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Key Points
- Tetralogy of Fallot is a rare, complex
congenital
heart defect (a problem with the heart's structure that's present at
birth).
- Tetralogy of Fallot involves four defects in the
heart:
- A large ventricular septal defect (VSD)
- Pulmonary stenosis
- Right ventricular hypertrophy
- An overriding aorta
- Together, these four defects mean that not enough
blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows out
to the body.
- Doctors don't know what causes most cases of
tetralogy of Fallot. Certain factors during pregnancy, heredity, and some
genetic disorders may increase the risk of having a baby with tetralogy of
Fallot.
- Cyanosis, or a bluish tint to the skin, lips, and
fingernails, is an important symptom of tetralogy of Fallot. Other symptoms
include a
heart
murmur, slower-than-normal growth and development, and clubbing (a rounded
shape to the skin or bone around the fingernails).
- An
echocardiogram
is an important test for diagnosing tetralogy of Fallot and for following the
problem over time. This test helps diagnose problems with how the heart is
formed and how well it's working. Other tests used to diagnose this defect
include
EKG
(electrocardiogram), chest x ray, pulse oximetry, and
cardiac
catheterization.
- Tetralogy of Fallot must be repaired with
open-heart surgery, either soon after birth or later in infancy. Some babies
who are very small or weak have a temporary procedure that improves blood flow
to the lungs and gives the baby time to grow and get strong enough for the full
repair.
- Treatment involves widening or replacing the
pulmonary valve and enlarging the passageway from the right ventricle to the
pulmonary arteries. These procedures improve the flow of blood to the lungs,
allowing it to pick up enough oxygen for the body's needs. Surgeons also close
the VSD with a patch. The patch stops the mixing of oxygen-poor and oxygen-rich
blood in the ventricles. Fixing these two defects resolves problems caused by
the other two defects.
- Advances in treatment mean that most children
born with tetralogy of Fallot grow to adulthood. They need lifelong care by
trained specialists to stay as healthy as possible.
- Adults who had surgery to repair tetralogy of
Fallot may have long-term heart problems, such as heart function problems,
arrhythmias,
or problems resulting from the original repair. These problems are treated with
medicines, procedures, and surgery.
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