1. An overview for patients and caregivers
Understanding PAH Testing
An overview for patients and caregivers
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

2. Disclaimer
This presentation is sponsored by and being made on behalf of United Therapeutics Corporation (UTC)
The presenter of this program is compensated by UTC
This information is provided for educational purposes and is not intended as treatment advice
Patients should talk with their healthcare provider to determine an appropriate care and treatment plan
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

3. What We Will Cover In this presentation, we will review
The effects of PAH
Why we test
Several common tests
Pulmonary function
Echocardiogram
B-type natriuretic peptide
6-minute walk
Right heart catheterization
How to make the most of your appointment
Helpful resources
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

4. Heart and Lungs With PAH
The Effects of PAH
Normal Heart and Lungs
Blood vessels in your lungs harden and narrow
Less blood is able to flow from your heart to your lungs
Increased resistance in your heart and pulmonary arteries makes your heart work harder
Your heart is strained and the right side becomes enlarged
Heart and Lungs With PAH
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

5. Why We Test To diagnose PAH
To learn how PAH is affecting your heart and lungs
Establish a baseline
Monitor changes in your condition
To develop a plan for your treatment and care
To evaluate your treatment plan and decide when adjustments may be needed
PAH is a progressive disease.
Even with treatment, it can change over time.
Your doctor will repeat tests to monitor your condition.
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

6. Pulmonary Function Tests
What do they measure?
How much air your lungs are able to hold
How much air moves in and out of your lungs
Your lungs’ ability to exchange oxygen and carbon dioxide
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

7. Pulmonary Function Tests
What should I expect?
To be asked to inhale and exhale, sometimes normally and sometimes forcefully
To wear a mouthpiece attached to a machine called a spirometer or flow meter
To possibly experience some temporary shortness of breath or lightheadedness
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

8. Pulmonary Function Tests
Why are they important?
May indicate issues with the structure or function of your heart or lungs
May hint at other issues contributing to your symptoms
Help determine if there are other issues inside your lungs
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

9. Echocardiogram What does it measure?
How well your heart squeezes and relaxes
The pressures on the right side of your heart
The size and shape of your heart
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

10. Echocardiogram What should I expect?
To undress from the waist up and have electrodes attached and gel applied to your chest
To lie on your side while a sonographer moves a device called a transducer over your chest
A noninvasive procedure with no known side effects
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

11. Echocardiogram Why is it important? Helps monitor your condition
Shows how your heart is responding to treatment
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

12. B-Type Natriuretic Peptide (BNP)
What does it measure?
The amount of BNP hormone in your blood
BNP is a substance produced by the lower chambers of your heart when they are under stress
Normally, only a small amount of BNP is found in your blood
If your heart has to work harder than usual, it releases more BNP
May also be referred to as N-terminal pro b-type natriuretic peptide (NT-proBNP)
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

13. B-Type Natriuretic Peptide (BNP)
What should I expect?
A small amount of blood will be drawn
To possibly experience a prick or stinging sensation
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

14. B-Type Natriuretic Peptide (BNP)
Why is it important?
Gives clues about how hard your heart is working
Can give information about how your body is responding to treatment
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

15. 6-Minute Walk Test What does it measure?
The distance you can walk on a flat, hard surface in 6 minutes
The shortness of breath and fatigue you experience with activity
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

16. 6-Minute Walk Test What should I expect?
To walk laps in a hallway, going as far as you can in 6 minutes
To walk continuously if possible, only slowing down or stopping to rest if needed
To possibly experience some temporary shortness of breath or fatigue
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

17. 6-Minute Walk Test Why is it important?
Gives information about your oxygen needs
Demonstrates what you are physically able to do
Can indicate changes in your PAH
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

18. Right Heart Catheterization
What does it measure?
The pressures in your heart and pulmonary arteries
How much blood your heart pumps out to your lungs
The resistance and flow in your blood vessels
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

19. Right Heart Catheterization
What should I expect?
To lie on a hard table covered in sterile drapes
A thin, flexible catheter will be inserted through a vein in your neck or groin and into your heart
A numbing medicine may be applied at the site of insertion
Most patients find the procedure uncomfortable, rather than painful, and complications are uncommon
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

20. Right Heart Catheterization
Why is it important?
The only test that can diagnose PAH
Helps measure the severity of your PAH
Helps guide therapy decisions
Can help tell if there are other issues contributing to your symptoms or condition
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

21. Make the Most of Your Appointment
Ask your doctor
What do my test results mean?
Do my results show any signs of disease progression?
How do these results affect my treatment plan?
Do we need to repeat this test, and if so, when?
Keep in mind: Many tests will be repeated to monitor your condition and evaluate how well your treatment plan is working.
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

22. Make the Most of Your Appointment
Share with your doctor
How you’re feeling, especially if it has changed
Any new symptoms or side effects you’ve noticed
The activities that cause you to have symptoms
What you would like to be able to do
The information you share with your doctor, along with your test results, can help guide decisions about your treatment.
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

23. Call 1-877-948-9137 or enroll online at LivingPAH.com.
A Resource for You
Living PAH is an information and support program for patients and caregivers
Living PAH provides
E-newsletters, each with lifestyle tips
Tools and resources, including a set of low-sodium recipes
PAH disease education and treatment information
The opportunity to learn from other PAH patients and caregivers
Enroll and get access to all the support and resources Living PAH has to offer.
Call or enroll online at LivingPAH.com.
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

24. A Review We hope this presentation helped you better understand
The effects of PAH on your body
The many reasons for testing
What to expect during five common tests
How to talk to your doctor about your tests
Where you can find more information
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

25. Thank You
© 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162