Ebstein's anomaly of the tricuspid valve: Difference between revisions

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__NOTOC__
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = {{PAGENAME}} |
   Name          = |
   Image          = Ebstein's anomaly 001.jpg|
   Image          = Ebstein_diagram.jpg‎|
  Caption        = Chest X-ray of a patient with Ebstein's anomaly <br> [http://www.radswiki.net Image courtesy of RadsWiki]|
  DiseasesDB    = 4039 |
  ICD10          = {{ICD10|Q|22|5|q|20}} |
  ICD9          = {{ICD9|746.2}} |
  ICDO          = |
  OMIM          = 224700 |
  MedlinePlus    = |
  eMedicineSubj  = med |
  eMedicineTopic = 627 |
  MeshID        = D004437 |
}}
}}
{{SI}}
{{CMG}} and Claudia P. Hochberg, M.D. [mailto:chochber@bidmc.harvard.edu]
__NOTOC__
'''Associate Editor-In-Chief:''' {{CZ}}
{{Editor Join}}
==Overview==
'''Ebstein's anomaly''' is a congenital heart defect in which the opening of the [[tricuspid valve]] is displaced towards the apex of the [[right ventricle]] of the heart (congenital apical displacement of the [[tricuspid valve]] that typically causes significant [[tricuspid regurgitation]]).
The annulus of the valve is in 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. 50% of cases involve an atrial shunt (either a [[PFO]] or an [[ASD]]).
==Etymology==
Ebstein's anomaly was named after Wilhelm Ebstein.<ref>{{WhoNamedIt|synd|435}}</ref><ref>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.</ref>
==Diagnoses Ebstein's Anomaly of the Tricuspid Valve should be Distinguished from==
* Accessory pathway-mediated [[WPW syndrome]] and [[SVT]]
* [[Atrial septal defect]] ([[ASD]])
* [[Cyanotic congenital heart diseases]]
* [[Isolated, severe tricuspid regurgitation]]
* [[L-transposition of the great vessels]]
* [[Severe right heart failure]]
==Associated 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.
=== Anatomy ===
Typically, there are anatomic abnormalities of the [[tricuspid valve]], with enlargement of the anterosuperior leaflet of the valve which is often adherent to the right ventricular free wall.


About 50% of individuals with Ebstein's anomaly have an associated shunt between the right and [[left atrium]]s, either an [[atrial septal defect]] [[ASD]]  or a [[patent foramen ovale]] [[PFO]].
'''For patient information, click [[{{PAGENAME}} (patient information)|here]]'''
{{Ebstein's anomaly of the tricuspid valve}}


- The [[tricuspid valve]] leaflets are displaced toward the RV apex.  
{{CMG}}; '''Associate Editor-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.B.B.S.]] [mailto:psingh13579@gmail.com]; {{CZ}}}; '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


- The RV cavity is dilated and the free wall is thin.
==[[Ebstein's anomaly of the tricuspid valve overview|Overview]]==


- May be associated with LV dysfunction and [[mitral valve prolapse]] ([[MVP]]).
==[[Ebstein's anomaly of the tricuspid valve anatomy|Anatomy]]==


- It has also been associated with [[right ventricular outflow tract obstruction|pulmonic stenosis]] [[right ventricular outflow tract obstruction| (PS)]], [[VSD]], [[pulmonary hypertension]], [[aortic stenosis|bicuspid aortic valve]] and right sided aortic arch.
==[[Ebstein's anomaly of the tricuspid valve historical perspective|Historical Perspective]]==


-It is commonly associated with congenitally corrected [[transposition of the great vessels]] and occasionally with [[tetralogy of Fallot]].
==[[Ebstein's anomaly of the tricuspid valve pathophysiology|Pathophysiology]]==
- Is often associated with one or more accessory conduction pathways in 25%, and [[WPW]] in 13%. Usually this is a right sided accessory pathway.


- The presence of an [[ASD]] permits right-to-left shunting and [[cyanosis]]. The degree of [[cyanosis]] is related to the degree of [[tricuspid regurgitation]] ([[tricuspid regurgitation|TR]]), [[tricuspid stenosis]] ([[tricuspid stenosis|TS]]) and the compliance of the RV.
==[[Ebstein's anomaly of the tricuspid valve causes|Causes]]==


<div align="left">
==[[Ebstein's anomaly of the tricuspid valve differential diagnosis|Differentiating Ebstein’s anomaly of the tricuspid valve from other Disorders]]==
<gallery heights="175" widths="300">
Image:Ebstein diagram.jpg|Graphical represntation of Ebstein's Anomaly from the Mayo Clinic website (note there is also an ASD on this diagram)
Image:EbsteinAnomaly.png|Ebstein Anomaly
</gallery>
</div>


==[[Epidemiology and Demographics]]==
==[[Ebstein's anomaly of the tricuspid valve epidemiology and demographics|Epidemiology and demographics]]==


*Is a rare congenital heart disease.
==[[Ebstein's anomaly of the tricuspid valve risk factors|Risk Factors]]==
*About 1:1,000 patients with [[congenital heart disease]], or 1:210,000 live births.
*Males = females.
*Higher incidence in infants whose mothers are treated with [[Lithium]] Carbonate during pregnancy


== Genetics ==
==[[Ebstein's anomaly of the tricuspid valve natural history, complications & prognosis|Natural history, Complications, and Prognosis]]==
*Multifactorial inheritance, risk if a sibling has the disease is 1%.


