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 神奈川県立こども医療センター新生児科Department of Neonatology, Kanagawa Children’s Medical Center ◇ Kanagawa, Japan
肺動脈弁欠損を伴うFallot四徴症（TF/APV）は肺動脈弁欠損症候群（APVS）とも呼ばれ，しばしば予後不良である．本症は周産期医療における胎児心エコー検査上重要な疾患である．Bis-diamineは強力なteratogenでラットに先天性心疾患と胸腺低形成を生じる．Bis-diamine 200 mgを妊娠9日と10日にラット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胎仔に各種先天性心疾患（90％），胸腺低形成（100％）を生じた．肺動脈弁欠損を伴うFallot四徴症が46例にあり，その記録で次の所見が得られた．全例大きい心室中隔欠損があり，太い大動脈が心室中隔欠損に騎乗して起始していた．右室漏斗部はやや狭く，肺動脈弁は痕跡的または全く欠損し，動脈管は欠損していた．主肺動脈と左右肺動脈は拡大し，左右肺門部で最も大きく，気管と気管支を圧排して閉塞していた．これら肺動脈弁欠損を伴うFallot四徴症の生体内断面図を胎児エコーモデル図譜として提示する．
Bis-diamine was administered at a dose of 200 mg/kg to 40 pregnant rats on the 9th and 10th days of gestation. Following cervical dislocation of the rats on the 21st day, the fetuses were delivered by cesarean section and were frozen immediately in acetone cooled to −76°C with dry ice. Each frozen fetal thorax was cut transversely and the sectioned surface was serially photographed with a stereoscopic microscope (Wild Photomacroscope) at intervals of 500 µm. Of the 330 fetuses, 46 had tetralogy of Fallot and absent pulmonary valve (TF/APV).
In those with TF/APV, a large aorta was overriding a large ventricular septal defect. The right ventricular infundibulum was minimally stenotic, and the pulmonary valve was absent with a small residual ridge on the valve ring. The pulmonary arteries (PAs) were generally enlarged, particularly at the right and left pulmonary hilar regions, and compressed and obstructed the adjacent trachea and bronchi. The diameter of the enlarged PAs was equal to or as much as twice the diameter of the aorta. In normal fetuses, the diameter of the right and left PAs was half the aortic diameter. The bronchial obstruction in TF/APV extended peripherally. Pericardial and pleural effusions were mildly increased. Both ventricles were mildly enlarged. The thymus was either hypoplastic or absent. These pictures show wide-spread obstruction of the fetal trachea and bronchi in TF/APV.
Key words: tetralogy of Fallot; absent pulmonary valve; bronchomalacia; bronchial obstruction; neonatal respiratory failure
© 2019 特定非営利活動法人日本小児循環器学会© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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This page was last modified on 2019-10-09T17:54:11.000+09:00
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