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Ebstein’S Anomaly

What is Ebstein’s anomaly?

Ebstein anomaly is a congenital heart defect. In this defect, the tricuspid valve is malformed in the sense that it is placed lower than normal in the right ventricle of the heart and one or two of its three leaflets are stuck and don’t move properly. As a result of the defect, the tricuspid valve does not perform well and blood leaks back through it, reducing the efficiency of the heart.  Often, the Ebstein anomaly is also showing a hole in the wall between the two upper chambers of the heart. Ebstein’s anomaly can also lead to magnification of the heart  or even to heart failure. Ebstein anomaly is more widespread in children of white females.

What are the symptoms of Ebstein’s anomaly?

Depending on the level of the anomaly, symptom can appear in infants or develop slowly over time. Amongst symptoms:

• Cyanosis – bluish discoloration of mainly lips and fingertips. The Cyanosis is caused from a mixture of oxygenated and un-oxygenated blood resulting for the malfunctioning valve. Cyanosis usually appears early with infants diagnosed with Ebstein’s anomaly, but can appear for the first time in adults.

• Swelling of the stomach, legs and sometimes even the lever.

• Edema and ascites in the ankles

• Abnormal heart rhythm such as  Palpitations, pounding or racing ,skipping beats

• Fatigue and shortness of breath during exercise

• In rare cases – brain abscess, endocarditis, brain stroke

Available treatments forEbstein’s anomaly?

Treatment of Ebstein anomaly depends in the level of severity, the symptoms that appear with the particular patient and his/her age.

When the level is mild and symptoms are mainly heart bit or hear failure related, treatment can include:

• Medications to control hear rate or hear failure. Commonly is use are calcium channel blockers, beta blockers and digitalis and anti-arrhythmic drugs, diuretics, digoxin

• Therapy that includes radiofrequency ablation (RFA)

In cases where the symptoms are severe and indicate significant leak of malfunctioning of the valve, surgical treatment can be considered. Surgical treatment may involve:

• Correction of the tricuspid valve to improve its functioning and reduce leaks

• Replacement of the tricuspid valve with an artificial one

• Correction of other intra-cardiac defects (such as ASD) that can be associated with Ebstein anomaly.

In infants, treatment can include palliative procedures as a temporary relied until a more complex surgical treatment is possible.

more information on Ebstein's anomaly and other diseases can be found at www.diseasebase.com/ebsteins-anomaly

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