WordNet

deviation from the normal or common order or form or rule (同)anomalousness
a person who is unusual (同)unusual person
(astronomy) position of a planet as defined by its angular distance from its perihelion (as observed from the sun)

PrepTutorEJDIC

〈U〉〈C〉変則,例外;異常 / 〈C〉変則的な事態,異例な人(物)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/10/30 02:40:32」(JST)

wiki en

Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart.

Presentation

The annulus of the valve is still in the normal position. The valve leaflets, however, are to a varying degree, attached to the walls and septum of the right ventricle. There is subsequent 'atrialization' of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size.

S3 heart sound
S4 heart sound
Triple or quadruple gallop due to widely split S1 and S2 sounds plus a loud S3 and/or S4
Systolic murmur of tricuspid regurgitation = Holosystolic or early systolic murmur along the lower left sternal border depending on the severity of the regurgitation
Right atrial hypertrophy
Right ventricular conduction defects
Wolff-Parkinson-White syndrome often accompanies

Risk factors

There is an enlargement of the aorta; there may be an increased risk of abnormality in infants of women taking lithium during the first trimester of pregnancy^[1] (though some have questioned this)^[2] and in those with Wolff-Parkinson-White syndrome.

History

Ebstein's anomaly was named after Wilhelm Ebstein,^[3]^[4] who in 1866 described the heart of the 19-year-old Joseph Prescher.

Related abnormalities

While Ebstein's anomaly is defined as the congenital displacement of the tricuspid valve towards the apex of the right ventricle, it is often associated with other abnormalities.

Anatomic abnormalities

Typically, there are anatomic abnormalities of the tricuspid valve, with enlargement of the anterior leaflet of the valve. The other leaflets are described as being plastered to the endocardium.

About 50% of individuals with Ebstein's anomaly have an associated shunt between the right and left atria, either an atrial septal defect or a patent foramen ovale. ^[5]

Electrophysiologic abnormalities

About 50% of individuals with Ebstein's anomaly have evidence of Wolff-Parkinson-White syndrome, secondary to the atrialized right ventricular tissue.

10-lead ECG of a woman with Ebstein's anomaly. The ECG shows signs of right atrial enlargement, best seen in V1. Other P waves are broad and tall, these are termed "Himalayan" P waves. There is also a right bundle branch block pattern and a first degree atrioventricular block (prolonged PR-interval) due to intra-atrial conduction delay. There is no evidence of a Kent-bundle in this patient. There is T wave inversion in V1-4 and a marked Q wave in III; these changes are characteristic for Ebstein's anomaly and do not reflect ischemic ECG changes in this patient.

Other abnormalities that can be seen on the ECG include:

signs of right atrial enlargement or tall and broad 'Himalayan' P waves
first degree atrioventricular block manifesting as a prolonged PR-interval
low amplitude QRS complexes in the right precordial leads
atypical right bundle branch block
T wave inversion in V1-V4 and Q waves in V1-V4 and II, III and aVF.^[6]

Management

Pharmacological

Ebstein's cardiophysiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs like beta blockers, calcium channel blockers and digoxin are contraindicated.

If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response.

Surgical

The Canadian Cardiovascular Society (CCS) recommends surgical intervention for the following indications:^[7]

Limited exercise capacity (NYHA III-IV).
Increasing heart size (cardiothoracic ratio greater than 65%).
Important cyanosis (resting oxygen saturation of less than 90%). (Level B)
Severe tricuspid regurgitation with symptoms.
Transient ischemic attack or stroke

The CCS further recommends patients who require operation for Ebstein's anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.^[7]

References

^ Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK (2007). "Ebstein's anomaly". Circulation. 115 (2): 277–85. doi:10.1161/CIRCULATIONAHA.106.619338. PMID 17228014.
^ Yacobi S, Ornoy A (2008). "Is lithium a real teratogen? What can we conclude from the prospective versus retrospective studies? A review". Isr J Psychiatry Relat Sci. 45 (2): 95–106. PMID 18982835.
^ synd/435 at Who Named It?
^ W. Ebstein. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238-254.
^ كتاب A Practical Approach to Clinical Echocardiography, p.119 Jaypee Brothers, First Edition 2014, ISBN 978-93-5152-140-2.
^ Khairy P, Marelli AJ (December 2007). "Clinical use of electrocardiography in adults with congenital heart disease". Circulation. 116 (23): 2734–46. doi:10.1161/CIRCULATIONAHA.107.691568. PMID 18056539.
^ ^a ^b Silversides, C. K.; Salehian, O.; Oechslin, E.; Schwerzmann, M.; Vonder Muhll, I.; Khairy, P.; Horlick, E.; Landzberg, M.; Meijboom, F.; Warnes, C.; Therrien, J. (2010). "Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Complex congenital cardiac lesions". The Canadian journal of cardiology. 26 (3): e98–117. doi:10.1016/S0828-282X(10)70356-1. PMC 2851473. PMID 20352139.

