Ebstein's anomaly of the tricuspid valve

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Ebstein's anomaly of the tricuspid valve
Chest X-ray of a patient with Ebstein's anomaly
Image courtesy of RadsWiki
ICD-10 Q22.5
ICD-9 746.2
OMIM 224700
DiseasesDB 4039
eMedicine med/627 
MeSH D004437

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] and Claudia P. Hochberg, M.D. [2]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3]

Please Join in Editing This Page and Apply to be an Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [4] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.

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.[1][2]

Diagnoses Ebstein's Anomaly of the Tricuspid Valve should be Distinguished from

Associated Abnormalities

Epidemiology and demographics

Genetics

  • Multifactorial inheritance, risk if a sibling has the disease is 1%.

Diagnosis

History | Physical examination | Laboratory findings | CT and MRI

Treatment

Prognosis

References

  1. Template:WhoNamedIt
  2. 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.

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