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 Overview of Heart Defects About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more .)
Some studies have shown an increased risk of this abnormality in infants whose mothers used the medication lithium during pregnancy. Atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more 
, pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more , and Wolff-Parkinson-White syndrome Wolff-Parkinson-White (WPW) Syndrome Wolff-Parkinson-White syndrome is a disorder in which an extra electrical connection between the atria and the ventricles is present at birth. People may have episodes of a very rapid heartbeat... read more 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 Overview of Abnormal Heart Rhythms Abnormal heart rhythms (arrhythmias) are sequences of heartbeats that are irregular, too fast, too slow, or conducted via an abnormal electrical pathway through the heart. Heart disorders are... read more 
occurs.
Diagnosis of Ebstein Anomaly
Echocardiography
MRI
Echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more 
(ultrasonography of the heart) confirms the diagnosis.
Electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless procedure in which the heart’s electrical impulses are amplified and recorded. This record, the electrocardiogram (also known as an ECG)... read more 
(ECG) and chest x-rays X-Rays of the Chest Anyone thought to have a heart disorder has chest x-rays taken from the front and the side. Typically, the person is standing upright, but chest x-rays can be done with people lying in bed if... read more 
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.
American Heart Association: Common Heart Defects: Provides overview of common congenital heart defects for parents and caregivers
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
| Generic Name | Select Brand Names |
|---|---|
lithium |
Eskalith, Eskalith CR, Lithobid |
