Pulmonary arterial hypertension in patients with heterotaxy/polysplenia syndrome

Akimichi Shibata; Keiko Uchida; Jun Maeda; Hiroyuki Yamagishi

doi:10.1007/978-4-431-54628-3_9

Pulmonary arterial hypertension in patients with heterotaxy/polysplenia syndrome

Akimichi Shibata, Keiko Uchida, Jun Maeda, Hiroyuki Yamagishi

Research output: Chapter in Book/Report/Conference proceeding › Chapter

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.

Original language	English
Title of host publication	Etiology and Morphogenesis of Congenital Heart Disease
Subtitle of host publication	From Gene Function and Cellular Interaction to Morphology
Publisher	Springer Japan
Pages	81-82
Number of pages	2
ISBN (Electronic)	9784431546283
ISBN (Print)	9784431546276
DOIs	https://doi.org/10.1007/978-4-431-54628-3_9
Publication status	Published - 2016 Jan 1

Keywords

BMPR2
Gene
TGF-β

ASJC Scopus subject areas

Medicine(all)
Biochemistry, Genetics and Molecular Biology(all)

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10.1007/978-4-431-54628-3_9

Cite this

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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.",

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AU - Shibata, Akimichi

AU - Uchida, Keiko

AU - Maeda, Jun

AU - Yamagishi, Hiroyuki

N1 - Publisher Copyright: © The Author(s) 2016.

PY - 2016/1/1

Y1 - 2016/1/1

N2 - 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.

AB - 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.

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KW - TGF-β

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ER -

Pulmonary arterial hypertension in patients with heterotaxy/polysplenia syndrome

Abstract

Keywords

ASJC Scopus subject areas

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