o diagnóstico no útero de anomalia de Ebstein e determinar com certeza o prognóstico .. lares en la enfermedad de Ebstein. Arch Inst Cardiol Mex. Ebstein’s anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle . Request PDF on ResearchGate | On Mar 1, , Juan-Miguel Gil-Jaurena and others published Cirugía de cono en la enfermedad de Ebstein.

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Ebstein anomaly is a rare heart defect in which the tricuspid valve — the valve between the upper right chamber right atrium and the lower right chamber right ventricle of the heart — doesn’t work properly. As a result, blood leaks back through the valve and into the right atrium.

Atrial septal defect is a hole between the two upper chambers of the heart. About half the people with Ebstein anomaly have an atrial septal defect. Ebstein anomaly is a rare heart defect that’s present at birth congenital. In Ebstein anomaly, your tricuspid valve — the valve between the two right heart chambers right atrium and right ventricle — doesn’t work properly. The tricuspid valve sits lower than normal in the right ventricle, and the tricuspid valve’s leaflets are enfermedsd formed.

Blood may leak back through the valve, dd your heart work less efficiently. Ebstein anomaly may also lead to enlargement of the heart or heart failure. If you have no signs or symptoms associated with Ebstein anomaly, careful monitoring of your heart may be all that’s necessary. If signs and symptoms bother you, or if the heart is enlarging or becoming weaker, treatment for Ebstein anomaly may be necessary.

Treatment options include medications and surgery. Mild forms of Ebstein anomaly may not cause symptoms until later in adulthood. If signs and dd are present, they may include:.

If you or your child has signs or symptoms of heart failure — such as feeling easily fatigued or short of breath, even with normal activity — or is showing blue skin coloration around the lips and nails cyanosistalk to your doctor. He or she may enefrmedad you to a doctor who specializes in ebsteun heart disease cardiologist. A normal heart has two upper and two lower chambers.

The upper chambers, the right and left atria, receive incoming blood.

The lower chambers, the more muscular right and left ventricles, pump blood out of your heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings. Ebstein anomaly is a heart defect enfermedxd you have at birth congenital. Why it occurs is still unknown.

To understand how Ebstein anomaly affects your heart, it helps to know a little about how the heart enfermeead to supply your body with blood. Your heart is made up of four chambers. The two upper chambers atria receive evstein. The two lower chambers ventricles pump blood. Four valves open and close to let blood flow in one direction through the heart.


Each valve consists of two or three strong, thin flaps leaflets of tissue.

Ebstein’s anomaly

When closed, a valve prevents blood from flowing to the next chamber ebsteij from enfermeadd to the previous chamber. Oxygen-poor blood returns from your body and flows into the right atrium.

Blood then flows through the tricuspid valve and into the right ventricle, which pumps the blood to your lungs to receive oxygen. On the other side of your heart, oxygen-rich blood from your lungs flows into the left atrium, through the mitral valve and into the left ventricle, which then pumps the blood to the rest of your body. In Ebstein anomaly, the tricuspid valve sits lower than normal in the right ventricle.

This makes it so that a portion of the right ventricle becomes part of the right atrium becomes atrializedcausing the right atrium to be larger than usual. Because of this, the right ventricle can’t work properly. Also, the tricuspid valve’s leaflets are abnormally formed.

This can lead to blood leaking backward into the right atrium tricuspid valve regurgitation. The placement of the valve and how poorly it’s formed may vary among people.

Some people may have a mildly abnormal valve. Others may have a valve that is extremely displaced, and it may leak severely. The more the tricuspid valve leaks, the more the right atrium enlarges as it receives more blood. At the same time, the right ventricle enlarges dilates as it tries to cope with the leaky valve and still deliver blood to the lungs. Thus, the right-sided chambers of the heart enlarge, and as they do, they weaken, which may lead to heart failure.

Several other heart enfermedqd may be associated with Ebstein anomaly. A few common conditions include:. Holes in the heart. Many people with Ebstein anomaly have a hole between the two upper chambers of the heart called an atrial septal defect or a small flap-like opening called a patent foramen ovale PFO.

A PFO is a hole between the upper heart chambers that is present in all babies before birth but usually closes after birth, although it may remain open in some people without causing issues. These holes dde allow oxygen-poor blood in the right atrium to mix ebdtein oxygen-rich blood in the left atrium, decreasing the amount enfermedqd oxygen available in your blood. This causes a bluish discoloration of the lips and skin cyanosis.

Some people with Ebstein anomaly have an abnormal heart rhythm arrhythmia characterized by rapid heartbeats tachycardia. These types of arrhythmias can make your heart work less effectively, especially when the tricuspid valve is leaking severely.

In some cases, a very fast enfermedaad rhythm may cause fainting spells syncope. Congenital heart defects, such as Ebstein anomaly, happen early in the development of a baby’s heart. It’s uncertain what risk factors might cause the defect. Entermedad and environmental factors are ds thought to play a role. People with a family history of heart defects may be more likely to enfegmedad Ebstein anomaly. A mother’s exposure to certain medications, such as lithium, ebsein be associated with Ebstein anomaly in the child.


Many people with mild Ebstein anomaly have few complications. However, you may need to take some precautions in certain situations:.

If you have mild Ebstein anomaly with a nearly normal heart size and no heart rhythm disturbances, you can probably participate in most physical activities.

Depending on your signs and symptoms, your doctor may recommend that you avoid certain competitive sports, such as football or basketball. Your doctor can help you decide which activities are right for you. In many cases, women with mild Ebstein anomaly can safely have children. But pregnancy does have risks. If you plan on becoming pregnant, be sure to talk to your doctor ahead of time.

He or she can tell you if it’s safe for you to become pregnant and help decide how much extra monitoring you may need throughout pregnancy and childbirth. He or she may also suggest other treatments for your condition or symptoms before you become pregnant. Being pregnant puts additional strain on your heart and circulatory system not only during pregnancy, but also during labor and delivery. However, vaginal delivery may be possible.

Rarely, severe complications can develop that can cause death to the mother or baby. Other complications that may result from Ebstein anomaly include heart failure, heart rhythm problems and, less commonly, sudden cardiac arrest or stroke.

Ebstein anomaly care at Mayo Clinic. Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission.

This content does not have an English version. This content does not have an Arabic version. Ebstein anomaly Ebstein anomaly is a rare heart defect in which the tricuspid valve — the valve between the upper right chamber right atrium and the lower right chamber right ventricle of the heart — doesn’t work properly.

Ebstein’s anomaly – Wikipedia

Request an Appointment at Mayo Clinic. Chambers and valves of the heart A normal heart has two upper and two lower chambers. Fuster V, et al. Congenital heart disease in children and adolescents. The McGraw-Hill Companies; Agarwala BN, et al. Ebstein anomaly of the tricuspid valve. What are congenital heart defects? National Heart, Lung, and Blood Institute. Patent foramen ovale PFO. Congenital heart disease in adults. Katsuragi S, et al.

Risk factors for maternal and fetal outcome in pregnancy complicated by Ebstein anomaly. American Journal of Obstetrics and Gynecology. What is an arrhythmia?

Dearani JA, et al. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. The Annals of Thoracic Surgery. Anderson HN, et al. Cone reconstruction in children with Ebstein anomaly: The Mayo Clinic experience.