Pulmonary arterial hypertension in patients with heterotaxy/polysplenia syndrome

Akimichi Shibata, Keiko Uchida, Jun Maeda, Hiroyuki Yamagishi

研究成果: Chapter

抄録

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.

本文言語English
ホスト出版物のタイトルEtiology and Morphogenesis of Congenital Heart Disease
ホスト出版物のサブタイトルFrom Gene Function and Cellular Interaction to Morphology
出版社Springer Japan
ページ81-82
ページ数2
ISBN(電子版)9784431546283
ISBN(印刷版)9784431546276
DOI
出版ステータスPublished - 2016 1月 1

ASJC Scopus subject areas

  • 医学(全般)
  • 生化学、遺伝学、分子生物学(全般)

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