WordNet

control consisting of a mechanical device for controlling the flow of a fluid
device in a brass wind instrument for varying the length of the air column to alter the pitch of a tone
one of the paired hinged shells of certain molluscs and of brachiopods
the entire one-piece shell of a snail and certain other molluscs
a structure in a hollow organ (like the heart) with a flap to insure one-way flow of fluid through it
having two cusps or points (especially a molar tooth); "bicuspid teeth"; "bicuspid leaves" (同)bicuspidate
(of brass instruments) having valves

PrepTutorEJDIC

(液体・ガスなどの流れを調節する)『バルブ』,弁 / (血液の流れを調節する)弁,弁膜 / (管楽器の)バルブ / (2枚貝の)から,貝がら / 《英》真空管
(歯などが)2つの尖(せん)部(突起)を持つ / 小臼歯(きゅうし)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/08/27 21:59:28」(JST)

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A bicuspid aortic valve (BAV) is most commonly a congenital condition of the aortic valve where two of the aortic valvular leaflets fuse during development resulting in a valve that is bicuspid instead of the normal tricuspid configuration. Normally the only cardiac valve that is bicuspid is the mitral valve (bicuspid valve) which is situated between the left atrium and left ventricle. Cardiac valves play a crucial role in ensuring the unidirectional flow of blood from the atrium to the ventricles, or the ventricle to the aorta or pulmonary trunk.

Incidence/prevalence

Heart bicuspid aortic valve anatomy.

Bicuspid aortic valves are the most common cardiac valvular anomaly, occurring in 1- 2% of the general population. It is twice as common in males as in females.^[1]

Bicuspid aortic valve is a heritable condition, with a demonstrated association with Notch 1.^[2] Both familial clustering and isolated valve defects have been documented. The incidence of bicuspid aortic valve can be as high as 10% in families affected with the valve problem. Recent studies suggest that BAV is an autosomal dominant condition with incomplete penetrance. Other congenital heart defects are associated with bicuspid aortic valve at various frequencies, including coarctation of the aorta.^[3]

Classification

Bicuspid aortic valves may assume three different types of configuration^[4]:

"Real" bicuspid valves with two symmetric leaflets
A tricuspid architecture with a fusion of two leaflets
A tricuspid architecture with a fusion of three leaflets

Presentation

In many cases, a bicuspid aortic valve will cause no problems.^[5] However BAV may become calcified later in life, which may lead to varying degrees of severity of aortic stenosis that will manifest as murmurs. If the leaflets do not close correctly, aortic regurgitation can occur. If these become severe enough, they may require heart surgery. People with BAV may become tired more easily than those with normal valvular function and have difficulty maintaining stamina for cardio-intensive activities-due to poor heart performance. The heart is put under more stress in order to either pump more blood through a stenotic valve or attempt to circulate regurgitation blood through a leaking valve.

Diagnosis, treatment, and prognosis

Heart bicuspid aortic valve diagram

The condition can be associated with a heart murmur located at the right second intercostal space. Often there will be differences in blood pressures between upper and lower extremities. The diagnosis can be assisted with echocardiography (EchoCG) or magnetic resonance imaging (MRI).

Most patients with bicuspid aortic valve whose valve becomes dysfunctional will need careful follow-up and potentially valve replacement at some point in life. Regular EchoCG and MRI may be performed. For diagnosed patients, genetic testing is done to allow for future offspring with the disease to be monitored and treated early in life.

Patients with bicuspid aortic valve should be followed by a cardiologist or cardiac surgeon with specific interest in this valve pathology.

If the valve is normally functioning or minimally dysfunctional, average lifespan is similar to that of those without the anomaly.^[6]

Aorta complications

One of the most notable associations with BAV is the tendency for these patients to present with ascending aortic aneurysmal lesions. The extracellular matrix of the aorta in patients with BAV shows marked deviations from that of the normal tricuspid aortic valve. It is currently believed that an increase in the ratio of MMP2 (Matrix Metalloproteinases 2) to TIMP1 (Tissue Inhibitor Metalloproteinases 1) may be responsible for the abnormal degradation of the valve matrix and therefore lead to aortic dissection and aneurysm. However, other studies have also shown MMP9 involvement with no differences in TIMP expression. The size of the proximal aorta should be evaluated carefully during the work-up. The initial diameter of the aorta should be noted and annual evaluation with CT scan, or MRI to avoid ionizing radiation, should be recommended to the patient; the examination should be conducted more frequently if a change in aortic diameter is seen. From this monitoring, the type of surgery that should be offered to the patient can be determined based on the change in size of the aorta.

Co-arctation of the aorta (a congenital narrowing in the region of the ductus arteriosus) has also been associated with BAV.

References

^ Tzemos N, Therrien J, Yip J et al. (September 2008). "Outcomes in adults with bicuspid aortic valves". JAMA 300 (11): 1317–1325. doi:10.1001/jama.300.11.1317. PMID 18799444. http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=18799444.
^ Garg V, Muth AN, Ransom JF et al. (2005). "Mutations in NOTCH1 cause aortic valve disease". Nature 437 (7056): 270–274. doi:10.1038/nature03940. PMID 16025100.
^ Chhabra, Anikar (2002). Pathology Recall. LWW.
^ Kumme, anja (2007). Klassifikation bikuspider Aortenklappen.
^ "Ask the Pediatric Cardiologist - Bicuspid Aortic Valve". http://www.americanheart.org/presenter.jhtml?identifier=991. Retrieved 2007-08-08.
^ Michelena HI, Desjardins VA, Avierinos JF et al. (May 2008). "Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community". Circulation 117 (21): 2776–2784. doi:10.1161/CIRCULATIONAHA.107.740878. PMC 2878133. PMID 18506017. http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=18506017.

