Ebstein's anomaly

Common Name(s)

Ebstein's anomaly

Ebstein’s anomaly is a rare heart defect present at birth (congenital). In people with Ebstein’s anomaly, the tricuspid valve, which separates the two right chambers in the heart, is malformed. Normally, the tricuspid valve lets blood flow from the right atrium (upper chamber) to the right ventricle (lower chamber). In Ebstein’s anomaly, the valve is lower than it should be and allows blood to leak back into the atrium. This forces the heart to work harder, and can lead to heart enlargement or failure. Ebstein’s anomaly may be associated with atrial septal defects, heart arrhythmias and Wolff-Parkinson-White syndrome.

Symptoms may include shortness of breath, fatigue, heart palpitations (arrhythmias), or bluish skin or lips (cyanosis). Some people with Ebstein’s abnormality do not develop any severe symptoms, and many will not develop symptoms until later in adulthood. The causes of the anomaly are not yet known, but is believed to be caused by both genetic and environmental factors. People with a family history of heart defects or have heart defects themselves may be more likely to have a baby with Ebstein’s anomaly. A mother’s exposure to medications containing large amounts of lithium or benzodiazepines may also increase the risk for her baby.

Chest X-rays, ultrasounds, MRIs, or EKGs may be performed to diagnose Ebstein’s anomaly. All of these tests will allow a cardiologist to get a clearer picture of the heart. Treatments vary depending on the severity of the anomaly and the symptoms. Treatments can include regular monitoring, medications, surgery, or, in very severe cases, a heart transplant. If you or a loved one has been diagnosed with Ebstein’s anomaly, talk to a cardiologist (a heart doctor) about the most current treatment options. Support organizations and genetic counselors are also good sources of information and can help connect you with others affected by congenital heart conditions.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Ebstein's anomaly" for support, advocacy or research.

Saving Little Hearts

Saving Little Hearts is dedicated to helping children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages.

Last Updated: 15 Mar 2013

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General Support Organizations

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How do you compare to others with this condition?

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Ebstein's anomaly" for support, advocacy or research.

Saving Little Hearts

Saving Little Hearts is dedicated to helping children with congenital heart defects and their families by providing emotional assistance and educational information primarily through the distribution of Care Packages.

http://www.savinglittlehearts.com

Last Updated: 15 Mar 2013

View Details

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Ebstein's anomaly" returned 121 free, full-text research articles on human participants. First 3 results:

BNP and haematological parameters are markers of severity of Ebstein's anomaly: correlation with CMR and cardiopulmonary exercise testing.
 

Author(s): Olga Hösch, Thuy-Trang Ngyuen, Peter Lauerer, Andreas Schuster, Shelby Kutty, Wieland Staab, Christina Unterberg-Buchwald, Jan M Sohns, Thomas Paul, Joachim Lotz, Michael Steinmetz

Journal: Eur Heart J Cardiovasc Imaging. 2015 Jun;16(6):670-5.

 

Ebstein's anomaly (EA) involves a displaced and dysplastic tricuspid valve resulting in an atrialized portion of the right ventricle and an enlargement of the functional right ventricle and right atrium. Biomarkers targeting heart failure such as brain natriuretic peptide (BNP) or ...

Last Updated: 21 May 2015

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Paradoxical embolism in Ebstein's anomaly.
 

Author(s): Vibhav Rangarajan, Joan Briller, Afshin Farzaneh-Far

Journal: Eur. Heart J.. 2015 Feb;36(5):315.

 

Last Updated: 3 Feb 2015

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Electrophysiological study in Ebstein's anomaly with no evidence of accessory pathway.
 

Author(s): Larissa de Oliveira, Ana Karyn E de Freitas, Niraj Mehta, Marcio Rogério Ortiz, Leonardo A Mulinari, Cláudio L Pereira da Cunha

Journal: Arq. Bras. Cardiol.. 2014 Oct;103(4):e48-51.

 

Last Updated: 29 Oct 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Ebstein's anomaly" returned 3 free, full-text review articles on human participants. First 3 results:

Ebstein's anomaly.
 

Author(s): Christine H Attenhofer Jost, Heidi M Connolly, Joseph A Dearani, William D Edwards, Gordon K Danielson

Journal: Circulation. 2007 Jan;115(2):277-85.

 

Last Updated: 17 Jan 2007

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Ebstein's anomaly - review of a multifaceted congenital cardiac condition.
 

Author(s): C H Attenhofer Jost, H M Connolly, W D Edwards, D Hayes, Carole A Warnes, G K Danielson

Journal: Swiss Med Wkly. 2005 May;135(19-20):269-81.

 

Ebstein's anomaly (EA) is a rare but fascinating congenital heart disorder accounting for <1% of all congenital heart defects. Since its description in 1866, dramatic advances in diagnosis and therapy have been made. In this review, we describe current diagnostic criteria and classification, ...

Last Updated: 29 Jun 2005

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Electrode catheters and the diagnosis of Ebstein's anomaly of the tricuspid valve.
 

Author(s): H Watson

Journal: Br Heart J. 1966 Mar;28(2):161-71.

 

Last Updated: 25 Apr 1990

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Last Updated: 22 Sep 2016

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