Pulmonary Hypertension

Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs
(the pulmonary arteries) is abnormally high.
Many disorders can cause pulmonary hypertension.
People usually have shortness of breath upon exertion and loss of energy, and some
people feel light-headed or fatigued on exertion.
Chest x-rays, electrocardiography, and echocardiography give clues to the diagnosis,
but measurement of blood pressure in the right ventricle and the pulmonary artery is
needed for confirmation.
Treatment of the cause and use of drugs that improve blood flow through the lungs
are helpful.
Blood travels from the right side of the heart through the pulmonary arteries into the lungs.
There, carbon dioxide is removed from the blood and oxygen is added. Normally, the
pressure in the pulmonary arteries is low, allowing the right side of the heart to be less
muscular than the left side (because relatively little muscle and effort are needed to push the
blood through the lungs via the pulmonary arteries). In contrast, the left side of the heart is
more muscular because it has to push blood through the entire body against a much higher
pressure.
If the pressure of the blood in the pulmonary arteries increases to a sufficiently high level,
the condition is called pulmonary hypertension. With pulmonary hypertension, the right side
of the heart must work harder to push the blood through the pulmonary arteries into the
lungs. Over time, the right ventricle becomes thickened and enlarged and heart failure
develops (see see Heart Failure).



Cor Pulmonale: A Disorder Stemming From Pulmonary Hypertension
Cor pulmonale is pulmonary hypertension related to the underlying lung
disorder. The right ventricle of the heart becomes enlarged and thickened,
eventually resulting in heart failure.
Cor pulmonale develops because the pulmonary arteries constrict and become
thickened in response to low oxygen levels. This thickening narrows the
passageway through which blood flows through the lung, and this narrowing, in
turn, increases the pressure in the pulmonary arteries. Once pulmonary
hypertension develops, the right side of the heart has to work harder to
compensate, but the increased effort causes it to become enlarged and thickened.
These changes can lead to right-sided heart failure.
The failing right ventricle places a person at risk of pulmonary embolism

