Description

Ebstein anomaly is a rare heart disease that is present at birth. That means that it is a congenital defect of the heart.

In this condition, the valve that separates the top and bottom of the right side of the heart chambers does not form correctly. This valve is called the tricuspid valve. As a result, the valve does not close as it should. The blood moves backwards from the bottom to the top of the camera, making it more difficult for the heart to work.

In people with Ebstein anomaly, the heart can grow bigger. The condition can lead to heart failure.

Treatment of Epstein's anomaly depends on the symptoms. Some people without symptoms, only the need for regular health checkups. Others may need medication and surgery.

Symptoms

Some babies are born with Ebstein anomaly have few or no symptoms. Others have a tricuspid valve leaking severely and becomes more noticeable symptoms. Sometimes the symptoms do not occur until later in life.

The symptoms of Epstein's anomaly may include:

  • Blue or gray lips or fingernails. Depending on the color of the skin, these color changes may be more difficult or more easy to see.
  • Fatigue.
  • Sensation of a pounding or fast heartbeat or irregular heartbeat.
  • Shortness of breath, especially with activity.

When to see a doctor

Serious diseases of the heart of a baby is often diagnosed at the time of birth or during the pregnancy.

Make an appointment if you or your baby has symptoms of a possible heart disease. These symptoms include shortness of breath, or easily tired, with little activity, irregular heartbeat, or changes in the color of the skin. You may be sent to a physician skilled in diseases of the heart, called a cardiologist.

Causes

Ebstein anomaly is a condition of the heart that a person is born. The cause is not known. To understand more about Epstein's anomaly, it can help to know how the heart works.

How the heart works

The typical heart has four chambers.

  • The two upper chambers are called atria. They receive the blood.
  • The two lower chambers are called the ventricles. The blood pump.

Four valves open and close to allow blood to flow in only one direction through the heart. Each valve has two or three strong, thin flaps of tissue. The flaps are called leaflets or cusps.

  • A valve that is closed let the blood flow in the next room.
  • A closed valve also prevents the blood to return to the previous camera.

In a typical heart, the tricuspid valve is located between the two chambers of the heart. In Epstein's anomaly of the tricuspid valve is less than the usual in the right lower chamber of the heart. Also, the shape of the tricuspid valve of the flaps is changed. This can cause the blood to flow backward in the upper right part of the heart of the camera. When this happens, the condition is called regurgitation of the tricuspid valve.

Heart conditions associated with Epstein anomaly

Babies who are born with Ebstein anomaly can have other heart conditions, including:

  • Holes in the heart. A hole in the heart can reduce the amount of oxygen in the blood. Many infants with Ebstein anomaly is a hole between the two upper chambers of the heart. This hole is called an atrial septal defect. Or there may be an opening, called a patent foramen ovale, also called a PFO. A HOLE is a hole between the upper chambers of the heart that all have babies before they are born. It usually closes after birth. But it can remain open in some people.
  • Irregular heartbeats, called arrhythmias. An irregular heartbeat may include palpitations, pounding or throbbing of the heart. The changes in the beat of your heart may make it more difficult for the heart to work as it should.
  • The Wolff-Parkinson-White (WPW) syndrome. In this condition, an extra of the signaling pathway between the heart's upper and lower chambers causes a rapid heartbeat and fainting.

Risk factors

Epstein's anomaly occurs as the baby, also called a fetus grows in the uterus during pregnancy.

Researchers are not sure exactly what that increases the risk of Epstein's anomaly. The genetic and environmental factors are involved. The use of some medications during pregnancy, such as lithium may increase the risk of Ebstein anomaly in the baby.

Complications

The possible complications of Epstein's anomaly include:

  • Irregular heart beats.
  • Heart failure.
  • The sudden cardiac arrest.
  • Stroke.

Pregnancy and Ebstein anomaly

It is possible to have a successful pregnancy with mild Ebstein anomaly. But pregnancy, labor and delivery put more strain on the heart. Rarely, the extreme complications can develop that can cause serious health problems in the mother or the baby.

Before getting pregnant, talk with your health care team about the possible risks and complications. Together, you can plan for any special care during pregnancy.

