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Treatment Guide
Pulmonary
Hypertension
Pulmonary hypertension is a frightening diagnosis,
one that can literally take your breath away. Everyday
activities such as climbing steps can leave you short
of breath, fatigued, or even provoke chest pain. The
good news is that lifestyle changes and medical
treatment can greatly improve your symptoms and
your quality of life.
CHOOSING YOUR CARE
Thanks to effective treatment options, many
patients with pulmonary hypertension are able to
lead productive and satisfying lives.
At Cleveland Clinic’s Respiratory Institute, we have
been providing expert care to patients with all types of
pulmonary hypertension for more than 20 years. Our
Pulmonary Hypertension Program is one of the largest
and most comprehensive treatment programs in the
country, offering the best treatment options available
and the benefits of cutting-edge research and clinical
trials. Our team of doctors and nurses collaborates
closely with our specialists in cardiology and other
related departments to ensure that you receive the
most complete and timely care.
Cleveland Clinic’s Respiratory Institute has
a long history of excellence and innovation,
and is ranked the #3 pulmonology program
in the nation by U.S. News & World Report.
Cleveland Clinic also is consistently ranked
among America’s Top 4 hospitals.
Using this guide
Please use this guide as a resource to
learn about the causes of pulmonary
hypertension and your treatment options.
As a patient, you have the right to ask
questions and seek a second opinion.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
respiratory institute | PULMONARY HYPERTENSION treatment guide
What is pulmonary hypertension?
Pulmonary hypertension is a rare lung disorder affecting your pulmonary arteries,
which are the arteries that carry blood from your heart to your lungs to pick up
oxygen. In patients with pulmonary hypertension, these arteries are narrowed,
making it harder for blood to flow through the vessels and resulting in abnormally
high pressure. This high pulmonary pressure puts a strain on the right ventricle of
the heart, which causes it to enlarge and gradually lose its ability to pump enough
blood to your lungs. Eventually, patients can develop right-sided heart failure.
Why do the pulmonary arteries
narrow?
There is no definitive answer for why the pulmonary arteries narrow. Research
shows that it likely begins with an injury to the layer of cells lining the small
blood vessels in the lungs. This injury appears to change the way the cells
interact with the smooth muscle cells in the vessel wall. Both cells proliferate
and the smooth muscle contracts more than it should, which narrows the vessel. What causes the injury itself is not known.
What are the causes?
Some of the known causes of pulmonary hypertension are:
rheumatic disorders, Liver diseases, other lung conditions,
and heart disease
Pulmonary hypertension is often a secondary disease, meaning it is caused by
another underlying condition. Among the diseases that can result in pulmonary
hypertension are rheumatic disorders such as scleroderma and lupus; chronic
liver disease and cirrhosis; lung conditions such as tumors, emphysema, chronic
obstructive pulmonary disease (COPD), and pulmonary fibrosis; and heart diseases such as left heart failure, aortic valve disease, mitral valve disease and
congenital heart disease.
Thromboembolic disease
A blood clot in a large pulmonary artery can result in the development of pulmonary hypertension.
Low oxygen conditions
High altitude living, obesity and sleep apnea can lead to pulmonary hypertension.
Genetics
Pulmonary hypertension is inherited in a small number of cases. If you have a
close family member with pulmonary hypertension, see a doctor for early evaluation if you experience any symptoms.
Diet drugs
Those who use or have taken fen-phen (fenfluramine and phentermine) in the past
are 23 times more likely to develop pulmonary hypertension, sometimes even
years later.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
respiratory institute | PULMONARY HYPERTENSION treatment guide
Other conditions also may cause pulmonary hypertension, and in some cases, the
cause is not known.
What are the symptoms?
The first sign of pulmonary hypertension is usually shortness of breath during
everyday activities that normally do not require much exertion, such as climbing
steps or light housework. As the disease progresses, you may feel overly tired,
dizzy or faint, or notice swelling in your ankles, abdomen or legs, or a bluish tone
to your lips or skin. As strain increases on the heart, you may experience chest
pains.
Types of Pulmonary
Hypertension (PH)
The term “pulmonary hypertension”
encompasses a group of diseases
characterized by high pulmonary
artery pressures (high blood pressure in the vessels in the lungs).
Types of PH
Unfortunately, symptoms usually do not appear until the condition has progressed.
They can range in severity, and not all patients show the same symptoms.
Pulmonary arterial hypertension
(PAH) – Originates at the pulmonary
arteries. This can be idiopathic (of
no known cause), heritable (runs in
families or has a genetic cause), or
associated with an underlying disease or condition.
How is pulmonary
hypertension diagnosed?
Pulmonary venous hypertension
(PVH) – A result of left heart disease like heart failure or heart valve
disease
Over time, additional symptoms may include an irregular heartbeat, a racing
heart, shortness of breath that gets progressively worse during activity, and trouble
breathing at rest. As the condition progresses, symptoms can occur with minimal
activity, and eventually, any activity may become too difficult.
