WordNet

a relation between things or events (as in the case of one causing the other or sharing features with it); "there was a connection between eating that pickle and having that nightmare" (同)connexion, connectedness
a connecting shape (同)connexion, link
the state of being connected; "the connection between church and state is inescapable" (同)link, connectedness
shifting from one form of transportation to another; "the plane was late and he missed his connection in Atlanta" (同)connexion
an instrumentality that connects; "he soldered the connection"; "he didnt have the right connector between the amplifier and the speakers" (同)connexion, connector, connecter, connective
(usually plural) a person who is influential and to whom you are connected in some way (as by family or friendship); "he has powerful connections"
a supplier (especially of narcotics)
determine the sum of; "Add all the people in this town to those of the neighboring town" (同)tot, tot up, sum, sum_up, summate, tote up, add, add together, tally, add up
damage beyond the point of repair; "My son totaled our new car"; "the rock star totals his guitar at every concert"
add up in number or quantity; "The bills amounted to $2,000"; "The bill came to $2,000" (同)number, add up, come, amount
deviating from the general or common order or type; "advanced forms of life may be anomalous in the universe"
a small amount (especially of a drink); "a tot of rum"
of or contained in or performing the function of the veins; "venous inflammation"; "venous blood as contrasted with arterial blood"; "venous circulation"

PrepTutorEJDIC

『総計の』,全体の / 『全くの』,完全な(absolute) / 『合計』,総額,総数,総量(sum) / …‘を'合計する / 合計…‘に'なる,総額が…‘に'のぼる(amount to)
変則的な(irregular) / 異常な(abnormal)
幼児,小児 / (特に酒類の)少量,一杯,一口
…‘を'合計する《+up+名,+名+up》 / 合計(…に)なる《+up to+名》
静脈[血]の / 葉脈の多い

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/07/31 11:57:49」(JST)

wiki en

Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return^[1]) is a congenital defect of the pulmonary veins.

Total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection, also known as total anomalous pulmonary venous drainage and total anomalous pulmonary venous return, is a rare cyanotic congenital heart defect in which all four pulmonary veins are malpositioned and make anomalous connections to the systemic venous circulation. (Normally, pulmonary veins return oxygenated blood from the lungs to the left atrium where it can then be pumped to the rest of the body). A patent foramen ovale, patent ductui arteriosa or an atrial septal defect must be present, or else the condition is fatal due to a lack of systemic blood flow.

In some cases, it can be detected prenatally.^[2]

There are four variants: Supracardiac (50%): blood drains to one of the innominate veins (brachiocephalic veins) or the superior vena cava; Cardiac (20%), where blood drains into coronary sinus or directly into right atrium; Infradiaphragmatic (20%), where blood drains into portal or hepatic veins; and a mixed (10%) variant.

TAPVC can occur with obstruction, which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot enter the new vein as easily.

Signs and symptoms

right ventricular heave
Loud S1
fixed split S2
S3 gallop
systolic ejection murmur at left upper sternal border
cardiomegaly
right axis deviation on ECG
Snowman sign or `figure of 8 configuration` on chest radiograph
right ventricular hypertrophy
cyanosis, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema
Cottage-loaf sign, that is, chest X-ray appearance similar to a cottage loaf, also known as the ‘snow man’ sign or ‘figure of 8’ sign.^[3]

Treatment

In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. With obstruction, surgery should be undertaken emergently. PGE1 should be given because a patent ductus arteriosus allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.

Partial anomalous pulmonary venous connection

A Partial anomalous pulmonary venous connection (or Partial anomalous pulmonary venous drainage or Partial anomalous pulmonary venous return) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins.

It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. In less severe cases, with smaller amounts of blood flow, diagnosis may be delayed until adulthood, when it can be confused with other causes of pulmonary hypertension.^[4] There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally.^[5] It is associated with other vascular anomalies, and some genetic syndromes such as monosomy X.

Diagnosis

It can be diagnosed with CT scan, angiography, transesophageal echocardiography, or cardiac MRI.^[6]^[7] Unfortunately, less invasive and expensive testing, such as transthoracic echocardiography and CT scanning are generally less sensitive.

Treatment

It is sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure.^[8]^[9] However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases.

