総肺静脈還流異常症 total anomalous pulmonary venous return

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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 venous return carries 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 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

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 right 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.^[3] There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally.^[4] It is associated with other vascular anomalies, and some genetic syndromes such as monosomy X.

It can be diagnosed with CT scan, angiography, transesophageal echocardiography, or cardiac MRI.^[5]^[6] Unfortunately, less invasive and expensive testing, such as transthoracic echocardiography and CT scanning are generally less sensitive.

It is sometimes treated with surgery.^[7]^[8] 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. doi:10.1136/heart.85.4.433. PMC 1729686. PMID 11250972.
^ 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. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage. Circulation 2004;109:207-14.
^ 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. doi:10.1016/S0735-1097(97)82758-1. PMID 9137235.
^ ElBardissi AW, Dearani JA, Suri RM, Danielson GK (March 2008). "Left-sided partial anomalous pulmonary venous connections". Ann. Thorac. Surg. 85 (3): 1007–14. doi:10.1016/j.athoracsur.2007.11.038. PMID 18291189.
^ 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. doi:10.1016/j.athoracsur.2007.05.046. PMID 18036929.

UpToDate Contents

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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. 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
4. 小児におけるチアノーゼの病因および評価 etiology and evaluation of cyanosis in children
5. 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

English Journal

Transcatheter embolization of anomalous systemic arterial supply with amplatzer vascular plugs II in scimitar syndrome.

Noble S, Miró J, Ibrahim R.SourceDepartment of Medicine, Montreal Heart Institute, Université de Montréal, Montreal, Quebec, Canada.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions.Catheter Cardiovasc Interv.2012 Mar 14. doi: 10.1002/ccd.23329. [Epub ahead of print]
A 57-year-old woman suffering from recurrent pneumonia and considered to be high risk for surgical correction of an atrial septal defect (ASD) associated with a right lung sequestration vascularized by arteries coming from the abdominal aorta and an abnormal venous drainage into the inferior vena ca
PMID 22419616

The scimitar syndrome confirmed by 320-slice computerized tomography.

Zhu J, Gao YH, Zhu SB, Liu Y.SourceDepartment of Thoracic Cardiovascular Surgery, Wuhan General Hospital of Guangzhou Command, Wuhan, People's Republic of China; Southern Medical University, Guangzhou, People's Republic of China.
The American journal of cardiology.Am J Cardiol.2012 Mar 1;109(5):773. Epub 2011 Dec 10.
Scimitar syndrome is a relatively rare variety of congenital heart disease characterized by partial or complete anomalous pulmonary venous connection of the right lung into the inferior vena cava. There are virtually no reports of the use of 320-slice computed tomography in establishing the diagnosi
PMID 22154321

Japanese Journal

総肺静脈還流異常症(TAPVR) (特集これが大切! 1カ月以内の新生児疾患) -- (見落としてはならない重症疾患・対応に迷う疾患)

中西敏雄
小児科診療 74(4), 593-597, 2011-04-00
NAID 40018758348

チアノーゼ性先天性心疾患(肺血流増加群) (ここまで分かる小児の単純X線写真とエコー画像) -- (心臓・循環器)

渋谷和彦
小児科臨床 61(-), 543-550, 2008-00-00
NAID 40015910997

Related Pictures

Supracardiac-Type I-TAPVR. The heart has a TAPVR - Całkowite nieprawidłowe Pulmonary Venous Drainage (TAPVR TAPVR TAPVR), as well as the more well-known TAPVR TAPVR TAPVR Total Anomalous Pulmonary Venous

★リンクテーブル★

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

「総肺静脈還流異常症」

Anomalous pulmonary venous connection
	Classification and external resources
	; Illustration of total anomalous pulmonary venous connection
ICD-10	Q26.2-Q26.4
ICD-9	747.41-747.42
eMedicine	ped/2816

　　[★]

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

[show details]

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)

疫学

先天性心疾患の約1%
全体の80%以上は新生児期から肺うっ血、肺高血圧、心不全となり早急な手術が必要(SSUR.370)

病型

I型：上心臓型：垂直動脈から無名静脈、あるいは下大静脈に灌流還流
II型：心臓型：右房、冠静脈洞
III型：下心臓型：横隔膜を貫いて下降し、下大静脈、門脈に灌流

病態

右心系への容量負荷、右室への容量負荷？(右室肥大)、肺うっ血、肺高血圧、
III型では肺静脈の還流路に狭窄(pulmonary venous obstruction, PVO)を来しやすく、肺うっ血を来しやすい(SSUR.370)
重症度はIII型>I型>II型
頻度はI型>II型、III型

検査

胸部単純X線写真

I型：上心臓型

心陰影：雪だるま陰影(snowman shadow)

次の構造によって８の字(雪だるま)が描かれる

上大静脈　腕頭静脈(無名静脈、垂直静脈)

右心室　　左心

肺野：すりガラス陰影：肺うっ血による

治療

心房中隔欠損、卵円孔開存が存在しなければ、緊急にバルーン心房中隔裂開術(BAS)、Blalock-Hanlon手術を施行
I型：共通肺静脈管を左心房に吻合。垂直動脈結紮切除。心房中隔欠損の閉鎖。
II型：右心房にパッチを当て、肺静脈血を左心房に流出させる。
III型：共通肺静脈管を左心房に吻合する。

禁忌

酸素投与　→　肺血管抵抗を下げて肺血流を増加させてしまう。

予後

手術死亡率：15% (SSUR.372)
手術生存者の5-15%に肺静脈の狭窄 (SSUR.372)
約60%が生後1ヶ月で心不全となり、約80%が1歳以内に脂肪。

参考

https://www.beaumonthospitals.com/health-library/P06611

「total anomalous pulmonary venous return」

　　[★] 総肺静脈還流異常症 TAPVR

「totally anomalous pulmonary venous return」