External links

Ebstein's Malformation information from Seattle Children's Hospital Heart Center
Ebstein's Anomaly at Cardiopedia
Overview and diagram at Mayo Clinic
Information and support for those affected by Ebstein's anomaly
Ebstein's anomaly information for parents.

UpToDate Contents

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1. エブスタイン奇形の臨床症状と診断 clinical manifestations and diagnosis of ebstein anomaly
2. エブスタイン奇形のマネージメントと予後 management and prognosis of ebstein anomaly
3. パルスオキシメータを用いた、新生児における重篤な先天性心疾患のスクリーニング newborn screening for critical congenital heart disease using pulse oximetry
4. 三尖弁の心エコー評価 echocardiographic evaluation of the tricuspid valve
5. 先天性心疾患女性における妊娠：特定病変 pregnancy in women with congenital heart disease specific lesions

English Journal

Implantation of a Right Ventricular Implantable Cardioverter-Defibrillator Lead in the Right Ventricular Outflow Tract in a Patient with Ebstein Anomaly and Right Ventricular Lead Dislocation.

Pecha S, Yildirim Y, Hahnel F, Reichenspurner H, Aydin MA.SourceDepartment of Cardiovascular Surgery, University Heart Center Hamburg, Hamburg, Germany.
The Thoracic and cardiovascular surgeon.Thorac Cardiovasc Surg.2012 Dec 20. [Epub ahead of print]
An 80-year-old patient with Ebstein anomaly and prior implantable cardioverter-defibrillator (ICD) implantation was sent to our heart center because of right ventricular (RV)-ICD lead dislocation. Positioning of the new RV lead was difficult, no stable position could be found, and sensing and thresh
PMID 23258759

Case study and review: Treatment of tricuspid prosthetic valve thrombosis.

Zhang DY, Lozier J, Chang R, Sachdev V, Chen MY, Audibert JL, Horvath KA, Rosing DR.SourceDivision of Internal Medicine, University of Miami Miller School of Medicine, Miami, FL, United States.
International journal of cardiology.Int J Cardiol.2012 Dec 15;162(1):14-9. doi: 10.1016/j.ijcard.2011.09.081. Epub 2011 Oct 14.
Prosthetic valve thrombosis (PVT) is a severe and life-threatening complication of heart valve replacement. Conventional therapy is surgical thrombectomy or valve replacement. Medical thrombolysis is another emerging option. We report the case of a 57year old woman with a history of Ebstein anomaly
PMID 22000268

Japanese Journal

山形県立中央病院第26回CPC(2013年10月2日) 急性膵炎の治療中に突然死した50才代女性例

田村元 [司会],今孝志 [症例提示],柳川直樹 [病理他]
山形県立病院医学雑誌 48(1), 35-45, 2014-01
NAID 40020120151

5年間の胎児心エコー検査で検出された軽度異常所見の経過と予後について

佐藤工,佐藤啓,今野友貴
日本小児循環器学会雑誌 30(5), 563-568, 2014
… の肺動脈弁逆流4例，動脈管の蛇行3例，鏡像型右胸心2例を認めた．TR例の1例で，初回の検査では軽度であった逆流が軽度〜中等度に増加して，軽度の心拡大を伴うようになり，出生後に右室低形成を伴わないEbstein奇形と診断した．TR例の残り8例を含めて，その他の異常所見例は全て，一過性所見もしくは不変であり，治療介入を要するものはなかった．結語：胎児心エコー検査で遭遇する軽度の異常所見のうち，初回 …
NAID 130004700080

成人期に診断された Ebstein 病に対する Hetzer 法による1手術例

中尾充貴,森田紀代造,黄義浩,阿部貴行,橋本和弘
日本心臓血管外科学会雑誌 43(4), 195-199, 2014
… 症例は28歳女性．8歳のときにWPW症候群を指摘されたが，自覚症状なく経過していた．20歳ころより出現した上室性頻拍を契機に，26歳時にEbstein病と診断された．28歳時に当院にてWPW症候群に対するカテーテルアブレーションを施行し頻拍発作は消失したが，三尖弁閉鎖不全に伴う右心不全遷延にて三尖弁形成術の手術適応となった．心エコー上，三尖弁中隔尖・後尖の高度の落ち込みと右房化右室を認め，Carpentier type …
NAID 130004679597

Related Pictures

★リンクテーブル★

リンク元	「エプスタイン奇形」「三尖弁」
関連記事	「anomaly」

「エプスタイン奇形」

Ebstein's anomaly
	Pathological specimen and ultrasound image of a heart with Ebstein's anomaly. Abbreviations: RA: Right atrium; ARV: Atrialized right ventricle; FRV: Functional right ventricle; AL: Anterior leaflet; SL: Septal leaflet; LA: Left atrium; LV: Left ventricle; asterisk: grade II tethering of the tricuspid septal leaflet.
Classification and external resources
Specialty	medical genetics
ICD-10	Q22.5
ICD-9-CM	746.2
OMIM	224700
DiseasesDB	4039
MedlinePlus	007321
eMedicine	med/627
MeSH	D004437
	[edit on Wikidata]

