Ebstein Anomaly

(Ebstein's Anomaly)

ByLee B. Beerman, MD, Children's Hospital of Pittsburgh of the University of Pittsburgh School of Medicine
Reviewed/Revised Apr 2023

Ebstein anomaly is an abnormality of the tricuspid valve that causes malfunction of the valve with regurgitation (blood leaking backward into the atrium) or stenosis (obstruction to blood flowing into the right ventricle) The right side of the heart may become very enlarged.

  • Symptoms depend on the child's age when symptoms begin.

  • The newborn may have cyanosis (a bluish color of the skin).

  • Abnormal heart rhythms may develop at any age.

  • Diagnosis is with electrocardiography (ECG) and imaging of the heart.

  • Treatment is with medication and surgery.

(See also Overview of Heart Defects.)

Atrial septal defect, pulmonic valve stenosis, and Wolff-Parkinson-White syndrome often occur together with Ebstein anomaly.

Symptoms of Ebstein Anomaly

Newborns' skin may be bluish in color (indicating the body is not receiving enough oxygen), or symptoms may not develop until adulthood, when an abnormal heart rhythm occurs.

Diagnosis of Ebstein Anomaly

  • Echocardiography

  • MRI

Echocardiography (ultrasonography of the heart) confirms the diagnosis.

Electrocardiography (ECG) and chest x-rays are typically done.

Magnetic resonance imaging (MRI) provides valuable additional detail regarding the tricuspid valve, which helps doctors plan surgery.

Treatment of Ebstein Anomaly

  • Medication

  • Surgery

Medication is needed when newborns have severe cyanosis. Medications called prostaglandins may be given to help keep the ductus arteriosus open to allow the right ventricle to maintain blood flow until surgery can be done.

If the abnormal tricuspid valve causes severe symptoms or enlargement of the heart, the valve is surgically repaired.

More Information

The following English-language resources may be useful. Please note that The Manual is not responsible for the content of these resources.

  1. American Heart Association: Common Heart Defects: Provides overview of common congenital heart defects for parents and caregivers

  2. American Heart Association: Infective Endocarditis: Provides an overview of infective endocarditis, including summarizing prophylactic antibiotic use, for patients and caregivers

Drugs Mentioned In This Article
Test your KnowledgeTake a Quiz!
Download the free Merck Manual App iOS ANDROID
Download the free Merck Manual App iOS ANDROID
Download the free Merck Manual App iOS ANDROID