EBSTEIN’S ANOMALY
Doreen Fofonoff, MN, RN, CCN(C)
In the normal heart, the tricuspid valve has three "flaps" or leaflets. The valve splits the top right heart chamber (right atrium) from the bottom right heart chamber (right ventricle). The leaflets of the valve act as one-way gates, letting blood flow from the right upper chamber into the right lower chamber. The valve leaflets close once the blood has flowed into the right lower chamber. This stops the blood from leaking back into the right upper chamber when the right lower chamber pumps blood into the lungs.
In Ebstein's anomaly, the tricuspid valve is moved downward into the right lower chamber. This may cause the right upper chamber to be larger than usual and the right lower chamber to be smaller. As well, one or two of the three valve leaflets may be stuck to the wall of the heart. As a result, the leaflets do not close properly and may leak. When the valve leaks, some of the blood pumped by the right lower chamber flows backwards into the right upper chamber. As a result, the heart does not work as well. The right upper chamber of the heart may enlarge as a result of the increased blood that leaks back into it.
Over half of the people with Ebstein’s anomaly also have a hole in the wall between the heart's two upper chambers (atria). This hole is called an atrial septal defect or ASD. The hole causes oxygen rich blood from the left upper chamber to blow into the right upper chamber with each heart beat. This decreases the amount of blood that would normally go forward to the body.
One in four patients with Ebstein’s anomaly may also have abnormal heart rhythms called arrhythmias. These rhythms are most often fast, but may also be slow.
Diagnosis of Ebstein’s Anomaly
Severe cases of Ebstein’s are often diagnosed shortly after birth. Ebstein’s may not be found until a person starts to have problems (symptoms) in their late teens or early adult life. People with mild Ebstein’s may not have any problems at all and their defect may be found during routine exams.
The heart doctor (cardiologist) may confirm the diagnosis of Ebstein’s anomaly by doing a full physical exam and several tests. These tests may include:
- An ECG (electrocardiogram) to look for an increase in size of the right upper chamber and abnormal heart rhythms.
- An echocardiogram (ultrasound of the heart) to look for a leaky and abnormal tricuspid valve, a large right upper chamber, and a small right lower chamber. This test will also provide information about how well the heart pumps.
- A chest x-ray to look for an increase in the size of the heart.
- A Holter monitor (24 hour ECG) to look for abnormal heart rhythms.
- An exercise stress test to see how much the person can exercise and to see if abnormal heart rhythms occur during exercise.
People with mild Ebstein’s may not have any symptoms at all. However, those with moderate or severe Ebstein’s may have shortness of breath with exercise, decreased energy, dizziness, lightheadedness, feel tired, or have slow or very rapid heart rhythms (arrhythmias) or blueness of the lips, fingers or toes (cyanosis).
Treatment Options
Treatment of Ebstein’s depends on the severity of the defect and the person’s symptoms. The heart doctor may advise regular check-ups if the person has no symptoms or no abnormal heart rhythms. If symptoms are present, medication may be given to improve the work of the heart. Medicine may also be used to prevent fast abnormal heart rhythms. For some people, the source of the rapid heart rhythm may also be removed by a procedure called radiofrequency ablation. The ablation uses sound waves to destroy the tissue that is causing the abnormal rhythm.
Surgery may be advised when the patient has severe symptoms, or when the heart starts to enlarge and the heart does not work as well. The surgeon may repair the tricuspid valve or replace it with a tissue or mechanical valve. If there is an ASD, it is usually closed at the same time. A Maze procedure may be done during surgery if the person has abnormal fast heart rhythms. During this procedure, the surgeon makes a series of cuts in the right upper chamber of the heart. These cuts interrupt the electrical pathways that cause the abnormal heart rhythm.
Ongoing Care:
Regular Check-Ups: Ebstein’s anomaly is uncommon so people with Ebstein's anomaly should receive ongoing care with regular check-ups at an adult congenital heart centre where the heart doctors have expertise in congenital heart defects. Regular check-ups allow the heart doctor to detect problems early so that the best care and treatments can be offered.
Report Symptoms: Symptoms such as decreased stamina, increased shortness of breath, fatigue, cyanosis or episodes of rapid heart rhythm, fainting, or swelling of the feet and legs should be reported to the family doctor or heart doctor. These problems may develop or worsen as the valve leaks more.
Activity Restrictions: Being physically active is good for the heart, so people with Ebstein’s should stay active. Ask the heart doctor which activities are safe. If the valve leakage is mild and tests show no abnormal heart rhythms, people can usually perform most physical activities. However, if the valve leakage is moderate or severe, the heart doctor may advise some physical activities be avoided.
Endocarditis Prevention: Endocarditis is an infection that can damage the inner lining of the heart or the heart valves. People with Ebstein’s anomaly are at risk of endocarditis. They usually need antibiotics before certain dental or surgical procedures to prevent endocarditis. As well, they should also look after their teeth and gums and avoid behaviors that may increase the risk of endocarditis (intravenous drug use, body piercing and tattooing).
Pregnancy: Pregnancy puts many demands on the normal heart. Patients with Ebstein’s should consult with their heart doctor about the safety of pregnancy, before they become pregnant. The heart doctor will assess what effect the pregnancy will have on the heart valve and the work of the heart. The heart doctor will decide what care is required before, during and after the pregnancy.
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