　　[★]

英: Ebstein's anomaly Ebstein anomaly
同: エブシュタイン奇形, Ebstein奇形

[show details]

エブスタイン奇形 : 約 2,860 件
エプスタイン奇形 : 約 9,000 件

まとめ

三尖弁中隔尖・後尖の右室内への変異により、右室の狭小化、三尖弁閉鎖不全症(TR)を来し、さらに合併する心房中隔欠損・卵円孔開存により右左シャントをしめし、またWPW症候群合併による発作性頻拍を伴うこともある。乳児から心不全を呈する場合、予後は不良である。軽度の場合、手術は不要であるが、心不全を来した場合、三尖弁形成術・三尖弁置換術を行う。(SSUR.378 YM.C-127)

概念

三尖弁、右室心筋形成異常
三尖弁中隔尖と後尖が右室内で下方へ変位し、その部分の心筋形成異常、右室の狭小化、三尖弁閉鎖不全症、心房中隔欠損症を伴う先天性心奇形(PED.926)

検査

心電図

巨大P波、PR延長、WPW症候群の合併、完全右脚ブロック(YN.C-127)

病態

三尖弁閉鎖不全症、右室の狭小化による右室拍出力低下、心房中隔・卵円孔開存による右左シャント、WPW症候群

症状

胎児期新生児早期発症例：機能不十分な右室に生理的肺高血圧による後負荷　→　TRによる逆流増加　→　呼吸不全・心不全　→　予後不良
新生児期以降発症：軽度のチアノーゼ、運動能力低下を認めるのみで、無症状のことも　→　成人になるにつれ右室拡張期圧↑　→　右房圧↑　→　不整脈、動悸、運動時の息切れ、易疲労感、チアノーゼ(右→左シャントがあるとき)

YN.C-127

右心不全、チアノーゼ(40-70%)、(WPW症候群による発作性)上室性頻拍(25%)

治療

軽症例の場合は手術適応とならない。チアノーゼ、心拡大、心不全が強い場合に手術適応(YN.C-127)。

手術療法

新生児：
成人：三尖弁形成術(予後良い)、三尖弁置換術

YN.C-127

新生児：三尖弁入口を自己心膜で閉鎖、将来Fontan手術。
幼児以降：三尖弁形成術、心房中隔欠損閉鎖術

「三尖弁」

　　[★]

英: tricuspid valve (Z), TV
ラ: valva tricuspidalis
同: 右房室弁 right atrioventricular valve valva atrioventricularis dextra
関: 房室弁

右心房と右心室との間の右房室口の弁

臨床関連

Henry Gray (1825-1861). Anatomy of the Human Body. 1918.

「anomaly」

　　[★]

n.

異常、変則。異形。

関: aberrant、aberrantly、aberration、abnormal、abnormality、abnormally、anomalous、anomalously、defect、deviant、exceeding、exception、exceptional、extraordinarily、extraordinary、unusual、unusually

[pmid17228014-1] Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK (2007). "Ebstein's anomaly". Circulation. 115 (2): 277–85. doi:10.1161/CIRCULATIONAHA.106.619338. PMID 17228014.

[pmid18982835-2] Yacobi S, Ornoy A (2008). "Is lithium a real teratogen? What can we conclude from the prospective versus retrospective studies? A review". Isr J Psychiatry Relat Sci. 45 (2): 95–106. PMID 18982835.

[3] synd/435 at Who Named It?

[4] W. Ebstein. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin, Leipzig, 1866, 238-254.

[5] كتاب A Practical Approach to Clinical Echocardiography, p.119 Jaypee Brothers, First Edition 2014, ISBN 978-93-5152-140-2.

[pmid18056539-6] Khairy P, Marelli AJ (December 2007). "Clinical use of electrocardiography in adults with congenital heart disease". Circulation. 116 (23): 2734–46. doi:10.1161/CIRCULATIONAHA.107.691568. PMID 18056539.

[CCS2009-7] Silversides, C. K.; Salehian, O.; Oechslin, E.; Schwerzmann, M.; Vonder Muhll, I.; Khairy, P.; Horlick, E.; Landzberg, M.; Meijboom, F.; Warnes, C.; Therrien, J. (2010). "Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Complex congenital cardiac lesions". The Canadian journal of cardiology. 26 (3): e98–117. doi:10.1016/S0828-282X(10)70356-1. PMC 2851473. PMID 20352139.

匿名

検索

案内

Ebstein anomaly

WordNet

PrepTutorEJDIC

Wikipedia preview

wiki en

Contents

Presentation

Risk factors

History

Related abnormalities

Anatomic abnormalities

Electrophysiologic abnormalities

Management

Pharmacological

Surgical

References

External links

UpToDate Contents

English Journal

Japanese Journal

Related Links

Related Pictures

★リンクテーブル★

「エプスタイン奇形」

まとめ

概念

検査

心電図

病態

症状

YN.C-127

治療

手術療法

YN.C-127

「三尖弁」

臨床関連

「anomaly」