External links

Bicuspid Aortic Foundation Homepage
Cedars Sinai Heart Center - Bicuspid Aortic Disease
Bicuspid Aortic Valve Discussion Board

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. 成人における大動脈二尖弁の臨床症状および診断 clinical manifestations and diagnosis of bicuspid aortic valve in adults
2. 成人における大動脈二尖弁疾患のマネージメント management of adults with bicuspid aortic valve disease
3. 大動脈二尖弁の女性における妊娠 pregnancy in women with a bicuspid aortic valve
4. 小児における大動脈弁狭窄 valvar aortic stenosis in children
5. 胸部大動脈瘤の疫学、危険因子、病因、および自然史 epidemiology risk factors pathogenesis and natural history of thoracic aortic aneurysm

English Journal

Frequency of congenitally bicuspid aortic valves in patients ≥80 years of age undergoing aortic valve replacement for aortic stenosis (with or without aortic regurgitation) and implications for transcatheter aortic valve implantation.

Roberts WC, Janning KG, Ko JM, Filardo G, Matter GJ.SourceDepartment of Internal Medicine (Division of Cardiology), Baylor University Medical Center, Dallas, Texas; Department of Pathology, Baylor University Medical Center, Dallas, Texas; Department of Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
The American journal of cardiology.Am J Cardiol.2012 Jun 1;109(11):1632-6. Epub 2012 Mar 27.
The purpose of the present report was to determine the frequency of a congenitally bicuspid aortic valve in patients ≥80 years of age old with aortic stenosis (AS) severe enough to warrant aortic valve replacement. Transcatheter aortic valve implantation (TAVI) has traditionally been reserved for
PMID 22459301

Genetics of thoracic aortic aneurysms.

Jondeau G, Boileau C.SourceAP-HP, Centre de Référence National pour le syndrome de Marfan et apparentés, Hôpital Bichat, 75018, Paris, France, guillaume.jondeau@bch.aphp.fr.
Current atherosclerosis reports.Curr Atheroscler Rep.2012 Jun;14(3):219-26.
Understanding of the genetics of thoracic aortic aneurysm is increasing rapidly, due to discovery of novel genes, better definition of the phenotypes associated with mutations in a given gene and better understanding of the pathophysiology. Beyond Marfan syndrome, usually related to mutation in FBN1
PMID 22415348

Japanese Journal

Reduction Aortoplasty for the Ascending Aortic Aneurysm With Aortic Valve Disease : Does Bicuspid Valve Matter?

HWANG Ho Young,SHIM Mi-Suk,PARK Eun-Ah,AHN Hyuk
Circulation journal : official journal of the Japanese Circulation Society 75(2), 322-328, 2011-01-25
… Background: The outcomes of reduction ascending aortoplasty (RAA) performed with aortic valve surgery were evaluated and the results of RAA in patients with bicuspid aortic valve (BAV) were compared with those in patients with tricuspid valve. …
NAID 10027426875

Adult Bicuspid Aortic Valve Endocarditis with Extensive Paravalvular Invasion Attributable to Disseminated Varicella Zoster Infection

Chen Shao-Wei,Tsai Feng-Chun,Chou An-Hsun
Annals of Thoracic and Cardiovascular Surgery advpub(0), 1112080123, 2011
… He later developed an extensively destructive endocarditis affecting a congenital bicuspid aortic valve, accompanied with leaflet perforation, complete atrio-ventricular (AV) block, and invasion of vegetation to both left and right atrium; …
NAID 130001257828

Related Pictures

★リンクテーブル★

リンク元	「大動脈二尖弁」
関連記事	「bicuspid」「aortic」「valve」

「大動脈二尖弁」

Bicuspid aortic valve
	Classification and external resources
ICD-9	746.4
OMIM	109730
DiseasesDB	1392
MedlinePlus	007325
eMedicine	ped/2486

　　[★]

英: bicuspid aortic valve
ラ: valvula bicuspidalis
同: 二尖大動脈弁

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二尖大動脈弁 : 約 9,170 件
大動脈二尖弁 : 約 85,100 件

「bicuspid」

　　[★] 小臼歯、二尖の、(心臓)二尖弁の

関: bicuspid valve、premolar

「aortic」

　　[★]

adj.

大動脈の

関: aorta、aortae、aortal

「valve」

　　[★]

n.

弁

[pmid18799444-0] Tzemos N, Therrien J, Yip J et al. (September 2008). "Outcomes in adults with bicuspid aortic valves". JAMA 300 (11): 1317–1325. doi:10.1001/jama.300.11.1317. PMID 18799444. http://jama.ama-assn.org/cgi/pmidlookup?view=long&pmid=18799444.

[pmid16025100-1] Garg V, Muth AN, Ransom JF et al. (2005). "Mutations in NOTCH1 cause aortic valve disease". Nature 437 (7056): 270–274. doi:10.1038/nature03940. PMID 16025100.

[2] Chhabra, Anikar (2002). Pathology Recall. LWW.

[3] Kumme, anja (2007). Klassifikation bikuspider Aortenklappen.

[4] "Ask the Pediatric Cardiologist - Bicuspid Aortic Valve". http://www.americanheart.org/presenter.jhtml?identifier=991. Retrieved 2007-08-08.

[pmid18506017-5] Michelena HI, Desjardins VA, Avierinos JF et al. (May 2008). "Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community". Circulation 117 (21): 2776–2784. doi:10.1161/CIRCULATIONAHA.107.740878. PMC 2878133. PMID 18506017. http://circ.ahajournals.org/cgi/pmidlookup?view=long&pmid=18506017.

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案内

bicuspid aortic valve