==Diagnosis==
==Diagnosis==
[[Ebstein's anomaly of the tricuspid valve history and symptoms|History and Symptoms]] | [[Ebstein's anomaly of the tricuspid valve physical examination|Physical Examination]] | [[Ebstein's anomaly of the tricuspid valve electrocardiogram | Electrocardiogram]] | [[Ebstein's anomaly of the tricuspid valve chest x ray|Chest X Ray]] | [[Ebstein's anomaly of the tricuspid valve MRI|MRI]] | [[Ebstein's anomaly of the tricuspid valve CT|CT]] | [[Ebstein's anomaly of the tricuspid valve echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Ebstein's anomaly of the tricuspid valve other imaging findings|Cardiac Catheterization]] | [[Ebstein's anomaly of the tricuspid valve other diagnostic studies|Electrophysiological Testings]] | [[Ebstein's anomaly of the tricuspid valve ACC/AHA Guidelines for diagnostic tests |ACC/AHA Guidelines for Diagnostic Tests ]] | [[Ebstein's anomaly of the tricuspid valve ACC/AHA Guidelines for evaluation of Patients|ACC/AHA Guidelines for Evaluation of Patients]]


[[Ebstein's anomaly of the tricuspid valve history|History]] | [[Ebstein's anomaly of the tricuspid valve physical examination|Physical Examination]] | [[Ebstein's anomaly of the tricuspid valve laboratory findings|Laboratory Findings]] | [[Ebstein's anomaly of the tricuspid valve CT and MRI|CT and MRI]]
==Treatment==
 
[[Ebstein's anomaly of the tricuspid valve medical therapy|Medical Therapy]] | [[Ebstein's anomaly of the tricuspid valve surgery|Surgery]] | [[Ebstein's anomaly of the tricuspid valve primary prevention|Prevention]] | [[Ebstein's anomaly of the tricuspid valve ACC/AHA Guidelines for reproduction|ACC/AHA Guidelines for Reproduction]]
==[[Ebstein's anomaly of the tricuspid valve treatment|Treatment]]==
 
 
==[[Ebstein's anomaly of the tricuspid valve prognosis|Prognosis]]==
 
 
==References==
{{Reflist}}
 
==Additional Resources==
 
* Emma C. Ferguson, Rajesh Krishnamurthy, and Sandra A. A. Oldham. [http://radiographics.rsnajnls.org/cgi/content/abstract/27/5/1323 Classic Imaging Signs of Congenital Cardiovascular Abnormalities.] RadioGraphics 2007 27: 1323-1334.
* Joris P. A. Beerepoot, and Pamela K. Woodard. [http://radiology.rsnajnls.org/cgi/content/full/231/3/747 Case 71: Ebstein Anomaly]. Radiology 2004 231: 747-751.
 
==External Links==
* [http://goldminer.arrs.org/search.php?query=Ebstein's%20anomaly Goldminer: Ebstein's anomaly]
* [http://heartcenter.seattlechildrens.org/conditions_treated/ebsteins_malformation.asp Ebstein's Malformation information from Seattle Children's Hospital Heart Center]
* [http://www.mayoclinic.org/ebsteins-anomaly/abnormalities.html Overview and diagram at Mayo Clinic]
* [http://www.ebsteins.org Information and support for those affected by Ebstein's anomaly]


{{Congenital malformations and deformations of circulatory system}}
[[Category:Cardiovascular system]]
{{SIB}}
[[Category:DiseaseState]]
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]
[[Category:Disease]]


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Latest revision as of 15:20, 6 March 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Priyamvada Singh, M.B.B.S. [2]; Cafer Zorkun, M.D., Ph.D. [3]}; Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]

Overview

Anatomy

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Pathophysiology

Causes

Differentiating Ebstein’s anomaly of the tricuspid valve from other Disorders

Epidemiology and demographics

Risk Factors

Natural history, Complications, and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography or Ultrasound | Cardiac Catheterization | Electrophysiological Testings | ACC/AHA Guidelines for Diagnostic Tests | ACC/AHA Guidelines for Evaluation of Patients

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