Primary Pulmonary Hypertension
Pressure helps blood flow through blood vessels.
Hypertension occurs when this pressure gets too high.
When this happens in the blood vessels in the lungs it
is called pulmonary hypertension. Normal
pressure for blood flow through the lungs is one-sixth
of the pressure for the rest of the body. Pressure may
rise for any number of reasons. It creates a potentially
serious condition. The right side of the heart needs to
pump harder to move blood against the pressure. If left
untreated the right side of the heart can eventually
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The blood vessels in the lungs are very sensitive to
oxygen. The lower the level of oxygen, the narrower the
vessels become. Higher pressure will be needed to push
blood through the narrow blood vessels. Pressure will
also increase when there is more blood in the lungs than
These situations may be caused by:
Chronic obstructive pulmonary disease (COPD) is
the most common cause. This condition causes
destruction of lung tissue. This decreases the
number of blood vessels. It also lowers the
amount of oxygen available.
COPD is a combination of chronic
bronchitis and emphysema
Usually due to years of tobacco
Heart abnormalities can result in extra blood in
the lungs. Some are conditions people are born
with. Others may develop over time. Some of
these conditions are:
Septal defects (holes between
the right and left sides of the
heart, present at birth)
Tight (stenotic) or leaky heart
Some lung diseases scar the lungs. This makes
them less flexible.
Scleroderma (systemic sclerosis)
— stiffen blood vessels,
effectively preventing them from
opening which raises the
Patients who have large sections
of lung removed surgically
Systemic lupus erythematous
In obstructive sleep apnea breathing stops at
times through the night. This lowers available
oxygen. As a result, pulmonary blood pressure
will also increase.
Muscle weakness can make it difficult to
breathe. This will lower the available oxygen.
This weakness is common in neuromuscular
disorders such as:
Mountain climbers all develop the condition. It
is the natural result of breathing thin air.
This is one cause of high altitude sickness.
Pulmonary embolism is a build up of blood clots
in the lungs. These clots plug up the blood
A type of chest wall deformity is known as
pectus excavatum. Rarely, severe scarring of the
chest wall, can prevent chest expansion. It has
the same effect as scarring of the lungs
Idiopathic pulmonary arterial hypertension is
due to defects in the arteries of the lungs.
There is no known cause. It is likely due to
Other factors and conditions that can create pulmonary
Exposure to certain substances, such as cocaine
HIV positive status
The following factors increase your chance of developing
Asthma or other chronic lung disease
Recurring pulmonary emboli
Obstructive sleep apnea
Low thyroid (myxedema)
Certain congenital and valvular heart conditions
Muscle weakness diseases
Home at high altitude (over 10,000 feet)
Pectus excavatum or other severe chest deformity
If you have any of these, see your doctor. They suggest
a condition that may need medical attention.
Progressive shortness of breath
Ankle swelling from fluid retention
Heart pain ( angina )
Your doctor will ask about your symptoms and medical
history. A physical exam will be done. You may be
referred to a specialist in heart or lung diseases. A
cardiologist focuses on the heart. A pulmonologist
focuses on the lungs.
Tests may include the following:
Blood gas determination
Pulmonary function test
Computerized axial tomography (CAT) scan
Most cases of pulmonary hypertension are due to another
disease. In this case the underlying cause is treated.
For some, no treatable cause is found. In this case,
there are several medications that might be of benefit.
Talk with your doctor about the best plan for you.
Treatment options include the following:
Calcium channel blockers
Phosphodiesterase inhibitors (sildenafil)
Prostacyclin (epoprostenol, iloprost) — These
must be administered by continuous infusion
either intravenously, subcutaneously, or by
Endothelin receptor blockers (bosentan)
This is a drastic treatment. It is only recommended for
To help reduce your chances of getting pulmonary
hypertension, take the following steps:
Do not smoke
Control asthma effectively
Maintain proper weight
Treat conditions that can lead to pulmonary
American Heart Association http://www.americanheart.org/
National Library of Medicine