Ebstein anomaly

Ebstein anomaly is a rare heart defect present from age of birth (congenital). If you suffer from this condition, the tricuspid valve is located not in the correct position, as well as the flaps of your valve (leaflets) are not formed properly. This means that the valve is not functioning correctly.

It is possible that blood leaks back into this valve and causes the heart to function less effectively. Ebstein anomalies may also result in an increase in the size of the heart as well as heart failure¹.

 

If you do not have any symptoms or signs that suggest Ebstein anomalies, a regular check-up of your heart could be all you require. It is possible to seek treatment when symptoms or signs are bothering you or if your heart is growing or becoming weaker. Treatment options include medication as well as surgery.

 

Symptoms

Mild types of Ebstein anomalies may not show symptoms until the later years of adulthood. Aspects and signs may include:

• Breathlessness, particularly when you exert yourself
• Fatigue
• Heart palpitations or irregular cardiac rhythms (arrhythmias)
• A blue-colored discoloration of the skin and lips due to the low levels of oxygen (cyanosis)

When should you see a doctor?

If your child or you is suffering from symptoms or signs of heart disease — for example, feeling fatigued or short of breath even when you’re doing normal activitiesor if the area around your lips and nails appears blue or you notice swelling in your legs, speak to your physician. They might refer you to a specialist who is specialized in congenital heart diseases (cardiologist).

Causes

Ebstein anomaly refers to a defect in the heart is present from birth (congenital). The reason for this is not known. To comprehend the way Ebstein anomalies affect the heart, it is helpful to understand how your heart functions to provide your body with blood.

How does your heart work

The heart is comprised of four chambers. The two chambers in the upper (atria) get blood. The lower chambers (ventricles) circulate blood.

Four valves are open and closed to allow blood flow to flow one way through the heart. Each valve is made up of two or three solid tiny flaps (leaflets) of tissue. A valve that is closed stops blood from flowing into the next chamber, or in returning back to the chamber that it was in.

Your body’s oxygen-poor blood flows into your right atrium. The blood is then channeled via the tricuspid valve and into the right ventricle. It then pumps the blood to the lung. On the opposite aspect of the heart, the oxygen-rich blood that comes from your lungs flow into the left atrium, which flows through the mitral valve, and then to the left ventricle which pumps blood throughout your body.

What happens in Ebstein anomaly?

The tricuspid valve is located within the heart’s right chambers (right ventricle and left atrium).

In Ebstein anomaly is a condition in which the tricuspid valve lies lower than the normal right ventricle. This causes some of the right ventricles is an atrium of the right leading to the right atrium in the atrium to grow and not function properly.

The leaflets of the tricuspid valve may not be properly made. This could lead to bleeding backward towards the left atrium (tricuspid valve regurgitation).

The position of the valve as well as how the valve’s structure is shaped is different from person to person. Certain people have an unusual valve. Some valves leak a lot.

Other heart problems that are related to Ebstein anomalies

Commonly, heart problems are related to:

• Holes within the heart. Many patients with Ebstein anomalies have holes between the two chambers in the heart referred to as an atrial septal deficiency, or an opening referred to as the patent foramen ovale (PFO). A PFO There is a hole in the upper and lower chambers of the heart that all infants have prior to birth. It usually closes after birth. It may be left open in certain individuals without causing any problems.The holes may reduce levels of oxygen in your bloodstream, resulting in an eerie blue discoloration of your skin and lips (cyanosis).
• Heartbeats that are abnormal (arrhythmias). An irregular heart rhythm or fast heartbeats makes an obstacle for your heart to function efficiently, particularly when the tricuspid valve leaks massively. Sometimes, a fast heartbeat can trigger fainting episodes (syncope).
• Wolff-Parkinson-White (WPW) syndrome. People who have WPW Syndromes suffer from an abnormal heart’s electrical system which can cause rapid heart rate and fainting episodes.

Risk factors

Heart defects that are congenital including Ebstein anomaly, can occur when the baby’s heart develops within the womb of the mother.

Doctors don’t know which risk factors are linked with the condition. Environmental and genetic factors are thought to play a part. A history of family members with heart disorders or a mother’s use or use of certain medicines like lithium during pregnancy may increase the chance of Ebstein anomalies in the child.

Complications

Mild Ebstein anomalies are unlikely to cause any issues.