because blood flow is abnormally low, so blood tends to pool in the legs. If clots
form in the pooled blood, they may eventually travel to and lodge in the lungs,
with dangerous consequences.
There may be few symptoms of cor pulmonale until the disorder is quite
advanced. When symptoms do occur, people describe shortness of breath during
exertion, light-headedness, fatigue, and chest pain. Symptoms of heart failure,
such as swelling (edema) in the legs and progressively worse shortness of breath,
also develop.
A number of tests are available to help doctors diagnose cor pulmonale, but the
diagnosis is often suspected on the basis of the physical examination. By
listening through a stethoscope, doctors can hear certain characteristic heart
sounds that occur when the right ventricle becomes strained. Chest x-rays can
show the enlarged right ventricle and pulmonary arteries. Doctors evaluate the
function of the left and right ventricles with echocardiography, radionuclide
studies, and cardiac catheterization.
Treatment is usually directed at the underlying lung disorder. Measures to relieve
right-sided heart failure are also taken. Because people with cor pulmonale are at
increased risk of pulmonary embolism, doctors may prescribe an anticoagulant to
be taken long-term.
Causes
There are many causes of pulmonary hypertension, including HIV infection, drugs and
toxins, lung disorders, and low blood oxygen levels (hypoxia). One of the most common
causes of pulmonary hypertension is left-sided heart failure, which can occur if
One of the heart valves does not work properly
The left ventricle is stressed by high blood pressure
A heart attack or some other disorder involving the heart diminishes the ability of
heart muscle to pump
Lung disorders can also lead to pulmonary hypertension. One of the most common
conditions is chronic obstructive pulmonary disease (COPDsee see Chronic Obstructive
Pulmonary Disease (COPD)). When the lungs are impaired by a disorder, more effort is
needed to pump blood through them. Over time, COPD destroys the small air sacs (alveoli)
together with their small vessels (capillaries) in the lungs. The single most important cause
of pulmonary hypertension in COPD is the narrowing (constriction) of the pulmonary
arteries that occurs as a result of low blood oxygen levels. Having sleep apnea and living in
or prolonged visiting in places that are at high altitudes can also cause pulmonary
hypertension by lowering levels of oxygen in the blood. Other lung disorders that may cause
pulmonary hypertension include pulmonary fibrosis, cystic fibrosis, sarcoidosis, and
Langerhans' cell granulomatosis (histiocytosis).
Less often, pulmonary hypertension is caused by extensive loss of lung tissue from surgery
or trauma. Other causes include heart failure, scleroderma, obesity with reduced ability to
breathe (pickwickian syndrome), neurologic diseases involving the respiratory muscles,
chronic liver disease, and HIV infection. Pulmonary hypertension also occurred in some
people who took the diet drugs dexfenfluramine and phentermineSome Trade Names
ADIPEX-P
(fen-phen) during the 1990s. A cause of sudden pulmonary hypertension is pulmonary
embolism, a condition in which blood clots become lodged in the arteries of the lung (see
see Pulmonary Embolism (PE)). In the tropics, schistosomiasis, a parasite disorder, is a
common cause.
A small group of people have pulmonary hypertension without any identifiable cause
(called idiopathic pulmonary hypertension). Women are affected by idiopathic pulmonary
hypertension twice as often as men, and the average age at which the diagnosis is made is
about 35 years.
Symptoms
Shortness of breath upon exertion is the most common symptom of pulmonary
hypertension, and virtually everyone who has the condition develops it. Some people feel
light-headed or fatigued on exertion. The person is likely to feel weak because body tissues
are not receiving enough oxygen. Other symptoms, such as coughing and wheezing, are
usually caused by the underlying lung disorder. Swelling (edema), particularly of the legs,
may occur because fluid may leak out of the blood vessels and into the tissues. Swelling is
usually a sign that right-sided heart failure has developed.
Some people with pulmonary hypertension have connective tissue disorders, especially
systemic sclerosis (sclerodermasee see Systemic Sclerosis).
Diagnosis
Based on the symptoms, doctors may suspect pulmonary hypertension in people who have
an underlying lung disorder. A chest x-ray may show that the pulmonary arteries are
enlarged. Electrocardiography (ECG) and echocardiography enable doctors to look for
certain problems with the right side of the heart before cor pulmonale develops. For
example, thickening of the right ventricle or a partial reversal (back flow) of blood through
the tricuspid valve between the right atrium and right ventricle may be detected on an
echocardiogram. Pulmonary function tests help doctors assess the extent of lung damage. A
sample of blood may be taken from an artery in an arm to measure the level of oxygen in
the blood.
A definite diagnosis of pulmonary hypertension usually requires passing a tube through a
vein in an arm or a leg into the right side of the heart to measure the blood pressure in the
right ventricle and the pulmonary artery.
Treatment
Treatment of pulmonary hypertension is best directed at the cause when the cause has been
identified. Vasodilators (drugs to dilate blood vessels), such as calcium channel blockers
and prostacyclin analogs, are often helpful for pulmonary hypertension that occurs in people
with scleroderma, chronic liver disease, and HIV infection. In contrast, these drugs have not
proved effective for people with pulmonary hypertension due to an underlying lung
disorder. For most people with idiopathic pulmonary hypertension, vasodilators, such as
prostacyclin, drastically reduce blood pressure in the pulmonary arteries. Prostacyclin given
intravenously through a catheter that is surgically implanted in the skin improves the quality
of life, increases survival, and prolongs the time until lung transplantation needs to be
considered. Before administering vasodilators, however, doctors usually first test the
effectiveness of these drugs while the person is in a cardiac catheterization laboratory,
because their use may be dangerous in some people. Subcutaneous (under the skin) and
inhaled forms of prostacyclin are now available and are effective in some people.
Endothelin (a substance in the blood that causes constriction of the vessels) receptor
blockers, bosentanSome Trade Names
TRACLEER
and ambrisentanSome Trade Names
LETAIRIS
, given by mouth, have been effective in some people with mild disease. A drug similar to
prostacyclin, called iloprostSome Trade Names
VENTAVIS
, can be administered by inhalation and, as a result, has a much lower risk of complications
than prostacyclin. Oral sildenafilSome Trade Names
VIAGRA
is very effective in some people with pulmonary arterial hypertension.
In people with pulmonary hypertension who have a low level of oxygen in the blood, the
continuous use of oxygen through nasal prongs or an oxygen mask may reduce blood
pressure in the pulmonary arteries and may relieve shortness of breath. A diuretic drug is
usually given to assist the right ventricle in maintaining a normal volume for effective
beating and to reduce leg swelling. An anticoagulant may also be given to reduce the risk of
blood clots and subsequent pulmonary embolism (see see Pulmonary Embolism).
Lung transplantation is an established procedure for treating people with pulmonary
hypertension. Lung transplantation can be used only in people with severe disease who are
healthy enough to withstand the potential consequences and difficulties with the procedure.
Last full review/revision March 2008 by John H. Newman, MD