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 34(2): 55-62 (2018)
doi:10.9794/jspccs.34.55

ReviewReview

ラット胎仔先天性心疾患の断面像胎児心エコーのための22q11.2欠失症候群モデル動物図譜としてCross-sectional Morphology of Congenital Heart Disease: Fetal Echocardiography Indicating Chromosome 22q11.2 Deletion Syndrome in Rat Model

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

発行日:2018年3月1日Published: March 1, 2018
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Bis-diamineはラットに強力なteratogenで高率にヒト染色体22q11欠失症候群類似の先天性心疾患と胸腺低形成を生じさせるが,作用機序の詳細は不明である.Bis-diamine 200 mgを妊娠10日のラット40匹に胃内注入し,満期21日目に胎仔を全身急速凍結法,凍結ミクロトーム,実体顕微鏡(Wild M400)を用いて0.5 mm毎の胸部横断面を連続写眞で記録した.330胎仔に括弧内の率で次の先天性心疾患が生じた.各種のFallot四徴症(68%),総動脈幹残遺(18%),大動脈弓離断(2%),心室中隔欠損(3%),共通房室弁口(2%)である.Fallot四徴症では通常型(16%),肺動脈弁欠損型(14%),肺動脈弁閉鎖型(38%)があった.これらの心疾患には右側大動脈弓,鎖骨下動脈起始異常,血管輪などが合併した.肺動脈弁欠損のFallot四徴症では左右肺動脈の動脈瘤状の拡張があり,肺門部で気管支を圧迫して閉塞していた.同時に心房心室の拡大,心嚢液の貯留が生じた.共通房室弁口では両心房,両心室の拡大,房室弁の肥厚(異形成),心嚢液貯留があった.ヒトと異なり体肺側副血管はなかった.これらの胎生期先天性心疾患の生体内断面図を染色体22q11.2欠失症候群の胎児心エコーモデル図譜として提示する.

Bis-diamine, a potent teratogen, induces conotruncal diseases and thymic hypoplasia in rats similar to chromosome 22q11 deletion syndrome in humans. The precise mechanism underlying cardiac teratogenic effects of bis-diamine remains unknown. On the 10th day of gestation, 200 mg bis-diamine was administered to 40 pregnant rats. The fetuses were delivered by Cesarean section after the cervical dislocation of rats on the 21st day of gestation and were immediately frozen in acetone cooled to −76°C using dry ice. Each frozen thorax of the fetus was transversely cut, and the section surface was serially photographed with a stereoscopic microscope (Wild M400 Photomacroscope) at every 500 µm. The following cardiac diseases were recorded in 330 fetuses: Tetralogy of Fallot (16%), Tetralogy of Fallot with absent pulmonary valve (14%), Tetralogy of Fallot with pulmonary valvular and infundibular atresia (38%), truncus arteriosus (10%), ventricular septal defects (3%), interrupted aortic arch type B (2%), and atrioventricular septal defects (2%). Fetuses with the Tetralogy of Fallot and absent pulmonary valve showed pulmonary arteries aneurysmally dilated at the right and left pulmonary hila, obstructing the adjacent bronchus. Fetuses with atrioventricular canal defects showed thick nodular atrioventricular valve leaflets and signs of congestive heart failure, including the enlargement of all cardiac chambers and increased pericardial effusion. In addition, vascular diseases (right aortic arch, aberrant subclavian artery, and vascular ring) complicated the cardiac diseases. Thymus hypoplasia occurred in all fetuses (100%), while some fetuses developed diaphragmatic hernia (10%). No aortopulmonary collateral artery was noticed. These cross-sectional morphologies represent fetal echocardiographic diagnoses indicating congenital heart diseases associated with chromosome 22q11.2 deletion syndrome in rat model.

Key words: Tetralogy of Fallot; absent pulmonary valve; truncus arteriosus; fetal echocardiography; chromosome 22q11.2 deletion syndrome

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