Diagnosis

To diagnose Ebstein anomaly, a health care professional examines the baby and hear the heart and the lungs. A heart sound called a murmur may be heard.

Tests

Tests that are done to help diagnose Ebstein anomaly include:

  • Pulse oximetry. In this test, a sensor attached to a finger or toe-measures the amount of oxygen in the blood.
  • The echocardiogram. Sound waves are used to create images of the beating heart. An echocardiogram can show how blood flows through the heart and the heart valves.
  • Electrocardiogram (ECG or EKG). This simple test checks the heart beat. Adhesive patches to attach in the chest and sometimes the arms and legs. The cables connect the patches to a computer, that prints or displays the results.
  • Holter Monitor. This portable ECG device is worn by a day or more, to record the heart's activity during daily activities.
  • X-ray of the chest. An x-ray is an image of the heart, lungs, and blood vessels. It can show whether the heart is enlarged.
  • Cardiac magnetic resonance. A cardiac mri uses magnetic fields and radio waves to create images of the heart. This test can give a detailed view of the tricuspid valve. It also shows the size of the heart chambers and how they work.
  • The stress tests. These tests often involve walking on a treadmill or riding a stationary bike while the heart is on. An exercise stress test can show how the heart responds to exercise.
  • Electrophysiology study (EP study). A doctor threads a thin, flexible tube called a catheter into a blood vessel and the guide for the heart. More of a catheter can be used. The sensors in the tip of the catheter emit electrical signals and record the heart of the electricity. This test helps to show that part of the heart is the cause of a fast or irregular heartbeat. Treating an irregular heartbeat can be done during this test.
  • The cardiac catheterization. A doctor inserts a long, thin, flexible tube into a blood vessel, usually in the groin or wrist. Is guided into the heart. Contrast medium flows through the tube of the arteries in the heart. The dye helps the arteries to show up more clearly on X-ray images and video. During the test, the pressure and oxygen levels can be measured in different parts of the heart. Some heart disease treatments can also be done during this test.

Treatment

Treatment of Epstein's anomaly depends on:

  • The severity of the condition.
  • Symptoms.

The goals of treatment are to:

  • To reduce the symptoms.
  • To prevent complications, such as irregular heartbeat and heart failure.

The treatment may include regular health checkups, medications, or a heart procedure or surgery.

Regular medical checkups

If Epstein's anomaly does not cause irregular heart beats, or other symptoms, regular medical checkups can be all you need.

The checks are usually performed at least once a year. The exam usually includes a physical exam and imaging tests to look for in the heart.

Drugs

If you have Epstein's anomaly, you may receive medicine to:

  • Control irregular heartbeats or other heart rhythm changes.
  • Prevent the accumulation of fluid in the body. Too much fluid in the body can be a sign of heart failure.
  • Prevent blood clots. Blood clots can occur if the condition also occurs with a hole in the heart.

Some babies also have a inhalation of the substance called nitric oxide to help improve the flow of blood to the lungs.

Surgery or other procedures

The surgery is usually done if Epstein anomaly causes severe tricuspid regurgitation and is not heart failure or the difficulty increases with exercise.

The surgery may also be recommended if other symptoms, such as irregular heartbeats, are very bad or that affect the quality of life.

If surgery is needed, it is important to choose a surgeon who is familiar with Epstein's anomaly. The surgeon should have training and experience to perform the procedures to correct the condition.

Surgery to treat Epstein's anomaly and associated with heart disease may include:

