総動脈管 TA

同: persistent

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/12/03 06:17:30」(JST)

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The truncus arteriosus is a structure that is present during embryonic development. It is an arterial trunk that originates from both ventricles of the heart that later divides into the aorta and the pulmonary trunk.

Structure

The truncus arteriosus and bulbus cordis are divided by the aorticopulmonary septum. The truncus arteriosus gives rise to the ascending aorta and the pulmonary trunk. The caudal end of the bulbus cordis gives rise to the smooth parts (outflow tract) of the left and right ventricles (aortic vestibule & conus arteriosus respectively).^[1] The cranial end of the bulbus cordis (also known as the conus cordis) gives rise to the aorta and pulmonary trunk with the truncus arteriosus.

This makes its appearance in three portions.

Two distal ridge-like thickenings project into the lumen of the tube: the truncal and bulbar ridges.^[2] These increase in size, and ultimately meet and fuse to form a septum (aorticopulmonary septum), which takes a spiral course toward the proximal end of the truncus arteriosus. It divides the distal part of the truncus into two vessels, the aorta and pulmonary artery, which lie side by side above, but near the heart the pulmonary artery is in front of the aorta.
Four endocardial cushions appear in the proximal part of the truncus arteriosus in the region of the future semilunar valves; the manner in which these are related to the aortic septum is described below.
Two endocardial thickenings—anterior and posterior—develop in the bulbus cordis and unite to form a short septum; this joins above with the aortic septum and below with the ventricular septum. The septum grows down into the ventricle as an oblique partition, which ultimately blends with the ventricular septum in such a way as to bring the bulbus cordis into communication with the pulmonary artery, and through the latter with the sixth pair of aortic arches; while the left ventricle is brought into continuity with the aorta, which communicates with the remaining aortic arches.

Liver with the septum transversum. Human embryo 3 mm. long.
Illustration of truncus arteriosus in a fully formed heart

Clinical significance

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Truncus arteriosus video

Failure of the truncus arteriosus to close results in the condition known as persistent truncus arteriosus, a rare congenital heart defect. This is often just referred to as truncus arteriosus. Other pathologies of the truncus arteriosus include transposition of the great vessels (arteries in this case), and tetralogy of Fallot.^[3]

References

This article incorporates text in the public domain from the 20th edition of Gray's Anatomy (1918)

^ Le, T., Bhushan, V., et al. 'First Aid for the USMLE STEP1'. 2009.
^ Le, Tao; Bhushan, Vikas; Vasan, Neil (2010). First Aid for the USMLE Step 1: 2010 20th Anniversary Edition. USA: The McGraw-Hill Companies, Inc. p. 123. ISBN 978-0-07-163340-6.
^ Le, Tao; Bhushan, Vikas; Vasan, Neil (2010). First Aid for the USMLE Step 1: 2010 20th Anniversary Edition. USA: The McGraw-Hill Companies, Inc. p. 123. ISBN 978-0-07-163340-6.

External links

Truncus Arteriosus - Stanford Children's Health
Embryology at UNSW Notes/heart3b
Overview at mcgill.ca
Description and diagram at umich.edu

UpToDate Contents

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1. 総動脈幹 truncus arteriosus
2. 新生児におけるチアノーゼの心臓の原因 cardiac causes of cyanosis in the newborn
3. 先天性および小児冠動脈異常 congenital and pediatric coronary artery abnormalities
4. 新生児における先天性心疾患（CHD）：重症ＣＨＤの症状およびスクリーニング congenital heart disease chd in the newborn presentation and screening for critical chd
5. DiGeorge (22q11.2 deletion) syndrome: Management and prognosis

English Journal

Truncus arteriosus communis in combination with cor triatriatum sinsitrum.

Klusmeier E1, Haas NA1, Sandica E2.
Cardiology in the young.Cardiol Young.2016 Jan;26(1):194-6. doi: 10.1017/S1047951115000414. Epub 2015 Apr 30.
Truncus arteriosus communis is a rare CHD, accounting for only 1% of all congenital cardiac abnormalities. It has been associated with other malformations of the heart, mainly truncal valve (bicuspid/quadricuspid) and aortic arch abnormalities such as right, interrupted, and hypoplastic aortic arch.
PMID 25925708

Dysregulated endocardial TGFβ signaling and mesenchymal transformation result in heart outflow tract septation failure.

Ma MC1, Li P1, Shen H1, Estrada KD1, Xu J2, Kumar SR3, Sucov HM4.
Developmental biology.Dev Biol.2016 Jan 1;409(1):272-6. doi: 10.1016/j.ydbio.2015.09.021. Epub 2015 Oct 29.
Heart outflow tract septation in mouse embryos carrying mutations in retinoic acid receptor genes fails with complete penetrance. In this mutant background, ectopic TGFβ signaling in the distal outflow tract is responsible for septation failure, but it was uncertain what tissue was responsive to ec
PMID 26522286

Anatomic repair of complex transposition with en bloc rotation of the truncus arteriosus: 10-year experience†.

