1 国立循環器病研究センター教育推進部Department of Education and Research Promotion, Research and Development Initiative Center, National Cerebral and Cardiovascular Center ◇ Osaka, Japan
2 国立循環器病研究センター小児循環器科Division of Pediatric Cardiology, National Cerebral and Cardiovascular Center ◇ Osaka, Japan
先天性心疾患は，胎生期における心臓形態形成の過程が，胎児の遺伝子異常もしくは母体の環境要因により発症する多因子遺伝疾患と考えられている．心臓発生学を理解することは，先天性心疾患の形態診断，心疾患の病態把握，合併病変の予測，内科的治療方針の決定，外科手術手技の決定，長期予後の予測において大変重要である．本稿では，先天性心疾患の発症のメカニズムを理解するための胎生期の心臓形態形成のアウトラインについて解説する．近年目覚ましく進歩した，分子細胞生物学的手法および遺伝子改変マウスを用いた発生工学による先天性心疾患の発症メカニズムの知見は成書に譲ることとし，臨床医にとって基礎となる古典的な心臓形態形成の流れを中心に，図示してわかりやすく示す．
Congenital heart disease is a multifactorial inheritance disorder that is induced by genetic and environmental factors during morphogenesis of the cardiovascular system in the embryos. Cardiac embryology plays an important role in reaching a definitive diagnosis, understanding the pathophysiology of disease, selecting the most appropriate medical/surgical treatment, and predicting complications and prognosis. Therefore, a thorough understanding of the processes involved in cardiac embryology is essential for pediatric cardiologists and cardiac surgeons. In this review, cardiac embryology and cardiovascular morphogenesis are discussed to assist physicians in understanding the etiological mechanisms of congenital heart disease. The molecular and cellular mechanisms of congenital heart disease using genetically engineered mice are available in any expertized textbooks and journals. Thus, in this review, basic and comprehensive outlines of cardiac embryology and morphogenesis are presented using illustrations.
Key words: cardiovascular morphogenesis; congenital heart disease; cardiac embryology
© 2018 特定非営利活動法人日本小児循環器学会© 2018 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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This page was last modified on 2018-09-11T16:40:16.130+09:00
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