  • Repair of the tricuspid valve.This open-heart surgery corrects a damaged tricuspid valve. The surgeon can patch holes or tears in the valve flaps or remove excess tissue around the opening of the valve. Other repairs can also be done. A type of repair of the valve called the cone procedure can be done. The heart surgeon separates the muscle of the heart from the tissue that should have been formed of the tricuspid valve. The fabric is used to create a work of the tricuspid valve. Sometimes, the valve needs to be repaired again or replaced in the future.
  • The replacement of the tricuspid valve. If the valve cannot be repaired, surgery to replace the valve may be necessary. The replacement of the tricuspid valve surgery may be performed open-heart surgery or minimally invasive surgery. The surgeon removes the damaged or diseased valve and replaces it with a valve from a cow, pig or human heart tissue. This is called a biologic valve. Mechanical valves are not often used for the replacement of the tricuspid valve.
  • Closure of the atrial septal defect. This surgery is done to fix a hole between the upper chambers of the heart. Other heart conditions can also be repaired during this surgery.
  • Maze procedure. If Epstein anomaly causes an irregular heart beat, this treatment can be performed during valve repair or replacement surgery. The surgeon makes small incisions in the upper chambers of the heart to create a pattern of scar tissue. The pattern is called a labyrinth. The heart of the signals can't pass through the tissue of the scar. As well, the labyrinth of blocks of irregular heart rhythms. The heat or the cold of the energy can also be used to create scars.
  • Radiofrequency catheter ablation.This treatment is performed to correct the fast or irregular heartbeats. The doctor inserts one or more thin, flexible tubes called catheters into a blood vessel, usually in the groin area. The doctor's guide to the heart. The sensors in the tip of the catheters, the use of heat, called the radio frequency energy to cause damage to a small area of heart tissue. This creates scars, which blocks the heart of the signals that cause irregular heartbeats.
  • Heart transplant. If it is severe Ebstein anomaly is causing the heart to fail, a heart transplant may be required.

Repair of the tricuspid valve. This open-heart surgery corrects a damaged tricuspid valve. The surgeon can patch holes or tears in the valve flaps or remove excess tissue around the opening of the valve. Other repairs can also be done.

A type of repair of the valve called the cone procedure can be done. The heart surgeon separates the muscle of the heart from the tissue that should have been formed of the tricuspid valve. The fabric is used to create a work of the tricuspid valve.

Sometimes, the valve needs to be repaired again or replaced in the future.

Radiofrequency catheter ablation. This treatment is performed to correct the fast or irregular heartbeats. The doctor inserts one or more thin, flexible tubes called catheters into a blood vessel, usually in the groin area. The doctor's guide to the heart.

The sensors in the tip of the catheters, the use of heat, called the radio frequency energy to cause damage to a small area of heart tissue. This creates scars, which blocks the heart of the signals that cause irregular heartbeats.

Coping and support

These tips can help you manage Epstein's anomaly symptoms and improve comfort.

  • Get health checkups. Choose a center physician trained in the treatment of diseases of the heart present at birth. This type of doctor is called a congenital cardiologist. Tell your health care team if there are any new symptoms, or if symptoms worsen.
  • Take the medicine as he was told. Taking the right dose at the right time can help to relieve symptoms such as heart palpitations, fatigue and shortness of breath.
  • Stay active. Be physically active as possible. Exercise can help to strengthen the heart and improve blood flow. Ask your healthcare team how much exercise is appropriate for you or your child. Some people who are born with heart disease may need to limit your exercise or sports activities. But many others with a congenital heart defect may participate in such activities.
  • Create a useful network. Living with a disease of the heart can make some people feel stressed or anxious. Talking with a therapist or a counselor can be helpful.
  • Join a support group. Talk with other people who have experienced the same situation can bring comfort and encouragement. Ask your health care team if there are any of Epstein's anomaly support groups in your area.

Preparing for your appointment

You may be sent to a physician trained in the treatment of diseases of the heart, the flame of a cardiologist. Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do ahead of time. For example, you may be asked not to eat or drink anything for a while before certain tests.

Make a list of:

  • The symptoms, including any that may seem unrelated to Epstein's anomaly. Note when you began.
  • Important personal information, including a family history of heart disease.
  • All the medications, vitamins, or other supplements. Include the dose.
  • Questions to ask your health care team.

If you are seeing a new healthcare provider, to obtain a copy of the medical records. Send to the new office.

For Epstein's anomaly, specific questions to ask the health care professional include:

  • What is the most likely cause of these symptoms?
  • What tests are needed?
  • What treatments are available? Which do you recommend and why?
  • What are the side effects of treatment?
  • Are there any dietary or activity restrictions?
  • Is there any information that I can take home? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your healthcare provider is likely to ask you questions, such as:

  • The symptoms come and go, or do they happen all the time?
  • How severe are the symptoms?
  • Is there something to improve the symptoms?
  • What, if anything, makes the symptoms worse?
Symptoms and treatment of the anomaly Epstein