Tetralogy of Fallot
What is it?
Tetralogy of Fallot (TOF) is a birth defect
characterized by four conditions: a hole between the two
lower heart chambers (ventricular septal defect),
narrowing under or at the pulmonary valve (infundibular
stenosis), enlarged right ventricle, and a misaligned
aorta.
Who gets it?
TOF is the most common syndrome of congenital heart
defects, accounting for 10% of all cases.
What causes it?
The problem starts very early in the uterus with a
narrowed pulmonary valve and a hole between the
ventricles, however the cause of TOF is unknown.
What are the symptoms?
Infants with TOF often look blue at birth (cyanosis)
and have detectable heart murmurs. Growth and development
can be delayed, and children may easily become fatigued
and short of breath. Hypoxemic spells, also called
cyanotic spells or tet spells are also a common symptom of
TOF. All of these symptoms occur because not enough blood
is flowing to the lungs to supply the body with needed
oxygen.
How is it diagnosed?
In infants where TOF is suspected, a complete
evaluation of the circulatory system is required,
including testing the blood for its oxygen content,
ultrasound and x-rays of the heart accompanied by a
contrast agent to determine the amount of blood flowing in
the wrong direction. A search for other birth defects is
also necessary, because they tend to happen together.
What is the treatment?
To correct TOF, surgery is done to close the hole
between the two lower heart chambers and to relieve the
narrowing at the pulmonary valve. Surgery must be
carefully timed with attention to the progression of the
disease process, the size of the infant, and the size of
the various defects. There are temporary surgical
procedures that can prolong the time before corrective
surgery while the baby grows larger and stronger.
Self-care tips
After corrective surgery is done, most children are
able to do all normal things, though some may have
exercise restrictions and may need to take medications to
control heart rate and to improve heart function. They
will also need to take antibiotics before dental and other
procedures to prevent heart infections. Because
complications can occur, children with TOF should be seen
by a cardiologist regularly for the rest of their lives.
This information has been designed as a comprehensive and quick reference
guide written by our health care reviewers. The health information written
by our authors is intended to be a supplement to the care provided by your
physician. It is not intended nor implied to be a substitute for
professional medical advice.
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