies
Time Factors
Transposition of Great Vessels
United States

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