日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 37(2): 88-95 (2021)
doi:10.9794/jspccs.37.88

ReviewReview

総動脈幹遺残ラット胎仔心臓血管断面の天然色図譜Natural Color Pictures of Cross-sections of Fetal Rat Heart with Trunks Arteriosus

1東京女子医科大学循環器小児科Department of Pediatric Cardiology, Tokyo Women’s Medical University ◇ Tokyo, Japan

2聖マリア病院新生児科Department of Neonatology, St. Mary’s Hospital ◇ Fukuoka, Japan

発行日:2021年8月1日Published: August 1, 2021
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総動脈幹遺残は心臓流出路の円錐から動脈幹の中隔形成不全に起因する先天性心疾患である.新生時期に重篤な心不全に陥る致死的な疾患であるため胎児エコー診断が重要である.染色体22q11.2欠失症候群の31%に総動脈幹遺残を合併し,妊娠ラットへのbis-diamine投与により胎仔に染色体22q11.2欠失症候群に酷似した先天性疾患を生じる.本稿ではbis-diamineによりラットに生じた総動脈幹遺残の胎生期心臓血管断面像を検討した.妊娠9日目と10日目にbis-diamineを投与し,満期21日目に全身急速凍結法を用いて胎仔を固定した.ミクロトームで薄切した胸部横断面を実体顕微鏡下に連続写真で記録した.観察した胎仔の90%に心疾患があり.17%に総動脈幹遺残が認められた.代表的な4例の総動脈幹遺残胸部横断面の図譜を詳細に検討したところ,心室中隔欠損や右室流出路と主肺動脈の欠損,弁異形成や逸脱,4弁性などの総動脈幹弁の形態異常,さらに総動脈幹から起始する主肺動脈または左右肺動脈が確認された.その他右側総動脈幹弓や右鎖骨下動脈起始異常による大血管とその分枝の走行以上も明確に描出された.Bis-diamine投与による総動脈幹遺残モデルラットの図譜は,人の総動脈幹遺残に対する胎児エコー診断の一助となる可能性がある.

Truncus arteriosus is a congenital heart disease that is caused by insufficient septation of the cardiac outflow tract and arterial truncus. Fetal echocardiographic diagnosis of this disease is important because of its high neonatal mortality. About 30% of chromosome 22q11.2 deletion syndrome is associated with truncus arteriosus. Bis-diamine, administered to pregnant rats, induces these congenital anomalies similar to those seen in chromosome 22q11.2 deletion syndrome. This is a study of cross-section of the truncus arteriosus which is induced by bis-diamine in fetal rats. Bis-diamine was administered to pregnant rats on 9th and 10th day, and full-term fetuses were fixed with a whole-body freezing method. The frozen fetal thorax was cut with a freezing microtome transversely, and the cut surface was photographed serially for subsequent stereo-microscopic study.

Congenital heart disease was present in 90% of the studied hearts, including truncus arteriosus in 17%. Study of four typical cases of truncus arteriosus in cross-section revealed ventricular septal defect, absence of the right ventricular outflow tract, and proximal main pulmonary artery. In addition, there were dysplasia of the truncal valve including prolapse, thickening, quadricuspid-leaflets, large and small leaflets, and origin of the distal main pulmonary artery or right and left pulmonary artery from the truncus arteriosus. Moreover, right-sided truncus arteriosus arch and anomalous origin of the right subclavian artery, were clearly shown. These model pictures of truncus arteriosus will be helpful in clinical fetal echocardiographic diagnosis of truncus arteriosus.

Key words: truncus arteriosus; truncal valve dysplasia; truncal valve regurgitation; fetal echocardiography; chromosome 22q11.2 deletion syndrome

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This page was last modified on 2021-08-03T20:09:32.000+09:00


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