for Nurses

1. What Is PAH?

1What Is PAH?

What Is Pulmonary Arterial Hypertension?

Pulmonary arterial hypertension (PAH) is a rare subset of pulmonary hypertension (PH).1

  • It is a progressive, potentially life-threatening disease affecting the pulmonary arteries of the lungs2
  • The World Health Organization (WHO) classifies PH into 5 groups. PAH is WHO Group 13

WHO PH Clinical Classifications3

*CTEPH=chronic thromboembolic pulmonary hypertension

How Does PAH Affect the Right Side of the Heart?

How Common Is PAH?

What Signs and Symptoms Should You Be Looking For?

What Are the Different Types of PAH?

What to Look For in a Physical Examination

What Is the Right-Heart Catheterization Test?

How Do You Assess Functional Class?

The Importance of the 6-Minute Walk Test

How Is PAH Managed?

2 What is the role of the nurse?

Nurses are an essential part of the health care professional team in the journey of a patient with PAH.

  • Since it is a chronic condition, patients require an interdisciplinary team to continually monitor disease progression, making the role of the nurse vital19

Roles include19:

  1. Working directly with the physician and patient in monitoring treatment
    • Improve patient adherence
    • Reaching treatment goals
  2. Provide follow-up and day-to-day care
  3. Discuss care with the patient’s support system
  4. Assist patients with obtaining medication approval and delivery
  5. Managing fluid volume status12

Playing an Active Role in the Patient’s HCP Team

What Do I Need to Communicate?

How Do I Explain PAH to Patients?

What Is Life Like for PAH Patients?

The Discussion of Palliative Care

3What Is optimal treatment for my patient?

How Does the Patient Receive Optimal Treatment?

Patients who are involved in the decision making regarding their disease are more engaged and motivated.20

  • Patients who understand the benefits of being actively involved in decisions about their treatment and care are able to make informed decisions and to take ownership of their disease, which leads to improved outcomes32

What Is Patient Activation?

Importance of Patient Adherence

Patient’s Self-Care: Physical Care, Mental Health

Patient’s Support System: Involving the Caregiver

Role of Support Groups

4 Additional Resources

Patients increasingly seek information regarding PAH via the internet27 and it is vital that this information is correct and that health care providers, especially nurses, are aware of sources of high-quality information.

  • Additional links are provided below to assist with further learning about PAH in regards to the disease, management and patient care education

