Ebstein's Anomaly

Heart With Ebstein's Anomaly

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Ebstein's Anomaly is a rare defect, accounting for less than 1 percent of all congenital heart defects. The principal aberration occurring with Ebstein's Anomaly is a malformation of the tricuspid valve, which is located between the right atrium and right ventricle. Two of the three leaflets of the valve (the septal leaflet and the posterior leaflet) are displaced downward into the right ventricular cavity. These valve leaflets vary from mildly deformed to severely deformed. The third leaflet (the anterior leaflet) is not displaced but is typically large and redundant, often described as "sail-like". The portion of the right ventricle that sits above the displaced leaflets is usually thinner than normal and may be referred to as the atrialized portion of the right ventricle. A hole between the upper chambers of the heart, either an atrial septal defect or a patent foramen ovale, is virtually always present in association with Ebstein's anomaly. In some patients with this malformation, the pulmonary valve is also abnormal, either abnormally tight (pulmonary valve stenosis) or entirely closed (pulmonary valve atresia).

Most commonly, the deformed tricuspid valve has a tendency to leak, thus, as the right ventricle contracts some blood flows backwards from the right ventricle to the right atrium. Because of this backwards leakage of blood, a reduced volume of blood enters the right ventricle to be ejected to the lungs. The right atrium, which receives this leaking blood, is usually quite enlarged. Some of the blue blood from the right atrium may pass across the hole in the atrial septum into the left atrium. This blue blood then goes directly out to the body, which may be recognized as cyanosis in the patient. This is frequently present in newborns with Ebstein's anomaly and usually improves over the first weeks of life.

Infrequently, the deformed tricuspid valve forms an imperforate membrane. In this setting, rather than leaking, the tricuspid valve blocks blood from advancing into the right ventricle and out to the lungs.

In patients where the degree of valve deformity is mild and no symptoms are present, no intervention may be required other than prescribing antibiotics prior to dental or surgical procedures to prevent bacterial endocarditis. In patients with more severe involvement, cyanosis, shortness of breath, exercise intolerance and/or significant heart enlargement are often present and surgical intervention is warranted. The surgery usually consists of repairing or replacing the abnormal tricuspid valve and closing the hole between the atria.


What Is Normal Cardiac Anatomy?

When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

 
Learn More About Normal Cardiac Anatomy

Heart With Normal Cardiac Anatomy

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When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.

There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.

Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.

Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.

Patent Ductus Arteriosus (PDA)

What Is Patent Ductus Arteriosus?

Patent ductus arteriosus (PDA) is an extra blood vessel found in babies before birth and just after birth.

In most babies who have an otherwise normal heart, the PDA will shrink and close on its own in the first few days of life. If it stays open longer, it may cause extra blood to flow to the lungs. Problems are most likely if the PDA is large. Some smaller PDAs that don't close early will seal up on their own by the time the child is a year old.

Illustration: Healthy Heart

A patent ductus arteriosus (PAY-tent DUK-tus are-teer-ee-OH-sus) is more likely to stay open in a premature infant, particularly if the baby has lung disease. When this happens, doctors might need to close the PDA.

What Happens in Patent Ductus Arteriosus?

The ductus arteriosus is a normal blood vessel that connects two major arteries — the aorta and the pulmonary artery — that carry blood away from the heart.

Illustration: Patent Ductus Arteriosus

The lungs are not used while a fetus is in the womb because the baby gets oxygen directly from the mother's placenta. The ductus arteriosus carries blood away from the lungs and sends it directly to the body. When a newborn breathes and begins to use the lungs, the ductus is no longer needed and usually closes by itself during the first 2 days after birth.

If the ductus doesn't close, the result is a patent (meaning "open") ductus arteriosus. The PDA lets oxygen-rich blood (blood high in oxygen) from the aorta mix with oxygen-poor blood (blood low in oxygen) in the pulmonary artery. As a result, too much blood flows into the lungs, which puts a strain on the heart and increases blood pressure in the pulmonary arteries.

In infants born with other heart problems that decrease blood flow from the heart to the lungs or decrease the flow of oxygen-rich blood to the body, the PDA may actually help, and the doctor might prescribe medicine to keep the ductus arteriosus open.

What Causes Patent Ductus Arteriosus?

The cause of PDA is not known, but genetics might play a role. PDA is more common in premature babies and affects twice as many girls as boys. It's also common among babies with neonatal respiratory distress syndrome, babies with genetic disorders (such as Down syndrome), and babies whose mothers had rubella (also called German measles) during pregnancy.

What Are the Signs & Symptoms of Patent Ductus Arteriosus?

Babies with a large PDA might have symptoms such as:

  • a bounding (strong and forceful) pulse
  • fast breathing
  • not feeding well
  • shortness of breath
  • sweating while feeding
  • tiring very easily
  • poor growth

How Is Patent Ductus Arteriosus Diagnosed?

If a PDA is suspected, the doctor will use a stethoscope to listen for a heart murmur, which is often heard in babies with PDAs. Follow-up tests might include:

  • a chest X-ray
  • an EKG, a test that measures the heart's electrical activity and can show if the heart is enlarged
  • an echocardiogram, a test that uses sound waves to diagnose heart problems. These waves bounce off parts of the heart, creating a picture of the heart. In babies with PDA, an echo shows how big the opening is and how well the heart is handling it.
  • blood tests

How Is Patent Ductus Arteriosus Treated?

The three treatment options for PDA are medicine, catheter-based procedures, and surgery. A doctor will close a PDA if the size of the opening is big enough that the lungs could become overloaded with blood, a condition that can lead to an enlarged heart.

A doctor also might close a PDA to reduce the risk of developing a heart infection known as endocarditis, which affects the tissue lining the heart and blood vessels. Endocarditis is serious and requires treatment with intravenous (IV) antibiotics.

Reviewed by: Gina Baffa, MD
Date reviewed: October 05, 2017