Pulmonary Arterial Hypertension in Patients with Heterotaxy/Polysplenia Syndrome View Full Text


Ontology type: schema:Chapter      Open Access: True


Chapter Info

DATE

2016

AUTHORS

Akimichi Shibata , Keiko Uchida , Jun Maeda , Hiroyuki Yamagishi

ABSTRACT

Early progressive pulmonary arterial hypertension (PAH) is often observed in patients with heterotaxy/polysplenia especially who have an intracardiac systemic-to-pulmonary shunt. However, its etiology is uncertain and its management is not well established. There was only a Japanese report about PAH in consecutive patients with heterotaxy/polysplenia syndrome [1]. They seemed to develop pulmonary vascular obstructive disease earlier and more severe than expected, even in cases with only pre-tricuspid systemic-to-pulmonary shunt although more detailed analysis is required. More... »

PAGES

81-82

Book

TITLE

Etiology and Morphogenesis of Congenital Heart Disease

ISBN

978-4-431-54627-6
978-4-431-54628-3

Author Affiliations

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-4-431-54628-3_9

DOI

http://dx.doi.org/10.1007/978-4-431-54628-3_9

DIMENSIONS

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