endocardial cushion defect

心内膜床欠損症 ECD

WordNet

1. desert (a cause, a country or an army), often in order to join the opposing cause, country, or army; "If soldiers deserted Hitlers army, they were shot" (同)desert
2. an imperfection in an object or machine; "a flaw caused the crystal to shatter"; "if there are any defects you should send it back to the manufacturer" (同)fault, flaw
3. an imperfection in a bodily system; "visual defects"; "this device permits detection of defects in the lungs"
4. a failing or deficiency; "that interpretation is an unfortunate defect of our lack of information" (同)shortcoming
5. protect from impact; "cushion the blow" (同)buffer, soften
6. a soft bag filled with air or a mass of padding such as feathers or foam rubber etc.
7. the layer of air that supports a hovercraft or similar vehicle
8. softened by the addition of cushions or padding (同)cushiony, padded

PrepTutorEJDIC

1. 〈C〉『欠点』,弱点,欠陥 / 〈U〉〈C〉《古》不足,欠乏 / (国・党・主義などから)離脱する,裏切って出る《+『from』+『名』》;離反して(…に)転向する《+『to』+『名』》
2. 『クッション』,座ぶとん / クッション状の物;(衝撃などを和らげるための)詰め物,まくら / 玉突台のクッション / …‘に'クッションを当てる;〈衝撃など〉'を'クッションを当てて和らげる

UpToDate Contents

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• 1. 房室（AV）管欠損の臨床症状、病態生理、および診断 clinical manifestations pathophysiology and diagnosis of atrioventricular av canal defects
• 2. 房室（AV）管欠損のマネージメントおよび転帰 management and outcome of atrioventricular av canal defects
• 3. 成人における心房中隔欠損症の臨床症状および診断 clinical manifestations and diagnosis of atrial septal defects in adults
• 4. 幼児や小児の単独の心室中隔欠損症：解剖、臨床的特徴、診断 isolated ventricular septal defects in infants and children anatomy clinical features and diagnosis
• 5. ダウン症：臨床的特徴および診断 down syndrome clinical features and diagnosis

English Journal

• Tbx20 acts upstream of Wnt signaling to regulate endocardial cushion formation and valve remodeling during mouse cardiogenesis.

• Cai X, Zhang W, Hu J, Zhang L, Sultana N, Wu B, Cai W, Zhou B, Cai CL.SourceDepartment of Developmental and Regenerative Biology, The Mindich Child Health and Development Institute, and The Black Family Stem Cell Institute, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA.
• Development (Cambridge, England).Development.2013 Aug;140(15):3176-87. doi: 10.1242/dev.092502. Epub 2013 Jul 3.
• Cardiac valves are essential to direct forward blood flow through the cardiac chambers efficiently. Congenital valvular defects are prevalent among newborns and can cause an immediate threat to survival as well as long-term morbidity. Valve leaflet formation is a rigorously programmed process consis
• PMID 23824573

• Use of decongestants during pregnancy and the risk of birth defects.

• Yau WP, Mitchell AA, Lin KJ, Werler MM, Hernández-Díaz S.AbstractPrevious studies suggested that early pregnancy exposure to specific oral decongestants increases the risks of several birth defects. Using January 1993-January 2010 data from the Slone Epidemiology Center Birth Defects Study, we tested those hypotheses among 12,734 infants with malformations (cases) and 7,606 nonmalformed control infants in the United States and Canada. Adjusted odds ratios and 95% confidence intervals were estimated for specific birth defects, with controlling for potential confounders. Findings did not replicate several hypotheses but did support 3 previously reported associations: phenylephrine and endocardial cushion defect (odds ratio = 8.0; 95% confidence interval: 2.5, 25.3; 4 exposed cases), phenylpropanolamine and ear defects (odds ratio = 7.8; 95% confidence interval: 2.2, 27.2; 4 exposed cases), and phenylpropanolamine and pyloric stenosis (odds ratio = 3.2; 95% confidence interval: 1.1, 8.8; 6 exposed cases). Hypothesis-generating analyses involving multiple comparisons identified a small number of associations with oral and intranasal decongestants. Accumulating evidence supports associations between first-trimester use of specific oral and possibly intranasal decongestants and the risk of some infrequent specific birth defects.
• American journal of epidemiology.Am J Epidemiol.2013 Jul 15;178(2):198-208. doi: 10.1093/aje/kws427. Epub 2013 Jul 3.
• Previous studies suggested that early pregnancy exposure to specific oral decongestants increases the risks of several birth defects. Using January 1993-January 2010 data from the Slone Epidemiology Center Birth Defects Study, we tested those hypotheses among 12,734 infants with malformations (cases
• PMID 23825167

• Complex cardiac defects after ethanol exposure during discrete cardiogenic events in zebrafish: Prevention with folic acid.

