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1 Learning Objective Upon completion, participants should be able to: Describe evidence-based approaches to the measurement and management of health-related quality of life among patients with PAH 1

2 Recommendations for General Measures in PAH Recommendations lass Level t is recommended that PAH patients avoid pregnancy mmunizations of PAH patients against influenza and pneumococcal infection is recommended Psychosocial support is recommended in PAH patients Supervised exercise training should be considered in physically deconditioned PAH patients under medical therapy a B n-flight O 2 administration should be considered for patients in WHO- a F and V and those with arterial blood O 2 pressure consistently < 8 kpa (60 mm Hg) n elective surgery, epidural rather than general anesthesia is preferred whenever possible Excessive physical activity that leads to distressing symptoms is not recommended in PAH patients a Adapted from Galiè N, et al. Eur Heart J. 2016;37: Burden of PAH on Quality of Life Depression s ommon Among Patients With PAH 1-4 Patients With PAH Report: Feeling overwhelmed nability to maintain social activities Restrictions in daily tasks (eg, working, traveling, maintaining household chores) Reduction in sleep quality Frustrated with number of doctor Trouble Sleeping Feeling Down in the Morning Trouble oncentrating solation Low Energy Fear Loss of Libido appointments and mounting medical expenses Guilt Depressed Stress Difficulty Remembering Things Feeling Misunderstood P H Y S A L E M O T O N A L 1. Studer SM, et al. Advances in Pulmonary Hypertension. 2012;10: Delcroix M, et al. Eur Respir Rev. 2015;24: Verma S, et al. Lung ndia. 2016;33: Guillevin L, et al. Eur Respir Rev. 2013;22:

3 Burden of PAH on Quality of Life (cont) The debilitating impact of PAH on quality of life is clear when compared with other medical conditions Normal population [13] OPD [14] Renal failure [15] PAH [12] Highest and lowest results for the normal population Highest and lowest results for patients with PAH SF F-36 Score Physical Function Role Physical General Health Vitality Role Social Emotional Function Bodily Pain Mental Health Delcroix M, et al. Eur Respir Rev. 2015;24:621-9; Verma S, et al. Lung ndia. 2016;33:58-6; Guillevin L, et al. Eur Respir Rev. 2013;22: Screening Patients With PAH for Depression Q Why Screen Patients for Depression? Depression can impact adherence to PAH treatment, functional status, and accurate assessment of the patient s symptoms 1 Q Who is at Higher Risk of Developing Symptoms of Depression? Younger patients (< 30 years) 2 Female patients of childbearing years 2 Patients with greater functional impairment 1 Q How an Screen Patients for Depression? Use questionnaires such as the Zung Self-Rating Depression Scale, PHQ-9, and BD 3 Questionnaires can be completed while patients are waiting in office to be seen by physician 4 1. Guillevin L, et al. Eur Respir Rev. 2013;22: Hemnes AR, et al. Pulm irc. 2015;5: Verma S, et al. Lung ndia. 2016;33: Mcollister DH. J lin Pract. 2011;65:4-5. 3

4 General Principles to Follow When Supporting Patients With PAH Provide nformation From Recommended Books/Web Sites Provide nformation at Appropriate Time Encourage Joining Patient Support Group Refer to Psychosocial Services f Needed (Either Patient s Primary are Provider or a Specialist Such as a Psychiatrist) Pregnancy in Patients With PAH Pregnancy Should Be Avoided in Patients With PAH All patients should be counseled to avoid pregnancy with a thorough explanation of risks to both mother and fetus ontraceptive counseling should begin early after a patient is diagnosed Permanent contraception is strongly encouraged Progestin-only contraception and UDs are recommended Estrogen-containing contraception is not recommended Hemnes AR, et al. Pulm irc. 2015;5:

5 2015 ES/ERS PH Guidelines on Activity Level Patients Should Avoid Strenuous Exercise, but Mild Activities Seem to Be Beneficial 1 Small RTs Report That Exercise Training in Patients on Disease- Targeted Therapy Showed mprovements in 6MWD, Quality of Life, F, and Peak Oxygen onsumption 2,3,4 Patients t Should Avoid Heavy Physical Exertion or sometric Exercise 1 Recommendations Are Limited by Gaps in Knowledge About Optimal Method of Exercise Rehabilitation and the ntensity and Duration of Training 1 1. Galiè N, et al. Eur Heart J. 2016;37: Mereles D, et al. irculation. 2006;114: Weinstein AA, et al. Respir Med. 2013;107: han L, et al. hest. 2013;143: ontact nformation all toll-free info@med-iq.com Please visit us online at for additional activities sponsored by Med-Q. nstructions to Receive redit To receive credit, read the introductory ME material, watch the Webcast, and complete the evaluation, attestation, and post-test, t t answering at least 70% of the post-test t t questions correctly. The evaluation, attestation, and post-test may be accessed by clicking the Get redit tab at the bottom of the Webcast activity. 5

6 2016 Unless otherwise indicated, photographed subjects who appear within the content of this activity or on artwork associated with this activity are models; they are not actual patients or doctors. 6

7 Pulmonary Arterial Hypertension: Abbreviations and Acronyms 6MWD = 6 minute walk distance AE = adverse event BD = Beck Depression nventory BNP = brain natriuretic peptide B = calcium channel blocker PET = cardiopulmonary exercise testing = cardiac index MR = cardiac magnetic resonance OPD = chronic obstructive pulmonary disease DPAH = drug induced pulmonary arterial hypertension ERA = endothelin receptor antagonist ERS = European Respiratory Society ES = European Society of ardiology F = functional class HPAH = heritable pulmonary arterial hypertension PAH = idiopathic pulmonary arterial hypertension UD = intrauterine device V = intravenous M/M event = morbidity/morality event = composite of death from any cause or a complication related to pulmonary arterial hypertension MR = magnetic resonance NT probnp = N terminal pro B type natriuretic peptide NYHA = New York Heart Association PAH = pulmonary arterial hypertension PA = prostacyclin analogue PDE 5 = phosphodiesterase type 5 PH = pulmonary hypertension PHQ 9 = Patient Health Questionnaire 9 PVR = pulmonary vascular resistance RA = right arterial RAP = right atrial pressure RT = randomized controlled trial RV = right ventricular SF 36 = Medical Outcomes Study 36 item Form sg = soluble guanylate cyclase SvO 2 = mixed venous oxygen saturation TPR = total pulmonary resistance VE/VO 2 = ventilatory equivalents for carbon dioxide VO 2 = oxygen consumption WHO = World Health Organization

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