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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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 1-877-948-9137 or enroll online at LivingPAH.com. © 2016 United Therapeutics Corporation. All rights reserved. US/LPAH/MAR16/162

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

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