Juliann is scheduled for surgery on Wednesday, June 15th 2011 at UCLA. I have copied some information from the Mayo Clinic about her condition and procedure.
Definition
Ebstein's anomaly is a rare heart defect that's present at birth (congenital). In Ebstein's anomaly, your tricuspid valve — the valve between the chambers on the right side of your heart — doesn't work properly. Blood leaks back through the valve, making your heart work less efficiently. Ebstein's anomaly may also lead to enlargement of the heart or heart failure.
Treatment options include medications and surgery.
Mild forms of Ebstein's anomaly may not cause symptoms until later in adulthood. If signs and symptoms are present, they may include:
- Shortness of breath
- Fatigue, especially with exertion
- Leg swelling
- Heart palpitations or abnormal heart rhythms (arrhythmias)
- A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)
What happens in Ebstein's anomaly
In Ebstein's anomaly, the tricuspid valve is farther down into the right ventricle than it would be in a normal heart. This makes it so that a portion of the right ventricle becomes part of the right atrium (becomes atrialized), causing the right atrium to be larger than usual. Because of this, the right ventricle can't work properly.
In Ebstein's anomaly, the tricuspid valve is farther down into the right ventricle than it would be in a normal heart. This makes it so that a portion of the right ventricle becomes part of the right atrium (becomes atrialized), causing the right atrium to be larger than usual. Because of this, the right ventricle can't work properly.
In addition to the problems with the placement of the tricuspid valve, the valve's leaflets are abnormally formed. This can lead to blood leaking backward (regurgitating) into the right atrium. In severe cases, the leaflets may be tethered to the wall of the heart, leading to severe leakage of blood into the atrium.
Where the valve is placed and how poorly it's formed varies from person to person. In some people, the valve is only mildly abnormal. In others, the valve may be extremely displaced, and it may leak severely. The more the 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, leading to heart failure.
Abnormal heartbeats (arrhythmias). Some people with Ebstein's anomaly have an abnormal heart rhythm (arrhythmia) characterized by rapid heartbeats (tachycardia). These types of arrhythmias (tachyarrhythmias) can make your heart work less effectively, especially when the tricuspid valve is leaking severely. In some cases, a very fast heart rhythm may cause fainting spells (syncope).
The main goals of her surgery (as I understand it) is to repair her severely leaking tricuspid valve by removing the tethered chords on the leaflets, then reshaping the leaflets of her valve by taking from the larger one and adding to the smaller ones at a higher - more normal place. Also, since her right atrium has enlarged to 212 ml (should be around 40 ml) the surgeon is going to reduce this to bring it down to a more normal size (making her heart more efficient). A sleeve and collar will help keep the valve and right atrium at this new size for her. If this surgery goes as planned, she will not need an artificial valve or a pacemaker.
We appreciate all support and prayers from everyone to help make this happen.
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