Ebstein's anomaly of the tricuspid valve (patient information)

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 * Associate Editors-In-Chief: Priyamvada Singh, MBBS [mailto:psingh@perfuse.org]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
Ebstein's anomaly is a rare heart defect in which parts of the tricuspid valve are abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium). This condition is congenital, which means it is present from birth.

What are the symptoms of Ebstein's anomaly of the tricuspid valve?
Symptoms of Ebstein's anomaly develop after birth and inlcude bluish-colored lips and nails due to low blood exygen levels. Symptoms range from mild to severe. In severe cases, the baby appears very sick and has trouble breathing. However in older children symptoms are mild, and include:
 * Cough
 * Failure to grow
 * Fatigue
 * Rapid breathing
 * Shortness of breath
 * Very fast heartbeat

What are the causes of Ebstein's anomaly of the tricuspid valve?
The exact cause of Ebstein's anomaly is unknown, however the use of certain drugs (such as lithium or benzodiazepines) during pregnancy may play a role. Ebstein's anomaly occurs as a baby develops in the womb. This condition is rare but it is more common among Caucasians. The tricuspid valve is made up of three parts, called leaflets or flaps. These leaflets or flaps allow blood to flow from the right atrium (top chamber) to the right ventricle (bottom chamber) while the heart relaxes. The leaflets close to prevent blood from flowing from the right ventricle to the right atrium while the heart pumps. The leaflets are unusually deep in the right ventricle and are often larger than normal in cases of Ebstein's anomaly. Due to the defect, blood may go the wrong way back into the right atrium, when it should be flowing to the right ventricle. When the blood gets backed up it can lead to heart swelling and fluid buildup in the lungs or liver. Sometimes, blood can't get out of the heart into the lungs and the person may appear blue. However in most cases of Ebstein's anomaly, patients have a hole in the wall which separates the heart's two upper chambers and the blood flow across the hole. This may cause oxygen-poor blood to go to the rest of the body. There may also be narrowing of the valve that leads to the lungs (pulmonary valve).

Who is at highest risk?
Since the exact cause of Ebstein's anomaly is unknown, it is difficult to distinguish who is at risk. However, the use of certain drugs (such as lithium or benzodiazepines) during pregnancy may play a role. Even though this condition is very rare, it presents itself more among the Caucasian race.

When to seek urgent medical care?
Call your health care provider if your child develops symptoms of this condition. Seek immediate medical attention if breathing problems occur.

Diagnosis
There are many exams and tests that can diagnose this condition. However, for newborns who have severe leakage across the tricuspid valve will have very low levels of oxygen in their blood and significant heart swelling.This can be diagnosed by the doctor listening to the chest with a stethoscope to hear abnormal heart sounds, such as a murmur. Other tests that can help diagnose this condition include:
 * Chest x-ray
 * Magnetic resonance imaging (MRI) of the heart
 * Measurement of the electrical activity of the heart (EKG)
 * Ultrasound of the heart (echocardiogram)

Treatment options
Treatment options vary of the severity of the defect and the specific symptoms. Medical care may include:
 * Medications to prevent heart failure
 * Oxygen and breathing support
 * Surgery to correct the valve may be necessary for children who continue to worsen or who have more serious complications

Where to find medical care for Ebstein's anomaly of the tricuspid valve?
Directions to Hospitals Treating Ebstein's anomaly of the tricuspid valve

What to expect (Outlook/Prognosis)
The earlier the symptoms develop, the more severe the disease. The symptoms vary in severity, some patients have either no symptoms or very mild symptoms and others may worsen over time, developing blue coloring (cyanosis), heart failure, heart block, or dangerous heart rhythms. A severe leakage may lead to swelling of the heart and liver and congestive heart failure. Other complications may include:
 * Abnormal heart rhythms (arrhythmias), including abnormally fast rhythms (tachyarrhythmias) and abnormally slow rhythms (bradyarrhythmias and heart block)
 * Blood clots from the heart to other parts of the body
 * Brain adscess



Prevention
There is no prevention of this condition, other than talking to your doctor before pregnancy if you are taking medication that are thought to be related to developing this disease.