WordNet

a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
a curved oriental saber; the edge is on the convex side of the blade

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(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/10/25 10:15:49」(JST)

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Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung.^[1] This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava.^[2] This radiographic density often has the shape of a scimitar, a type of curved sword.^[2]

Presentation

Chest x-ray of a five-year-old girl with Scimitar syndrome. The heart (blue outline) is shifted into the right half of the chest, and the anomalous pulmonary venous return (red) has a shape reminiscint of a Scimitar.

The anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.

Diagnosis

The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, and more recently by CT angiography or MR Angiography.

Treatment

Surgical correction should be considered in the presence of significant left to right shunting (Qp:Qs ≥ 2:1) and pulmonary hypertension. This involves creation of an inter-atrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.

References

^ Sehgal, Arvind; Loughran-Fowlds, Allison (PDF), Clinical Brief: Scimitar Syndrome, medIND — Biomedical journals from India, http://medind.nic.in/icb/t05/i3/icbt05i3p249.pdf, retrieved 2008-02-28
^ ^a ^b Scimitar Syndrome, Children's Hospital Boston, http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html, retrieved 2008-02-28

External links

Holt, PD; Berdon, WE; Marans, Z; Griffiths, S; Hsu, D (February 2004). "Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome". Pediatric Radiology 34 (5): 409–13. doi:10.1007/s00247-004-1149-0. PMID 14872299.

UpToDate Contents

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1. 部分肺静脈還流異常 partial anomalous pulmonary venous connection
2. 肺発達異常の放射線学的所見 radiographic appearance of developmental anomalies of the lung
3. 成人における心房中隔欠損症の病態生理および臨床的特徴 pathophysiology and clinical features of atrial septal defects in adults
4. 小児における孤立性心房中隔欠損の臨床的特徴および分類 classification and clinical features of isolated atrial septal defects in children
5. 心肺移植 heart lung transplantation

English Journal

Scimitar Syndrome and Azygos Continuation of the Inferior Vena Cava Diagnosed in an Adult-An Unusual Association.

Celik M, Celik T, Iyisoy A, Ayten O.SourceDepartment of Cardiology, Van Army District Hospital, Van, Turkey Department of Pulmonary Diseases and Tuberculosis, Van Army District Hospital, Van, Turkey Department of Cardiology, School of Medicine, Gulhane Military Medical Academy, Etlik-Ankara, Turkey.
Congenital heart disease.Congenit Heart Dis.2012 Apr 2. doi: 10.1111/j.1747-0803.2012.00650.x. [Epub ahead of print]
Anormal drainage of the pulmonary veins into the inferior vena cava is known as scimitar syndrome. Scimitar syndrome often presents during infancy and rarely during adulthood, and the adult patients are mostly asymptomatic. It is usually in association with dextrocardia, hypoplasia of right lung, an
PMID 22469461

[Scimitar syndrome in adult life: a rare anomaly completely characterized by cardiovascular magnetic resonance].

Casolo G, Del Meglio J, Tessa C, Comella A, Vignali C.
Giornale italiano di cardiologia (2006).G Ital Cardiol (Rome).2012 Apr;13(4):304-5. doi: 10.1714/1056.11563.
PMID 22495648

Japanese Journal

肺分画症を合併した Scimitar 症候群の一手術例

橋本昌樹,多久和輝尚,奥村好邦,近藤展行,田中文啓,長谷川誠紀
日本呼吸器外科学会雑誌 = The journal of the Japanese Association for Chest Surgery 24(5), 30-34, 2010-07-15
… Scimitar症候群は部分肺静脈還流異常症の一亜型であり肺分画症をはじめ種々の合併奇形を併存することが知られている．症例は30歳代女性．背部痛を主訴に近医で胸部CTを行い，右肺から下大静脈へ流入する異常血管と右肺分画症を認め，Scimitar症候群を疑い当科紹介となった．肺動脈造影，大動脈造影を行い右肺から下大静脈へ流入するScimitar veinと下行大動脈から右肺S7に流入す …
NAID 10026485191

右肺静脈移植と心房内血流転換を行ったscimitar症候群の1例

宇野吉雅,鈴木孝明,保土田健太郎,石田治,福田豊紀
日本心臓血管外科学会雑誌 36(5), 305-308, 2007-09-15
… 症例は16歳,男性.生来自覚症状,異常所見を認めなかったが,7歳肺炎罹患時に胸部単純写真で右肺の異常陰影を指摘された.その後の精査でscimitar症候群と診断されたが,経過観察となっていた.今回15歳時の心臓カテーテル検査で,肺体血流比の上昇を認めたため手術施行となった.手術は,上行大動脈送血,上大静脈+大腿静脈2本脱血の体外循環,心停止下に右肺静脈移植と右房内血流転換を行った.本症例では心房中隔欠損がなく, …
NAID 110006419176

Related Pictures

★リンクテーブル★

リンク元	「シミター症候群」
関連記事	「syndrome」

「シミター症候群」

Scimitar syndrome
	Classification and external resources
	; Chest MR angiography in a patient with scimitar syndrome
ICD-10	Q26.8
ICD-9	747.49
MeSH	D012587

　　[★]

英: scimitar syndrome
同: Scimitar症候群、三日月刀症候群、半月刀症候群
関: 部分肺静脈還流異常症

[show details]

シミター症候群 : 約 792 件
三日月刀症候群 : 14 件
半月刀症候群 : 7 件
Scimitar症候群 : 約 3,080 件

部分肺静脈還流異常症のに含まれる疾患で、右肺静脈が垂直に下降し、横隔膜を越えてから下大静脈に還流するもの(SSUR.357)。

参考

英文。写真と解説。移動します。

http://www.ajronline.org/cgi/content/full/183/5/1497/FIG19

写真がでかすぎ。↑と同じです。

http://radiographics.rsna.org/content/24/1/e17/F32.large.jpg

http://radiographics.rsna.org/cgi/content-nw/full/e17v1/F11A

「syndrome」

　　[★]

n.

症候群

[0] Sehgal, Arvind; Loughran-Fowlds, Allison (PDF), Clinical Brief: Scimitar Syndrome, medIND — Biomedical journals from India, http://medind.nic.in/icb/t05/i3/icbt05i3p249.pdf, retrieved 2008-02-28

[chb2165po-1] Scimitar Syndrome, Children's Hospital Boston, http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html, retrieved 2008-02-28

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案内

scimitar syndrome