To make the correct diagnosis, your physician will take your complete medical
history and perform a thorough physical examination. Your doctor also will ask
you to describe your symptoms and will look for signs of other conditions that
can cause pulmonary hypertension.
During an exam, your doctor will listen to your heart and lungs, examine your jugular vein, check your legs, ankles and abdomen for swelling, and look for telltale
signs of low blood oxygen levels, such as a bluish tint in your nail bed.
Your healthcare provider also is likely to order tests, such as:
Blood tests – These tests check numerous factors, such as how well your liver and
kidneys are working; vascular or thyroid problems; blood oxygen levels; anemia;
elevated hemoglobin; HIV; and heart disease.
Echocardiogram – Using sound wave technology, your doctor will be able to see
how the right ventricle is functioning and measure how well blood is flowing
through the heart valves. This test allows your physician to estimate the level
of pressure in your pulmonary arteries.
PH due to low oxygen levels associated with underlying lung disease
like emphysema or interstitial lung
disease
Chronic thromboembolic pulmonary
hypertension (CTEPH) – Caused by
unresolved clots in the pulmonary
arteries
PH also can be the result of a variety of other less common conditions.
Chest X-ray – Shows whether the right ventricle and pulmonary arteries are
enlarged.
Walk test – This examines your exercise tolerance and how much oxygen is in
your blood during exertion.
Pulmonary function tests – These screen for other underlying lung conditions.
Polysomnogram or overnight oximetry – Tests for sleep apnea, which causes low
oxygen levels while sleeping.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
respiratory institute | PULMONARY HYPERTENSION treatment guide
Right heart catheterization – This procedure measures heart pressures and the
rate at which your heart pumps blood. It also can find any leaks between the two
sides of the heart.
Ventilation perfusion scan (V/Q scan) – Scans for evidence of blood clots along
the pathway to the lungs.
Chest CT scan – A CT scan can find blood clots and other lung conditions that
may cause or worsen pulmonary hypertension.
Pulmonary angiogram – Looks for blood clot blockages in the pulmonary arteries.
How is pulmonary
hypertension treated?
Many effective treatment options are available to improve your symptoms and
improve your quality of life. When deciding on a treatment plan, your doctor will
consider the severity of your symptoms, the progression of the disease, and
what medications are suitable for your type of PH. In general, treatment
includes lifestyle and dietary changes, and medication. As part of the treatment
plan, you will need to see your doctor regularly. Surgery may be needed for those
who have clots in the pulmonary arteries, and lung transplantation is reserved for
patients whose advanced disease does not respond to medical therapy.
Oxygen therapy
Not all patients with PH require oxygen therapy. But in individuals with low oxygen levels, this can be an important part of their treatment.
Blood thinners
Anticoagulants decrease blood clot formation so that blood can flow more easily
through blood vessels. Warfarin sodium (Coumadin®) is a commonly prescribed
anticoagulant for pulmonary hypertension.
Diuretics
“Water pills” can reduce swelling and ease breathing by helping to eliminate
extra fluid in your tissues and bloodstream. Your doctor also may direct you to
take potassium to replace nutrients lost from increased urination.
Medications
Medications used to treat pulmonary hypertension typically require close monitoring and routine follow-up by your physician. To be most effective and to minimize
risks and side effects, be sure to follow the instructions of your healthcare providers carefully.
Prostacyclin-like medications
These vasodilators lower pulmonary vessel pressure and may improve the pumping ability of the right side of the heart. Drugs such as epoprostenol (Flolan®,
Veletri®), treprostinil sodium (Remodulin® and Tyvaso®) and iloprost (Ventavis®)
dilate pulmonary arteries.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
respiratory institute | PULMONARY HYPERTENSION treatment guide
Endothelin receptor antagonists
The medications bosentan (Tracleer®) and ambrisentan (Letairis®) help block
the action of endothelin, which constricts blood vessels and raises blood pressure
in the lungs.
Phosphodiesterase inhibitors
Sildenafil (such as Revatio®) and tadalafil (Adcirca®) relax pulmonary smooth
muscle cells, leading to the dilation of the pulmonary arteries.
Surgical Options
Pulmonary thromboendarterectomy
Cleveland Clinic is one of few centers in the country with expertise in this complex
procedure, which can remove a large clot or clots in pulmonary arteries.
Benefits – Improved blood flow and lung function; increased survival rate.
This procedure can potentially cure patients with chronic thromboembolic
pulmonary hypertension (CTEPH).
Who is a candidate? Patients with CTEPH, or pulmonary hypertension that
is caused by clots in the pulmonary arteries.
Risks – This procedure requires cardiopulmonary bypass, hypothermia and
cardiac arrest and has a mortality rate of about 5 percent. After surgery,
many patients develop fluid in the lungs, which must be carefully treated in
the ICU. However, most doctors agree that the benefits of the surgery far outweigh the risks.
