Pulmonary Hypertension HF#6719

 

What is pulmonary hypertension?

 

This is high blood pressure inside the lungs.  The walls of the blood vessels that supply the lungs get thicker and often constrict or narrow.  This shrinks or blocks off the space inside them.  The vessels cannot carry as much blood.  For instance, when you attach a nozzle to a garden hose instead of water just flowing out of the hose, it squirts out with such force that it can cause damage to plants nearby.  As you tighten the nozzle, you might notice the hose itself getting stiffer because of back pressure.  The same thing happens when blood has trouble getting through your lungs.  The pressure backs up all the way to the heart.  The heart pumps harder and harder to try and push the blood through.  In time, your heart just can’t keep up.  There is less blood going through your lungs to pick up oxygen.  You become tired, dizzy, and short of breath. 

 

The lungs and blood flow

 

Blood vessels in our lungs make chemicals that are needed to make blood vessels widen or narrow.  A second job is to make, repair, or destroy cells in the vessel walls.  What’s harming you in pulmonary hypertension is too much or too little of some of these compounds. 

 

Causes

 

Sometimes the cause is unknown, but there are some known causes.

• Pulmonary arterial hypertension means high blood pressure in the arteries of the lungs.  The right side of your heart pumps the blood into the arteries of your lungs.  With this type, the right side of your heart has to work too hard to force blood through the lung vessels.  The right side of the heart is not made to work so hard. 
• Pulmonary venous hypertension results in increased pressure in the veins of the lungs.  A disease of the left side of the heart may cause increased pressure.  Mitral valve disease or a damaged left ventricle may cause increased pressure in the veins, which leads to fluid build up in the lungs and high blood pressure.

• Pulmonary hypertension from hypoxemia (low oxygen levels in the body) causes the blood vessels in the lung to become blocked.  This affects the amount blood and oxygen flowing through your body.  Emphysema, sleep apnea, or being exposed to high altitudes for a long time are a few causes.
• Pulmonary hypertension due to chronic thrombotic and/or embolic disease is caused by blood clots or a blockage in the lungs.  When blood clots in the lung form, blood flow is blocked and pressure builds up behind the blockage.

• Other causes may be due to an illness where there is a direct affect on the blood vessels in your lung such as in pulmonary capillary disease or an inflammatory disease like sarcoidosis. 

 

Symptoms

 

Because the symptoms are the same as those from many other diseases, it is often found by looking for and ruling out those other diseases.

• Trouble breathing
• Chest pain
• Feeling dizzy
• Fainting
• Chronic fatigue
• Swollen ankles and legs
• Being depressed
• Dry cough
• Raynaud’s syndrome

 

If pressures inside the lungs increase, then symptoms get worse.  Increased fatigue, increased ankle swelling, and fluid around the lungs and the abdomen are present with severe pulmonary hypertension. 

 

Treatment

 

This is a long-term disease.  Although it cannot be cured, it can be managed.  Discuss the treatment plans with your doctor:

• Manage the cause.  If you have a disease that caused the hypertension, that disease must be treated also.
• Take medicines.  Today, there are many medicines to help manage most people with pulmonary hypertension.  You must know your medicines and how they should be taken.
• Use oxygen.  Low levels of oxygen in your blood will make the problem worse and increase the heart’s workload.  Some patient’s need to wear oxygen in order to increase these levels. 
• Surgery can help correct congenital heart problems (birth defects), improve blood flow when old, scarred blood clots are removed, and repair or replace damaged heart valves.  Lung or heart/lung transplant may be an option if other treatments have failed. 

 

Medicines

 

Not every person with Pulmonary Hypertension needs each of the medicines listed below.  Since each person is different, their prescribed medicine plan may be different, too.

 

Blood thinners

Types:  Coumadin^® (warfarin), aspirin

Function:  These pills make blood less likely to clot or form a blockage.

 

Calcium channel blockers

Types:  Nifedipine (Procardia^®/Adalat^®),  Amlodipine Besylate (Norvasc^®), Diltiazem Hydrochloride (Cardizem^®, Cartia XT^®)

Function:  These work to decrease the pressure inside the lungs of some people with Pulmonary Arterial Hypertension. 

 

Lanoxin, Lanoxicaps (Digoxin^®)

Function:  This helps the heart pump blood more effectively.

 

Diuretics

Types:  Lasix (Furosemide^®), Bumex (Bumetanide^®), Demadex (Torsemide^®), Aldactone (Spironolactone^®), or metolazone

Function:  These work to reduce body fluid. 

 

Endothelin Receptor Antagonist

Types:  Bosentan (Tracleer^®), Ambrisentan (Letairis^®)

Function:  This works to reduce the high blood pressure in the lungs and allow the heart to pump blood better. 

 

Phosphodiesterase Inhibitors

Types:  Sildenafil (Viagra^® or Revatio^®), Tadalafil (Adcirca^®)

Function:  This works to reduce the high blood pressure in the lungs and allow the heart to pump blood better. 

 

Prostacyclin Analogue

Types:  Epoprostenol (Flolan^®), Treprostinil (Remodulin®, Tyvaso^®), Ventavis (Iloprost^®)

Function:  These drugs help open up blocked lung blood vessels and reduce blood pressure in the lungs.  Flolan^® is given by a continuous intravenous (IV) infusion.  Remodulin^® is given through a constant subcutaneous (under the skin) or IV infusion.  Iloprost^® is inhaled (like a breathing treatment) every 2-3 hours and Tyvaso® is inhaled 4 times daily.

 

There is new research being done all the time and new treatments are being found. 

 

 

The information provided should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Any duplication or distribution of the information contained herein is strictly prohibited.

Last Updated: 03/30/2010

Copyright © 03/30/2010 University of Wisconsin Hospitals and Clinics Authority. All rights reserved. Produced by the Department of Nursing. HF#6719

Print Health Fact For You