5references

  1. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D42-50.
  2. Lai YC, Potoka KC, Champion HC, et al. Pulmonary arterial hypertension the clinical syndrome. Circ Res. 2014;115(1):115-130.
  3. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.
  4. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-1619.
  5. Ross CA. Pulmonary Arterial Hypertension: Early recognition and treatment can make a lifetime difference for your patient. J Nurse Pract. 2007;3(6):404–409.
  6. Kovacs G, Berghold A, Scheidl S, et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systemic review. Eur Respir J. 2009;34(4):888-894.
  7. Peacock AJ, Murphy NF, McMurray JJ, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30(1):104-109.
  8. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023-1030.
  9. Rich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216–223.
  10. Badesch DB, Raskob GE, Elliot CG. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376-387.
  11. Palevsky HI. The early diagnosis of pulmonary arterial hypertension: can we do better? Chest. 2011;140(1):4-6.
  12. Archer-Chicko C. Nursing care of patients with pulmonary arterial hypertension. In: Yuan JXJ, Garcia JGN, Hales CA, et al, eds. Textbook of Pulmonary Vascular Disease. 1st ed. New York, NY: Springer Science+Business Media;2011:1531-1558.
  13. Rich JD, Rich S. Clinical diagnosis of pulmonary hypertension. Circulation. 2014;130(20):1820-1830.
  14. McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114(13):1417-1431.
  15. Crapo RO, Casaburi R, Coates AL, et al. ATS statement guidelines for the six-minute walk test. Am J Respir Crit Care Med. 2002;166(1):111-117.
  16. Barnett CT, Jacmann JS, Moore NC, et al. The effect of walking path configuration on six-minute walk test performance and gait variability: a pilot study. Gait & Posture. 2015;42(3):S54.
  17. Maron BA, Galiè N. Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: a review. JAMA Cardiol. 2016;1(9):1056-1065.
  18. Galiè N, Corris PA, Frost A, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013;62(suppl 25):D60-D72.
  19. Tartavoulle TM. Evaluation and management of the adult patient with pulmonary hypertension. J Nurse Pract. 2011;3(5):409-416.
  20. Graarup J, Ferrari P, Howard LS. Patient engagement and self-management in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):399-407.
  21. Gerwing J, Indseth T, Gulbrandsen P. A microanalysis of the clarity of information in physicians’ and patients’ discussions of treatment plans with and without language barriers. Patient Educ Couns. 2016;99(4):522-529.
  22. Rahaghi FF, Chastain VL, Benavides R, et al. Shared medical appointments in pulmonary hypertension. Pulm Circ. 2014;4(1):53-60.
  23. Guillevin L, Armstrong I, Aldrighetti R, et al. Understanding the impact of pulmonary arterial hypertension on patients’ and carers’ lives. Eur Respir Rev. 2013;22(130):535-542.
  24. Stewart T. Facilitating pulmonary arterial hypertension medication adherence: patient-centered management. Adv Pulm Hypertens. 2010;8(4):228-231.
  25. DeSmedt RH, Denig P, Haaijer-Ruskamp FM, et al. Perceived medication adverse effects and coping strategies reported by chronic heart failure patients. Int J Clin Pract. 2009;63(2):233-242.
  26. Dickens C, Lambert BL, Cromwell T, et al. Nurse overestimation of patients’ health literacy. J Health Commun. 2013;18(suppl 1):62-69.
  27. Parker RM, Ratzan SC, Lurie N. Health literacy: a policy challenge for advancing high-quality health care. Health Aff (Millwood). 2003;22(4):147-153.
  28. Poms AD, Turner M, Farber HW, et al. Comorbid conditions and outcomes in patients with pulmonary arterial hypertension: a REVEAL registry analysis. Chest. 2013;144(1):169-176.
  29. White J, Hopkins RO, Glissmeyer EW, et al. Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension. Respir Res. 2006;7:55.
  30. Bonnin M, Mercier FJ, Sitbon O, et al. Severe pulmonary hypertension during pregnancy: a mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology. 2005;102(6):1133-1137.
  31. Fenstad ER, Wordingham SE, Swetz KM. Pulmonary hypertension and palliative care: what, when, where, and why? Advances in Pulmonary Hypertension. 2016;15(1):26-31.
  32. Ellins J, Coulter A. The Health Foundation, Picker Institute Europe, Oxford, United Kingdom. How engaged are people in their health care? Findings of a national telephone survey. November 2005.
    https://www.picker.org/wp-content/uploads/2014/10/How-engaged-are-people-in-their-health-care-....pdf. Accessed November 27, 2018.
  33. Carman KL, Dardess P, Maurer M, et al. Patient and family engagement: a framework for understanding the elements and developing interventions and policies. Health Aff (Millwood). 2013;32(2):223-231.
  34. Hibbard JH, Stockard J, Mahoney ER, et al. Development of the patient activation measure (PAM): conceptualizing and measuring activation in patients and consumers. Health Serv Res. 2004;39(4 Pt 1):1005-1026.
  35. Eriksson M, Lindström B. Antonovsky’s sense of coherence scale and the relation with health: a systemic review. J Epidemiol Commun Health. 2006;60(5):376-381.
  36. Turner A, Anderson JK, Wallace LM, et al. An evaluation of a self-management program for patients with long-term conditions. Patient Educ Couns. 2015;98(2):213-219.
  37. Waxman A, Chen SY, Boulanger L, et al. Factors associated with adherence to phosphodiesterase type 5 inhibitors for the treatment of pulmonary arterial hypertension. J Med Econ. 2013;16(2):298-306.
  38. PHA Europe. The impact of pulmonary arterial hypertension (PAH) on the lives of patients and carers: results from an international survey. http://www.phaeurope.org/wp-content/uploads/International-PAH-patient-and-Carer-Survey-Report-FINAL1.pdf. Updated 2012. Accessed November 26, 2018.
© United Therapeutics Corporation 2018.
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US/DS/0228 12/18

This MPR/PulmonologyAdvisor Fact Pack is produced as a basic reminder of important information for healthcare professionals. Readers are advised to consult manufacturers and specialists if questions arise about specific products, treatments, or diseases. The publisher and editors do not assume liability for any errors or omissions. MPR, PulmonologyAdvisor, and Fact Pack are registered trademarks of Haymarket Media, Inc.

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