部分肺静脈還流異常症 partial anomalous pulmonary venous connection

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/02/13 22:31:45」(JST)

wiki en

Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium).^[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 reminiscent 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, Clinical Brief: Scimitar Syndrome (PDF), medIND — Biomedical journals from India, retrieved 2008-02-28
^ ^a ^b Scimitar Syndrome, Children's Hospital Boston, 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

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 部分肺静脈還流異常 partial anomalous pulmonary venous connection
2. 総肺静脈還流異常症 total anomalous pulmonary venous connection
3. 成人における心房中隔欠損症の外科的閉鎖および経皮閉鎖 surgical and percutaneous closure of atrial septal defects in adults
4. 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
5. 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

English Journal

[Life threatening haemoptysis associated with partial abnormal pulmonary venous return and pulmonary arterial hypertension.]

Pricopi C1, Debagh H2, Hernigou A3, Arame A2, Riquet M2.
Revue de pneumologie clinique.Rev Pneumol Clin.2015 Feb 26. pii: S0761-8417(15)00008-5. doi: 10.1016/j.pneumo.2014.12.005. [Epub ahead of print]
A 73-year-old man with chronic respiratory insufficiency was referred to the intensive care unit because of life threatening haemoptysis and hypoxemia. The patient was in respiratory failure with pulmonary arterial hypertension. The bleeding was issuing from the left inferior lobe. A small arterio-v
PMID 25727656

Sinus venosus atrial septal defect as a cause of palpitations and dyspnea in an adult: a diagnostic imaging challenge.

Donovan MS1, Kassop D1, Liotta RA2, Hulten EA1.
Case reports in medicine.Case Rep Med.2015;2015:128462. doi: 10.1155/2015/128462. Epub 2015 Jan 29.
Sinus venosus atrial septal defects (SV-ASD) have nonspecific clinical presentations and represent a diagnostic imaging challenge. Transthoracic echocardiography (TTE) remains the initial diagnostic imaging modality. However, detection rates have been as low as 12%. Transesophageal echocardiography
PMID 25705227

REPORT OF A RARE ANATOMIC VARIANT: LEFT UPPER LOBE PARTIAL ANOMALOUS PULMONARY VENOUS RETURN.

De Brucker Y, Ilsen B, Muylaert C, Goethals L, Nieboer K, Fares A, Jager T, de Mey J.
JBR-BTR : organe de la Société royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR).JBR-BTR.2015 Jan-Feb;98(1):37-8.
We report the CT findings in a case of partial anomalous pulmonary venous return (PAPVR) from the left upper lobe in an adult. PAPVR is an anatomic variant in which one to three pulmonary veins drain into the right atrium or its tributaries, rather than into the left atrium. This results in a left-t
PMID 26223064

Japanese Journal

肺動脈絞扼術後に感染性肺動脈瘤から主肺動脈右肺動脈瘻を呈した1例

黄義浩,森田紀代造,松村洋高,木ノ内勝史,中村賢,橋本和弘
日本心臓血管外科学会雑誌 35(5), 292-294, 2006-09-15
… 症例は9カ月の男児で,DORV, PAPVR, VSD, PH, polysplenia, azygos connectionに対し,生後6カ月時に当院で肺動脈絞扼術を施行した.術後の肺血流のコントロールは良好であったが,術後10日目に多剤耐性表皮ブドウ球菌による敗血症を呈し,2カ月後の胸部CT検査で細菌性心内膜炎による感染性肺動脈瘤および主肺動脈右肺動脈〓を認めた.内科治療中に心不全の急性増悪を呈したため準緊急的に動脈瘤切除,肺動脈再建術および再肺動脈絞扼術 …
NAID 110004788555

A-10 PAPVRを合併したと思われる三心房心の一治験例(一般口演心・大血管)

岡隆紀,石原和明,泉本浩史,川副浩平
日本小児外科学会雑誌 40(3), 399, 2004-05-20
NAID 110002114952

Related Pictures

17 Best images about RCIS Cardiac Congenital on Pinterest | Portal, Training and Children RiT radiology: ASD with PAPVR Ramblings and Rumblings: May 2011 Anomalous pulmonary venous connection - Wikipedia Radiology Rounds 71 Year Old Female, papvr | HeartBabyHome 67 - DORV, PAPVR, Azygos, HLV | heart defect: heterotaxy wit… | Flickr - Photo Sharing!

★リンクテーブル★

「PAP」

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

　　[★]

[1] Sehgal, Arvind; Loughran-Fowlds, Allison, Clinical Brief: Scimitar Syndrome (PDF), medIND — Biomedical journals from India, retrieved 2008-02-28

[chb2165po-2] Scimitar Syndrome, Children's Hospital Boston, retrieved 2008-02-28

匿名

検索

案内

案内

PAPVR