Ebstein's anomaly of the tricuspid valve
| 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 |
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]
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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
- 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
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
- ↑ Template:WhoNamedIt
- ↑ 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.
Additional Resources
- Emma C. Ferguson, Rajesh Krishnamurthy, and Sandra A. A. Oldham. Classic Imaging Signs of Congenital Cardiovascular Abnormalities. RadioGraphics 2007 27: 1323-1334.
- Joris P. A. Beerepoot, and Pamela K. Woodard. Case 71: Ebstein Anomaly. Radiology 2004 231: 747-751.