But, the potential complications of Ebstein anomalies include:

• Heart failure
• Sudden cardiac arrest
• Stroke

A few precautions prior to starting a sport or getting pregnant can help avoid complications.

If your heart’s size is in the normal range and you don’t have any heart rhythm issues it is likely that you are able to participate in all sports. Based on your symptoms or symptoms, your physician might suggest you refrain from specific sports that require competitiveness such as basketball or football.

If you’re considering having a child, talk with your physician. A lot of women who have mild Ebstein anomalies can have children. But labor, pregnancy, and delivery place additional stress upon your heart. In rare instances, severe complications could occur that can lead to the death of the mother or baby.

Together with your physician, you can determine how much surveillance you will require during pregnancy and birth. Sometimes, additional treatments for your symptoms or condition could be recommended prior to when you are pregnant.

 

Diagnosis

If your child or you does not show any signs or symptoms of heart issues The doctor may suspect an issue only when they detect abnormal heart sounds in a regular physical exam.

The sounds of the heart like an irregular heartbeat, don’t typically reason to be concerned. However, your physician or the doctor of your child may send you to a specialist who is specialized in treating heart problems (cardiologist) to identify the reason behind the murmur.

 

Your doctor might recommend several tests, including:

• Echocardiogram. This test uses sound waves to create high-quality photographs of your heart. It illustrates the structure of the tricuspid valve as well as the flow of blood through your heart.Sometimes it is necessary to have a transesophageal echocardiogram carried out. The test is performed using the tube that contains a small microphone (transducer) that is inserted into the area of the digestive tract which connects the throat towards the stomach (esophagus). Since your esophagus is in close proximity to the heart of your body, this exam will reveal a clear picture of the heart.
• Electrocardiogram (ECG). Sensors (electrodes) placed on your chest and limbs monitor the frequency and timing that your heart beats. An ECG could help identify issues in your heart’s rhythm and can help identify issues with your heart’s rhythm and Certain personal devices, like smartwatches, provide remote ECG monitoring. Discuss with your doctor to determine if this is a possibility for you.
• Holter monitor. A Holter monitor is a handheld ECG The device you wear you are away from your doctor’s office. It tracks the electrical activity of your heart as you go about your daily routine for a few hours.
• Chest Xray. A chest X-ray is a photograph of the lungs, your heart as well as blood vessels. It will tell your doctor if your heart has in a state of expansion.
• Cardiac MRI . A cardiac MRI Utilizes radio waves and magnetic fields to produce precise photographs of your heart. The test provides your doctor with an in-depth look at the tricuspid valve. It lets your doctor assess the dimensions the heart’s chambers, as well as how they function.
• Pulse Oximetry. In this test, the sensor that is attached to your toe or finger measures the amount of oxygen present in your blood.
• Stress test for exercise. During this test, the blood pressure and heart rate, as well as your heart rhythm and breathing are observed while you run on a treadmill, or ride a stationary bicycle. A stress test during exercise will reveal how your heart reacts to exercise. It can assist your physician to determine what degree of exercise is safe for you.
• Study of Electrophysiology (EP). To conduct this test, the physician threads flexible, thin tubes (catheters) equipped with electrodes through blood vessels and into the areas of the heart to track the electrical signals of your heart.Furthermore, your doctor could use the electrodes to cause your heartbeat to a rate that could trigger or even stop an arrhythmia. This could help your physician determine if medication is needed in treating arrhythmia.
• Catheterization for the heart. A long, thin tube (catheter) is placed through a vein within your arm, groin, or neck. It is then carried to the heart via the X-ray technique. The injection of a specific dye through the catheter allows your doctor an enhanced picture of the flow of blood through your heart as well as blood vessels and valves. Through the procedure, your doctor can assess the pressures and oxygen levels inside the heart and identify issues within the lungs and the heart.

 

Treatment

Treatment for Ebstein anomalies is based on the degree of problem and the signs and symptoms you experience. The purpose of treatment is to minimize the symptoms and prevent future complications, like arrhythmias, heart failure, and arrhythmias.

Regularly monitored

If there are no indications or symptoms, or if you have irregular beats in your heart, your physician might suggest keeping track of your heart condition through regular checks.

A follow-up appointment typically includes exams and physical examinations like an electrocardiogram, echocardiogram Holter monitor test, and stress test.