• Sarmah S, Marrs JA.SourceDepartment of Biology, Indiana University-Purdue University Indianapolis, Indianapolis, IN 46202.
• Developmental dynamics : an official publication of the American Association of Anatomists.Dev Dyn.2013 Jul 5. doi: 10.1002/dvdy.24015. [Epub ahead of print]
• BACKGROUND: Fetal alcohol spectrum disorder (FASD) describes a range of birth defects including various congenital heart defects (CHDs). Mechanisms of FASD-associated CHDs are not understood. Whether alcohol interferes with a single critical event or with multiple events in heart formation is not kn
• PMID 23832875

Japanese Journal

• ISP-14-7 HDlive image of fetal endocardial cushion defect(Group 14 Perinatology 4,IS Poster,International Session) :

• Aboellail Mohamed A.,Mashima Masato,Hanaoka Uiko,Kawanishi Kunihiro,Mori Nobuhiro,Kanenishi Kenji,Tanaka Hirokazu,Hata Toshiyuki
• 日本産科婦人科學會雜誌 67(2), 1041, 2015
• NAID 110009942189

• 64-Slice MDCT imaging of endocardial cushion defect associated with other cardiac and extracardiac abnormalities

• Oyama Noriko,Goto Daisuke,Sasaki Tsukasa,Tsutsui Hiroyuki,Tamaki Nagara,Terae Satoshi,Shirato Hiroki
• Journal of Cardiovascular Computed Tomography 4(3), 218-220, 2010-05
• NAID 120003169054

• 房室中隔欠損症の形態学

• 朴 仁三
• 日本小児循環器学会雑誌 = Acta cardiologica paediatrica Japonica 25(2), 110-117, 2009-03-01
• NAID 10024845320

Related Links

• Endocardial cushion defect: MedlinePlus Medical Encyclopedia

Endocardial cushion defect (ECD) is an abnormal heart condition. The walls separating all four chambers of the heart are poorly formed or absent. It is a ... Endocardial cushion defect occurs while a baby is still growing ...

• Endocardial Cushion Defects: Background, Pathophysiology ...

Predominant left-to-right shunting of blood through the heart occurs in these patients. In patients with partial defects, this occurs through the ostium primum atrial septal defect. When a complete endocardial cushion ...

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★リンクテーブル★

リンク元	「房室中隔欠損症」「ECD」
拡張検索	「complete endocardial cushion defect」
関連記事	「defect」「endocardial」「cushion」

「房室中隔欠損症」

　　[★]

英

atrioventricular septal defect AVSD

同

心内膜床欠損症 心内膜床欠損 endocardial cushion defect, ECD

総房室孔開存 common atrioventricular canal

関

心内膜床欠損症、完全型心内膜床欠損症

[show details]

房室中隔欠損症 : 約 31,500 件
房室中隔欠損 : 約 40,500 件
心内膜床欠損症 : 約 26,000 件
心内膜床欠損 : 約 32,100 件

疫学

• まれ？であるが、ダウン症候群では合併しやすい

病型

• 完全型　：心房中隔一次口閉鎖不全、僧帽弁異常、心室中隔閉鎖不全：ASD, VSD, MR, TR
• 不完全型：：心房中隔一次口閉鎖不全、僧帽弁異常：ASD, MR, TR

病態

• 完全型では肺血流増加

身体所見

• 聴診：収縮期逆流性雑音(MR,TR)、収縮期駆出性雑音(相対的PS)

検査

左室造影

• ガチョウの首(goose neck sign)

心電図

• 左軸変位：房室結節を含む刺激伝導系の後下方偏位による(参考3)　　　←　心房中隔欠損症では右軸偏位、房室中隔欠損症では左軸偏位
• PQ間隔延長
• 右室容量負荷による不完全右脚ブロック
• 一度房室ブロック？

心エコー

• 心室中隔の奇異性運動

診断

• 心エコー、心臓カテーテル検査による

予後

• 不完全型：
• 完全型：未治療のまま放置すれば、肺高血圧症により死亡。

参考

• 1. 心内膜欠損症

http://www.nurs.or.jp/~academy/igaku/s1/s153.htm

• 2.

http://www.geocities.jp/study_nasubi/c/c44.html

• 3. (2) 先天性心疾患の診断、病態把握、治療選択のための検査法の選択ガイドライン

http://www.j-circ.or.jp/guideline/pdf/JCS2010_hamaoka_h.pdf

「ECD」

　　[★]

• 心内膜床欠損症 endocardial cushion defect = 房室中隔欠損症 房室中隔欠損 atrioventricular septal defect AVSD
• 電子捕獲検出器 electron capture detector

「complete endocardial cushion defect」

　　[★] 完全型心内膜床欠損症

「defect」

　　[★]

• n.

• 欠損、欠損症、異常、欠陥、欠点

関

aberrant、aberrantly、aberration、abnormal、abnormality、abnormally、absence、agenesis、anomalous、anomalously、anomaly、defective、deficiency、deficient、deficit、deletion、deletional、deviant、drawback、extraordinarily、extraordinary、fault、flaw、inadequacy、miss、missing、morphological defect、shortcoming、unusual、unusually、weakness

　 　 　 　 　

「endocardial」

　　[★]

• adj.

• 心内膜の、内心膜性の

関

endocardium

「cushion」

　　[★]

• クッション