Pulmonary arterial hypertension in patients with heterotaxy/polysplenia syndrome

Akimichi Shibata, Keiko Uchida, Jun Maeda, Hiroyuki Yamagishi

Research output: Chapter in Book/Report/Conference proceedingChapter


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 languageEnglish
Title of host publicationEtiology and Morphogenesis of Congenital Heart Disease
Subtitle of host publicationFrom Gene Function and Cellular Interaction to Morphology
PublisherSpringer Japan
Number of pages2
ISBN (Electronic)9784431546283
ISBN (Print)9784431546276
Publication statusPublished - 2016 Jan 1


  • BMPR2
  • Gene
  • TGF-β

ASJC Scopus subject areas

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


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