Medications

If you suffer from irregular heart rhythms, medication may help regulate your heart rate, and keep a regular heart rhythm.

Your doctor may prescribe medication to reduce symptoms and signs that indicate heart disease, like medications to reduce the retention of water (diuretics).

If you suffer from certain heart rhythm disorders or an opening (atrial septal defect) between the upper and lower heart chambers, your medical professional could prescribe medication to stop blood clots.

A few babies receive an inhalable substance known as Nitric oxide, which helps improve the flow of blood to the lungs.

Other procedures or surgeries

The doctor may recommend surgery in the event that your symptoms are impacting your health. Surgery may also be suggested when your heart is expanding and the function of your heart decreases. If you’re considering surgery, you must select a surgeon who is experienced with the issue as well as one with knowledge and expertise in the procedures to fix it.

Different types of procedures can be used for the surgical treatment of Ebstein anomaly and the associated problems.

• Repair of the Tricuspid valve. Surgeons reduce the size of the valve opening to let the valve leaflets join to function correctly. A band may be put around the valve to hold it in its place. This process is generally performed when there is enough valve tissue that can allow repair.A more modern method of repair for the tricuspid valve is known as cone reconstruction. Surgery surgeons remove leaves of the valve and their heart muscles. The leaflets then are rotated and then reattached to form a “leaflet cone.”Sometimes, your valve may require repair or replacement in the near future.
• The replacement of the Tricuspid Valve. If the valve isn’t repairable surgically, the surgeon may take it off in order to substitute it an organ (bioprosthetic) or a mechanical valve. Mechanical valves aren’t commonly used for the replacement of tricuspid valves.If your heart has a valve that is mechanical and you’re using an anticoagulant to avoid blood clots. If you’re using any kind of artificial valve you’ll have to use a medication to prevent inflammation of the liner of your heart (endocarditis) prior to any dental procedure.
• The closure of atrial septal defects. If there’s a gap between the chambers that are higher in your heart (atrial septal defect) the surgeon will fix or replace the damaged valve. The surgeon is also able to repair other heart defects that you suffer from through this procedure.
• Maze process. If you have rapid heartbeats the surgeon can carry out the Maze procedure as part of valve replacement or repair surgery. The procedure is performed by the surgeon cuts small incisions into one of the chambers in your upper heart, to create an intricate pattern, also known as a maze, from scar tissue.Since scar tissue isn’t able to carry electricity around, it blocks the heart’s signals that trigger arrhythmias of various kinds. Cold temperatures that are extreme (cryotherapy) or warm (radiofrequency) energy may also be utilized to cause scars.
• Ablation of a radiofrequency catheter. If you have irregular or rapid heartbeats the doctor could perform this procedure. Your doctor will thread several catheters into the blood vessels and into your heart.Sensors located at the tips of the catheters make use of radiation (radiofrequency power) to destroy (ablate) tiny areas of the heart’s tissue. The ablated area blocks the abnormal heart signals that cause the arrhythmia. Some people may need repeat procedures.
• The process of transferring the heart. If you have an extremely Ebstein anomaly or heart dysfunction that is poor the possibility of a transplant to your heart could be required.

 

Helping to cope and providing support

Should you suspect that your kid is suffering from this mild Ebstein abnormality, read on to learn how is possible to do in order to control symptoms and increase comfort.

• Follow-up on medical treatment. See a cardiologist skilled with treating heart congenital diseases to check-up regularly. Notify any changes in symptoms or signs to your doctor. The timely treatment will help prevent the condition from getting worse.
• Use medications according to the prescription. Taking the right dose at the appropriate moment can help alleviate symptoms like heart racing, fatigue, and shortness of breath.
• Keep engaged. Be as physically active as the doctor of your child will allow. Exercise can improve the health of your heart and increase blood flow. Inviting breaks during play whenever necessary. Request a doctor’s note that you can hand to the child’s teachers or caregivers with a description of the restrictions on activities.
• Create an online Support community. Although many people with congenital heart issues lead a normal life, healthy ones but living having a problem with the heart may be a challenge for those who have children or requires special care. A serious health issue can cause mental, physical, and financial pressure. Being able to have family and friends with whom you can trust is crucial. Many people find support groups to be an excellent source of advice, comfort, and companionship.