Mair R1, Sames-Dolzer E2, Innerhuber M2, Tulzer A2, Grohmann E2, Tulzer G2.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.Eur J Cardiothorac Surg.2016 Jan;49(1):176-82. doi: 10.1093/ejcts/ezv056. Epub 2015 Feb 19.
OBJECTIVES: Transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction are commonly called complex transposition. The traditional method of repair is the Rastelli procedure. Aortic translocation (Nikaidoh 1984) provides a more anatomic repair of thi
PMID 25700698

Japanese Journal

染色体22q11欠失症：発見と合併心疾患

日本小児循環器学会雑誌 33(1), 3-9, 2017
NAID 130005530257

総動脈幹遺残修復術後の低酸素血症を契機に診断された, 左上大静脈遺残を伴わないPartially unroofed coronary sinusの1例

心臓 48(3), 312-316, 2016
NAID 130005439781

小児大動脈弁疾患に対するグルタールアルデヒド処理自己心膜を用いた大動脈弁形成術

日本小児循環器学会雑誌 31(6), 329-337, 2015
NAID 130005116619

Related Pictures

★リンクテーブル★

リンク元	「先天性心疾患」「22q11」「総動脈幹」「総動脈管」「動脈幹」
関連記事	「arteriosus」

「先天性心疾患」

Truncus arteriosus
	Heart of human embryo of about fourteen days. (Truncus arteriosis visible at top.)
	Diagrams to illustrate the transformation of the bulbus cordis. Ao. Truncus arteriosus. Au. Atrium. B. Bulbus cordis. RV. Right ventricle. LV. Left ventricle. P. Pulmonary artery.
Details
Gives rise to	aorta, pulmonary artery
System	Cardiovascular system
Identifiers
MeSH	A07.541.278.930
Code	TE E5.11.1.8.1.0.4
	Anatomical terminology [edit on Wikidata]

　　[★]

英: congenital heart disease, CHD
同: 先天性心血管奇形 congenital cardiovascular anomaly
関: 先天性心奇形

先天性心疾患.xls

先天性心疾患

右→左シャントが優位な疾患チアノーゼ性心疾患

左→右シャントが優位な疾患非チアノーゼ性心疾患

疫学

心室中隔欠損症	30.5
心房中隔欠損症	9.8
動脈管開存症	9.7
肺動脈狭窄症	6.9
大動脈縮窄症	6.8
大動脈狭窄症	6.1
ファロー四徴症	5.8
完全大血管転位	4.2
その他	20

症状

参考文献(2)より

心疾患による症状
	肺血流増加	肺血流減少	低心拍出
1．新生児期・乳児早期	多呼吸，陥没呼吸，呼吸困難，喘鳴，多汗，哺乳障害	チアノーゼ	蒼白，末梢冷感，冷汗，網状チアノーゼ，体重増加不良，弱い泣き声
2．乳幼児期	多呼吸，易感染性，反復する肺炎	チアノーゼ，低酸素発作，蹲踞	体重増加不良，運動発達遅延，易疲労性，顔色不良，やせ
3．小児期	運動能低下，息切れ	ばち状指	運動能低下，動悸
4．思春期以後　合併症による症状	胸痛，失神発作，突然死，喀血，不整脈，出血傾向，痛風，けいれん　等

酸素投与の悪影響(1)

1)動脈管依存型　→　酸素投与により動脈管が閉鎖方向に向かう

①肺循環が動脈管依存する疾患

肺動脈閉鎖、右室低形成、重症肺動脈狭窄

②体循環が動脈管依存する疾患（禁忌）

大動脈縮窄・大動脈離断、大動脈閉鎖

2)肺循環負荷型　→　酸素投与により肺血管が拡張し、肺血管抵抗も減少して肺うっ血が増強

①大きな心室・大血管の転位・欠損

心室中隔欠損、大動脈縮窄複合、完全心内膜床欠損、総動脈幹遺残、

完全大血管転位（II型）、三尖弁閉鎖（C型）、大動脈肺動脈窓など

②肺静脈閉塞性疾患（蘇生術も含め絶対禁忌）

総肺静脈環流異常、三心房心、僧帽弁狭窄、肺静脈狭窄など

合併症

QB.C-478

ファロー四徴症：脳血栓、脳膿瘍、感染性心内膜炎
大血管転位症：脳血栓　←　右左シャント
エブスタイン奇形：不整脈
動脈管開存症、心室中隔欠損症：感染性心内膜炎
肺動脈弁狭窄症：脳膿瘍

先天性疾患と先天心疾患

疾患	先天性心奇形	その他
22q11.2欠失症候群	ファロー四徴症、総動脈幹症(truncus arteriosus)、心室中隔欠損を伴う肺動脈弁閉鎖症、大動脈弓離断、右側大動脈弓、右側肺動脈の大動脈起始	胸腺低形成、低カルシウム血症、特有の顔貌、口蓋裂
ダウン症候群	(50%の例に合併)。心房中隔欠損症(ASD), 心室中隔欠損症(VSD)
先天性風疹症候群	心房中隔欠損症、動脈管開存症、末梢肺動脈狭窄
ターナー症候群	大動脈縮窄症	低身長・翼状頚、外反肘
ヌーナン症候群	肺動脈弁狭窄、肥大型心筋症	ターナー症候群に似る
マルファン症候群	僧帽弁逸脱症、大動脈弁閉鎖不全症
糖代謝異常合併妊娠母体からの出生児	大血管転位症
ウイリアムズ症候群	大動脈弁上狭窄、末梢肺動脈狭窄
無脾症、多脾症	単心房、単心室、総肺静脈還流異常