References

^ "Facts about Total Anomalous Pulmonary Venous Return or TAPVR". Retrieved November 10, 2013.
^ Allan LD, Sharland GK (April 2001). "The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus". Heart. 85 (4): 433–7. PMC 1729686 . PMID 11250972. doi:10.1136/heart.85.4.433.
^ Somerville J, Grech V (2009). "The chest x-ray in congenital heart disease 1. Total anomalous pulmonary venous drainage and coarctation of the aorta". Images Paediatr Cardiol. 11 (1): 7–9. ISSN 1729-441X. PMC 3232600 . PMID 22368552.
^ Sears EH, Aliotta JM, Klinger JR. Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension. Pulm Circ 2012;2:250-5.
^ Ho ML, Bhalla S, Bierhals A, Gutierrez F. MDCT of partial anomalous pulmonary venous return (PAPVR) in adults. J Thorac Imaging 2009;24:89-95.
^ Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, et al. (2004). "Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage". Circulation. 109: 207–14. PMID 14718402. doi:10.1161/01.cir.0000107842.29467.c5.
^ Ammash NM, Seward JB, Warnes CA, Connolly HM, O'Leary PW, Danielson GK (May 1997). "Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography". J. Am. Coll. Cardiol. 29 (6): 1351–8. PMID 9137235. doi:10.1016/S0735-1097(97)82758-1.
^ ElBardissi AW, Dearani JA, Suri RM, Danielson GK (March 2008). "Left-sided partial anomalous pulmonary venous connections". Ann. Thorac. Surg. 85 (3): 1007–14. PMID 18291189. doi:10.1016/j.athoracsur.2007.11.038.
^ Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG (December 2007). "Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection". Ann. Thorac. Surg. 84 (6): 2020–6; discussion 2020–6. PMID 18036929. doi:10.1016/j.athoracsur.2007.05.046.

External links

Total Anomalous Pulmonary Venous Return (TAPVR) - Stanford Children's Health

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. 総肺静脈還流異常症 total anomalous pulmonary venous connection
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. 重症先天性心疾患を有する新生児の識別 identifying newborns with critical congenital heart disease
4. 新生児におけるチアノーゼ性心疾患の診断および初期マネージメント diagnosis and initial management of cyanotic heart disease in the newborn
5. 部分肺静脈還流異常 partial anomalous pulmonary venous connection

English Journal

Postoperative Obstruction of the Pulmonary Veins in Mixed Total Anomalous Pulmonary Venous Connection.

Ho DY1, White BR2, Glatz AC2, Mascio CE3, Stephens P Jr2, Cohen MS2.
Pediatric cardiology.Pediatr Cardiol.2018 Jun 5. doi: 10.1007/s00246-018-1921-9. [Epub ahead of print]
PMID 29872881

Combined minimally invasive repair of the adult form of the scimitar syndrome and organic mitral regurgitation.

Inoue K1, Ueno T2, Hiraoka A1, Sakaguchi T1.
Interactive cardiovascular and thoracic surgery.Interact Cardiovasc Thorac Surg.2018 Jun 1;26(6):1037-1038. doi: 10.1093/icvts/ivy015.
PMID 29415266

The natural history and surgical outcome of patients with scimitar syndrome.

[No authors listed]
European heart journal.Eur Heart J.2018 May 23. doi: 10.1093/eurheartj/ehy298. [Epub ahead of print]
PMID 29800132

Japanese Journal

総肺静脈還流異常症，大血管転位症の麻酔管理

日臨麻会誌 37(4), 491-497, 2017
NAID 130006855489

本邦における2013，2014年の心臓血管外科手術の現状：日本心臓血管外科手術データベース（JCVSD）からの報告:2.　先天性心疾患手術

日心外会誌 46(5), 191-194, 2017
NAID 130006105334

総肺静脈還流異常症を合併した臓器錯位症候群

日小児循環器会誌 32(3), 221-222, 2016
NAID 130005157524

Related Pictures

★リンクテーブル★

リンク元	「総肺静脈還流異常症」「TAPVC」
関連記事	「connection」「anomalous」「total」「venous」「pulmonary」

「総肺静脈還流異常症」

Anomalous pulmonary venous connection
	Illustration of total anomalous pulmonary venous connection
Classification and external resources
Specialty	medical genetics
ICD-10	Q26.2-Q26.4
ICD-9-CM	747.41-747.42
eMedicine	ped/2816
	[edit on Wikidata]

　　[★]

英: total anomalous pulmonary venous connection TAPVC、total anomalous pulmonary venous return TAPVR
同: 総肺静脈還流異常
関: 肺静脈還流異常、部分的肺静脈還流異常、先天性肺静脈還流異常、三日月刀症候群; チアノーゼ性心疾患

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総肺静脈還流異常症 : 約 21,600 件
全肺静脈還流異常症 : 36 件

NSU.387

まとめ

先天的な心奇形であって、左心房に還流すべき肺静脈血が右心系に流入する病態である。生存に心房中隔欠損か卵円孔開存が必要であり、病態によっては肺静脈閉塞による肺うっ血を来す。右心系の容量負荷により右室肥大、不完全右室ブロックをきたす。チアノーゼ性心疾患であり、多くの場合、生後一ヶ月以内にチアノーゼ、右心不全、肺うっ血による呼吸困難を来す。治療は緊急的に、バルーン心房中隔裂開術(BAS)やBlalock-Hanlon手術を行う。(SSUR.370 YN.C-115)