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Pulmonary Hypertension
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What is it?
High blood pressure in the arteries that supply the lungs is called pulmonary hypertension (PH)
or pulmonary arterial hypertension (PAH). The blood pressure measured by a cuff on your arm
isn’t directly related to the pressure in your lungs. The blood vessels that supply the lungs
constrict and their walls thicken, so they can’t carry as much blood. As in a kinked garden hose,
pressure builds up and backs up. The heart works harder, trying to force the blood through. If
the pressure is high enough, eventually the heart can’t keep up, and less blood can circulate
through the lungs to pick up oxygen. Patients then become tired, dizzy and short of breath.
If a pre-existing disease triggered the PH, doctors call it secondary pulmonary
hypertension. That’s because it’s secondary to another problem, such as a left heart or lung
disorder. However, congenital heart disease can cause PH that’s similar to PH when the cause
isn’t known, i.e., idiopathic or unexplained pulmonary arterial hypertension. In this case, the
PAH is considered pulmonary arterial hypertension associated with congenital heart disease,
such as associated with a VSD or ASD (either repaired or unrepaired). The problem is due to
scarring in the small arteries in the lung.
It’s important to repair congenital heart problems (when possible) before permanent pulmonary
hypertensive changes develop. Intracardiac left-to-right shunts (such as a ventricular or atrial
septal defect, a hole in the wall between the two ventricles or atria) can cause too much blood
flow through the lungs. This situation is sometimes called Eisenmenger complex. In the past,
the holes can’t be repaired due to increased stress on the heart produced by the high pressures
in the scarred lung blood vessels. This situation is changing with the availability of new
medicines. Heart valve conditions, such as mitral stenosis (a narrowing of the mitral valve), can
also cause secondary PH. Fixing the valve usually reverses the PH.
Cyanosis due to Pulmonary Hypertension with Congenital Heart Disease
When there is a communication between the right and left heart, blood goes from the low
oxygen chamber or vessel to the high oxygen chamber or vessel. An example would be
shunting from the right atrium to the left atrium in patients with atrial septal defect (ASD) and
pulmonary arterial hypertension. The patient who was once acyanotic becomes cyanotic. One
way the body compensates is to increase the ability of the blood to carry oxygen by increasing
the number of red blood cells, a condition called secondary erythrocytosis, sometimes referred
to as polycythemia. It is now recognized that removing blood with a procedure called
© 2010, American Heart Association
phlebotomy is only rarely needed. Symptoms that may prompt a doctor to recommend it are
headaches, excessive fatigue and worsening exercise tolerance. Before this is performed, it is
important that the doctors tests for iron deficiency. Phlebotomy is preferably performed in a
center with experience in adult congenital disease as special precautions are required.
Treating Pulmonary Hypertension
Once pulmonary hypertension has been diagnosed, often more medical therapy is needed.
You’ll require regular follow-up with a cardiologist or pulmonologist trained in caring for patients
with this condition.
As long as the underlying disease exists, it will keep causing PAH. Once you have PAH
(especially if you’ve had it for some time), curing the disease that caused it may not make the
high blood pressure go away. In that case, the PAH will usually need special treatment. While
PH is a serious illness, treatments are available. You may be treated with oxygen, agents to
help your heart pump better, diuretics, anticoagulants (blood thinners) and medications to lower
your PAH and reverse the scarring in the blood vessels in the lung. Sometimes lung transplants
also are done for PAH, but in congenital heart disease, usually heart and lung transplantation is
required.
A diagnosis of PAH doesn’t necessarily mean you can’t have an active, fulfilling life. Still, you
should consider certain measures and precautions. PAH is a lifelong illness that can be made
worse by a variety of factors, such as smoking or traveling to high altitude.
Once PAH has been diagnosed, you must seek advice about physical activity. If you have PAH,
you should be as active as physically possible. Strenuous physical activity; however is
associated with serious increases in pulmonary artery pressure. Therefore, avoid isometric
exercises and activities like heavy lifting, or bench press that can produce dangerous
symptoms, such as chest pain or dizziness. A supervised cardiopulmonary rehabilitation
program may help promote conditioning. Many patients with PAH report having “good and bad
days.” If you need to rest, do so.
Most medications often used to treat coexistent illnesses are safe for people with PAH. Still,
care should be taken with prescription medications and over-the-counter drugs. Any anesthetics
or sedatives can be very hazardous; ask your physician which medications are safe. Discuss
the use of decongestant cold medicines with your doctor.
Pregnancy is not recommended in women with PH. The changes associated with pregnancy
and delivery produce changes that can seriously endanger the life of the mother and baby.
Therefore it is important for women with PH to use a more permanent but safe form of
contraception. Because estrogen can aggravate PH, it’s important to avoid any contraception
containing estrogen. Progesterone forms of contraception are preferable. It is generally
recommended that women with PH have tubal ligation or use the Mirena IUD.
Additional precautions are often taken with PAH patients. These include supplemental oxygen
during air travel, antibiotic therapy for significant respiratory tract infections, pneumococcal
pneumonia vaccine and yearly flu vaccines (since pneumonia can be very serious with PH
patients). Also avoid conditions in which the ambient oxygen concentration may be decreased,
such as high altitude and travel in unpressurized airplane cabins. Before starting an exercise
program, ask your physician what activities are appropriate for you.
© 2010, American Heart Association
Finally, if you’ve begun medical treatment for your PAH, stopping any of your medicines without
your physician’s approval can be extremely dangerous. Medical therapy has significantly
improved the outlook for most patients with pulmonary hypertension, but it doesn’t “cure”
it. Don’t stop medical therapies unless your physician (trained in caring for PAH patients)
recommends doing so.
Preventing Endocarditis
People with PH and certain underlying congenital heart defects may need antibiotics before
certain dental procedures to prevent endocarditis. As the endocarditis recommendations have
changed, please discuss with your cardiologist.
For more information about PH, visit phassociation.org.
© 2010, American Heart Association