Ebstein's anomaly

Ebstein's anomaly
Classification & external resources

ICD-10	Q22.5
ICD-9	746.2
OMIM	224700
DiseasesDB	4039
eMedicine	med/627
MeSH	D004437

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.

Contents 1 Presentation 2 Risk factors 3 History 4 Related abnormalities 4.1 Anatomic abnormalities 4.2 Electrophysiologic abnormalities 5 References 6 External links

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.

Risk factors

There appears to be an increased risk of this abnormality in infants of women taking lithium during the first trimester of pregnancy, and in those with Wolff-Parkinson-White syndrome.

History

Ebstein's anomaly was named after Wilhelm Ebstein.^[1][2]

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

Electrophysiologic abnormalities

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

References

1. ^ synd/435 at Who Named It
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.

External links

• Ebstein's Malformation information from Seattle Children's Hospital Heart Center
• Diagram at kumc.edu
• Overview and diagram at Mayo Clinic

•  • e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747)
Cardiac chambers and connections	Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo)
Cardiac septa	Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome
Right: pulmonary and tricuspid valves	pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly
Left: aortic and mitral valves	aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome
Other congenital malformations of heart	Dextrocardia - Levocardia - Cor triatriatum
Great arteries	aorta (Patent ductus arteriosus, Aortic coarctation, Interrupted aortic arch, Overriding aorta, Aneurysm of sinus of Valsalva, Vascular ring) - Pulmonary atresia
Great veins	Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome
Other	Arteriovenous malformation (Cerebral arteriovenous malformation)
See also non-congenital conditions (I, 390-459)