Pulmonary Hypertension
What
is it?
Pulmonary hypertension is high blood pressure in
the lungs. This condition damages the blood
vessels in the lungs and reduces the amount of
oxygen the lungs can transfer to the blood. When
there is no apparent cause, it is called primary
pulmonary hypertension. When pulmonary
hypertension is caused by an underlying medical
condition, it is called secondary pulmonary
hypertension.
Who
gets it?
Primary
pulmonary hypertension is unusual in children,
occurring more often in young women. When it does
occur in children, it affects boys and girls
equally. Infants and children with lung or heart
disease are more likely to develop secondary
pulmonary hypertension. Pulmonary hypertension can
also run in families (familial pulmonary
hypertension).
What
causes it?
The
cause of primary pulmonary hypertension is
unknown. Familial pulmonary hypertension is caused
by chromosome abnormalities. Secondary pulmonary
hypertension is a complication of conditions such
as upper airway obstructions that limit the amount
of oxygen reaching body tissues (hypoxia),
congenital heart disease, blood clots, lung
disease such as pneumonia or meconium aspiration
syndrome, liver disorders, and other diseases that
affect the vascular system. The pulmonary artery
carries blood from the right side of the heart to
the lungs, where the blood receives oxygen. When
pulmonary hypertension damages the pulmonary
artery and other blood vessels in the lungs, it is
more difficult for the blood to get through. This
puts stress on the right side of the heart, which
has to work even harder to pump the blood to the
lungs, causing the right side of the heart to
enlarge.
What
are the symptoms?
Low
blood oxygen levels often cause dizziness and
fainting (syncope) as the first symptoms. An older
child may become extremely short of breath (dyspnea)
during exercise or any type of physical activity,
such as climbing stairs or walking. Other symptoms
include fatigue, chest pain, abnormal heart
rhythm, and a bluish tinge to the skin and lips.
Swelling, especially around the ankles, is a sign
of right-side heart failure. Untreated, pulmonary
hypertension can result in death.
How is
it diagnosed?
To
diagnose pulmonary hypertension, a doctor will
study your child’s medical history and perform a
thorough examination. He or she will order a
number of tests to determine the cause of the
child’s condition. Tests can include chest x-ray,
electrocardiogram, echocardiogram, angiography,
pulmonary function tests, and, in some cases,
cardiac catheterization.
What
is the treatment?
The
first priority is to raise the oxygen level in the
blood with oxygen therapy. Children who respond to
pulmonary vasodilators, which are medications that
relax the muscles in the blood vessel walls and
cause the blood vessels to widen, are treated with
calcium channel blockers. Calcium channel blockers
help the heart to pump blood by relaxing the
smooth muscle in the walls of the heart. Patients
who don’t respond to a vasodilator are treated
with prostacyclin, which is another drug that
dilates the blood vessels. The child may also be
given additional medications to treat heart
failure, if present. In secondary pulmonary
hypertension, surgery may be necessary to correct
any known cause. Patients who do not respond to
any treatment may need a lung transplant.
Self-care tips
There
is currently no known way to prevent pulmonary
hypertension. Because survival rates increase with
early treatment, it is important to see a doctor
if your child shows signs of pulmonary
hypertension.
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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