• The Heart House

Problems with the Hallways

The hallways leading away from the heart:

1. Aorta

Problem: Coarctation of the Aorta

If this hallway is narrowed as it makes the bend to go down to the body it is called Coarctation of the Aorta.

2. Pulmonary Artery

Problem 1: Branch Pulmonary Artery Stenosis

Problem 2: Transposition of Great Arteries
Fix: Prostaglandin, Atrial Septostomy, or Switch Operation

If the pulmonary artery is narrowed as it branches to either the right or left lung this condition is called Branch Pulmonary Artery Stenosis.

When these 2 main hallways leading away from the heart are swapped or transposed, it is called Transposition of Great Arteries.

The hallways emptying into the heart:

3. Pulmonary Veins

Problem: Total Anomalous Pulmonary Venous Connection

If the Pulmonary Veins that normally lead to the left atrium are rerouted to the wrong vein, this condition is called Total Anomalous Pulmonary Venous Connection.

1. Coarctation of the Aorta

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Problem with the aorta

Coarctation of the Aorta is a congenital heart defect that happens when there is a narrowing of the aorta as it makes the turn to supply the lower part of the body. The coarctation (narrowing) is usually located, just after the takeoff of the left subclavian artery (the branch to the left arm).

The left heart pump (Left Ventricle), then has to pump harder to force the blood to get through the narrowed area, so that the abdomen and legs can receive enough blood, with time the left ventricle muscle becomes thicker (hypertrophied).

The blood pressure gets higher, so as to push the blood across the narrowed area. As the blood squeezes through the obstructed area, it scatters under high pressure into strong jets; most of which move the blood forward but others hit hard against the walls of the aorta. Since so much moving energy is wasted in trying to force the blood through, the pressure beyond the narrowed area itself will be low.

In your child, this leads to high pressure in the arms and low pressure in the legs. High pressure in the arms would also be transmitted to high pressure to the arteries going to the brain. If this pressure goes untreated it may affect the arteries in the brain and cause stroke.


Because of high blood pressure in the in the upper part of the body, your child may complain of headaches, nose bleeds, or blurred vision. And since the lower body is receiving blood at low pressure, the growth of the lower body be compromised, the lower body will look small compared with the upper body. The legs may be shorter than usual, cold, and when your child tries to play or run he may complain of pain in his calves (intermittent claudication). This is like muscle cramps but they are caused by a lack of blood supply to exercising muscles.

If the doctor finds that your child has high blood pressure it is very important to check that he does not have coarctation of the Aorta. If he complains of his calves hurting when he plays that also the pulses in his legs are normal and he does not have decreased pulses. It is very important to recognize and treat this condition very early, because if the blood pressure stays high for a long time, after correcting the defect the blood pressure may not return back to normal and your child may need medication for his entire life.

Below is a body with a normal Left Ventricle and Aorta.

Here is a body with Coarctation of the Aorta.

Here's a "heart-house" depiction of coarctation of the aorta:

2a. Branch Pulmonary Artery Stenosis

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Problem with the pulmonary artery

Branch Pulmonary Artery Stenosis is characterized by small branch pulmonary arteries going to the lungs. Below, you can see how this defect looks in the heart.

At right you can see how this defect looks in a baby. The small pulmonary arteries create a heart murmur. A heart murmur is a swishing or whistling sound the doctor can hear when he listens to your child's heart through a stethoscope. To learn more about heart murmurs, click here.

2b. Transposition of Great Arteries

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Problem with the pulmonary artery

Transposition of the Great Arteries is a problem with the hallways coming out of the heart.

In a normal heart the Aortic hallway originates from the Left Ventricle and carries pink blood (with oxygen) from the Left Ventricle to the body. When this defect is present the Aortic hallway originates from Right Ventricle and therefore carries blue blood (no oxygen) to the body.

In a normal heart the Pulmonary hallway originates from the Right Ventricle and carries blue blood back to the heart to receive oxygen. When this defect is present the Pulmonary hallway originates from the Left Ventricle and carries blood full of oxygen back to the lungs. This is a waste of time and resources because that blood does not need to be getting more oxygen.


A baby born with this defect would be born deeply blue and needs immediate attention because his body is receiving mainly blue blood, or not-oxygenated blood.

Temporary Fix:

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To temporarily fix the problem doctors would probably start your baby on a medicine called Prostaglandin which opens communication between the two hallways called Ductus Arteriosis.

This forces the blood in the two hallways to mix so some of the blue blood in the aortic hallway gets to go to the Pulmonic hallway to get some oxygen and come back so the baby will be less blue. Sometimes this is enough.

Frequently that is not enough and your child may have to undergo an immediate Cardiac Casterization procedure which requires creating a hole between the back rooms of the heart (Aterial Septestomy) to force the pink and blue blood to mix.

Permanent Fix:

To permanently fix the problem doctors do an operation to switch the two arteries, called a Switch Operation where the two arteries are "switched" so the heart can function normally.

Total Anomalous Pulmonary Venous Connection

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Problem with the pulmonary vein

This condition happens when all the Pulmonary Veins coming back from the lungs to the Left Atrium do not in fact empty into the Left Atrium. Instead they go through a small chamber which then empties into a single large vein that goes to the right side of the heart.

The fresh pink blood coming back from the lungs mixes with the blue blood in the Superior Venacava. This mixing of blood then goes to the Right Atrium, through a hole to the Left Atrium, then to the Left Ventricle, and on to the Aorta.

However, most of the blood will end up going through the Tricuspid Valve to the Right Ventricle and on to the Pulmonary Artery. The Right Ventricle and Pulmonary Artery will both become larger because they are handling lots of blood. The lungs will be receiving huge amounts of blood and the baby will be very congested and breathing fast

If an obstruction happens to the major vein going to the right side of the heart the baby will be blue. This condition is usually treated with immediate surgery.

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Children's Heart Institute

P. O. Box 10066 McLean, VA 22102

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phone - 703-481-5801

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