Lung transplantation
Lung transplantation is a potential treatment for patients with advanced pulmonary hypertension. About 1,000 lung transplants are performed each year in the
U.S. A shortage of donors is a major limiting factor, and patients are added to a
national waiting list.
Benefits – In general, the right side of the heart will return to normal after
the transplant.
Who is a candidate? Transplantation is reserved for advanced pulmonary
hypertension that is not responsive to medical therapy.
Risks – Organ rejection; infection.
Heart/lung transplantation
This type of double organ transplant is very rare; nationally, 27 heart-lung transplants were performed in 2011, according to the U.S. Department of Health and
Human Services.
Benefits – Drastic improvement in lung and cardiac function.
Who is a candidate? Necessary for patients who have combined lung and
heart failure.
Risks – Organ rejection; infection.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
of note
Many effective treatment
options are available to improve
your symptoms and improve
your quality of life.
respiratory institute | PULMONARY HYPERTENSION treatment guide
Living with pulmonary hypertension:
Lifestyle and dietary changes
Making lifestyle and dietary changes are a vital part of managing pulmonary
hypertension. Taking charge of your diet and habits allows you to become an
active participant in managing the condition.
Diet recommendations for patients with PH include:
•Reducing sodium intake
•Monitoring your fluid intake. Cut down on fluid intake if you have become more
short of breath or if you notice swelling.
of note
•Eating foods that are high in vitamins and nutrients, like potassium
•Limiting your daily calories to achieve your ideal body weight, if needed
Clinical Trials/
Research
As a leading medical research
institution, research projects
aimed at understanding and
treating pulmonary hypertension
are ongoing at Cleveland Clinic.
We also participate in several
multicenter clinical trials
evaluating new therapies
for the treatment of
•Limiting foods containing refined sugar, saturated fats and cholesterol
•Eating high-fiber foods, such as whole grains, fruits and vegetables
Lifestyle recommendations include:
•Monitoring your weight. If you notice a gain of two pounds in one day or five
pounds over one week, inform your doctor.
•Losing weight, if you are overweight
•Annual health check-ups with your primary care physician
•Restricting lifting, pushing, or pulling to less than 20 pounds to avoid increasing
the pressure in your arteries and lungs
•Walking regularly. However, more strenuous forms of aerobic exercise should
only be performed after discussing with your physician. Any activities that cause
shortness of breath, dizziness or chest pain should be stopped immediately.
Contact your doctor if any of these symptoms occur.
•Do not smoke or chew tobacco.
•Avoid or reduce alcohol intake.
•Avoid pregnancy.
•Maintain close contact with your healthcare providers.
•Frequent follow-ups with your pulmonary hypertension specialist.
pulmonary hypertension.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
respiratory institute | PULMONARY HYPERTENSION treatment guide
Why should I choose
Cleveland Clinic?
The physicians and nurses at Cleveland Clinic’s Respiratory Institute are among
the best in the country and in the world for providing care to patients with all
forms of pulmonary hypertension.
Our teams are comprised of pulmonary and critical care physicians, advanced
practice nurses, research nurse coordinators and research fellows. These teams
collaborate closely with our world-renowned departments of cardiovascular
medicine, cardiovascular imaging, cardiothoracic surgery and lung transplantation
as well as specialists in hepatology, liver transplantation, sleep medicine and
rheumatology. This means that our patients receive the most comprehensive,
timely care, as well as benefit from cutting-edge research and the best treatment
options available for pulmonary hypertension.
Patient Services
Medical Concierge
Cleveland Clinic’s Medical Concierge program offers special, free concierge
services for our out-of-state patients and their families, acting as a personal
information resource throughout your stay in Cleveland. The service provides:
Making an
Appointment
Call 216.445.5763
or toll-free
800.223.2273,
ext. 55763, to make
an appointment
with a Cleveland
Clinic pulmonary
hypertension specialist.
•Assistance with coordinating multiple appointments
•Scheduling or confirming airline reservations in cooperation with Cleveland
Clinic’s Travel Services
•Assistance with hotel and housing reservations and providing discounts
when available
•Arranging taxi or car service between the airport and hotel
•Providing information about leisure activities for family members
Contact your Medical Concierge at 800.223.2273 ext. 55580 or at
[email protected].
Global Patient Services
Global Patient Services provides high-quality service to more than 3,000 people
from nearly 140 different countries each year. Global Patient Services helps
Cleveland Clinic patients through their medical care. Most of our interpreters
are foreign-born and have medical backgrounds. Contact Global Patient Services
at 216.444.8184.
MyConsult
Need a second opinion but cannot travel to Cleveland? Our MyConsult service
offers secure online second opinions. Patients can enter detailed health information and mail pertinent test results and receive an opinion from our experts that
includes treatment options or alternative recommendations. To learn more about
MyConsult, please visit clevelandclinic.org/myconsult.
Same-day appointments are available. Please call 216.444.2273 or toll-free 1.800.223.2273.
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