Pulmonary Hypertension: Diagnosis and Treatment

 


FREE PREVIEW. AAFP members and paid subscribers: Log in to get free access. All others: Purchase online access.


FREE PREVIEW. Purchase online access to read the full version of this article.

Am Fam Physician. 2016 Sep 15;94(6):463-469.

Author disclosure: No relevant financial affiliations.

Pulmonary hypertension is a common, complex group of disorders that result from different pathophysiologic mechanisms but are all defined by a mean pulmonary arterial pressure of 25 mm Hg or greater. Patients often initially present to family physicians; however, because the symptoms are typically nonspecific or easily attributable to comorbid conditions, diagnosis can be challenging and requires a stepwise evaluation. There is limited evidence to support screening of asymptomatic individuals. Echocardiography is recommended as the initial step in the evaluation of patients with suspected pulmonary hypertension. A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization. For certain categories of pulmonary hypertension, particularly pulmonary arterial hypertension, treatment options are rapidly evolving, and early diagnosis and prompt referral to an expert center are critical to ensure the best prognosis. There are no directed therapies for many other categories of pulmonary hypertension; therefore, family physicians have a central role in managing contributing comorbidities. Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health.

Pulmonary hypertension is a heterogeneous group of disorders characterized by a mean pulmonary arterial pressure of 25 mm Hg or greater at rest during right heart catheterization.14 Patients with pulmonary hypertension may initially present to family physicians, but symptoms such as dyspnea on exertion and fatigue are nonspecific and may be attributed to comorbid conditions.5 Pulmonary hypertension is categorized on the basis of pathophysiology, hemodynamics, and therapeutic options. In 2013, the classification scheme was updated to recognize five groups of pulmonary hypertension6  (Table 13,4,6).

View/Print Table

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Echocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension.

C

13, 22

Results from invasive hemodynamic testing with right heart catheterization, preferably at an expert center, should be obtained before initiating treatment with vasodilator therapy in patients with pulmonary arterial hypertension.

C

13

In patients with pulmonary hypertension due to lung disease or left heart disease, treatment should focus on optimizing comorbid conditions.

C

3, 911

In patients with pulmonary hypertension and hypoxia, supplemental oxygen should be administered to maintain saturation above 90%.

C

11, 28, 29

Use of vasodilator therapies in patients with pulmonary hypertension due to lung disease or left heart disease is potentially harmful and not recommended.

C

10, 11, 26

Patients with chronic thromboembolic pulmonary hypertension should receive lifelong anticoagulation in the absence of contraindications.

C

25

Patients with pulmonary hypertension should receive seasonal influenza vaccination and age-appropriate pneumococcal vaccination, unless contraindicated.

A

1, 31, 32

Perioperative assessment of patients with pulmonary hypertension should include echocardiographic assessment of right ventricular function.

C

33


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Echocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension.

C

13, 22

Results from invasive hemodynamic testing with right heart catheterization, preferably at an expert center, should be obtained before initiating treatment with vasodilator therapy in patients with pulmonary arterial hypertension.

C

13

In patients with pulmonary hypertension due to lung disease or left heart disease, treatment should focus on optimizing comorbid conditions.

C

3, 911

In patients with pulmonary hypertension and hypoxia, supplemental oxygen should be administered to maintain saturation above 90%.

C

11, 28, 29

Use of vasodilator therapies in patients with pulmonary hypertension due to lung disease or left heart disease is potentially harmful and not recommended.

C

10, 11, 26

Patients with chronic thromboembolic pulmonary hypertension should receive lifelong anticoagulation in the absence of contraindications.

C

25

Patients with pulmonary hypertension should receive seasonal influenza vaccination and age-appropriate pneumococcal vaccination, unless contraindicated.

A

1, 31, 32

Perioperative assessment of patients with pulmonary hypertension should include echocardiographic assessment of right ventricular function.

C

33


A = con

The Authors

show all author info

BETH DUNLAP, MD, is a family physician at Erie Family Health Center, Waukegan, Ill.; an assistant professor of family medicine at Northwestern University, Chicago, Ill.; and the associate program director for the Northwestern McGaw Family Medicine Residency at Lake Forest, Grayslake, Ill....

GEORGE WEYER, MD, is an assistant professor of medicine at the University of Chicago and also practices within the University of Chicago Primary Care Group.

Address correspondence to Beth Dunlap, MD, Northwestern University, 710 Lake Shore Dr., 4th Fl., Chicago, IL 60611 (e-mail: bdunlap@eriefamilyhealth.org). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014;146(2):449–475....

2. 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). Eur Heart J. 2016;37(1):67–119.

3. 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 [published correction appears in Circulation. 2009;120(2):e13]. Circulation. 2009;119(16):2250–2294.

4. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D42–D50.

5. Hoeper MM, Simon R, Gibbs J. The changing landscape of pulmonary arterial hypertension and implications for patient care. Eur Respir Rev. 2014;23(134):450–457.

6. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension [published correction appears in J Am Coll Cardiol. 2014;63(7):746]. J Am Coll Cardiol. 2013;62(25 suppl):D34–D41.

7. George MG, Schieb LJ, Ayala C, Talwalkar A, Levant S. Pulmonary hypertension surveillance: United States, 2001 to 2010. Chest. 2014;146(2):476–495.

8. Coghlan JG, Denton CP, Grünig E, et al.; DETECT study group. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–1349.

9. Guazzi M, Borlaug BA. Pulmonary hypertension due to left heart disease. Circulation. 2012;126(8):975–990.

10. Vachiéry JL, Adir Y, Barberà JA, et al. Pulmonary hypertension due to left heart diseases. J Am Coll Cardiol. 2013;62(25 suppl):D100–D108.

11. Seeger W, Adir Y, Barberà JA, et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013;62(25 suppl):D109–D116.

12. Pengo V, Lensing AW, Prins MH, et al.; Thromboembolic Pulmonary Hypertension Study Group. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350(22):2257–2264.

13. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008;359(21):2254–2265.

14. Klings ES, Machado RF, Barst RJ, et al. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014;189(6):727–740.

15. Lam CS, Borlaug BA, Kane GC, Enders FT, Rodeheffer RJ, Redfield MM. Age-associated increases in pulmonary artery systolic pressure in the general population. Circulation. 2009;119(20):2663–2670.

16. Strange G, Gabbay E, Kermeen F, et al. Time from symptoms to definitive diagnosis of idiopathic pulmonary arterial hypertension: the delay study. Pulm Circ. 2013;3(1):89–94.

17. Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur Respir J. 2010;35(5):1079–1087.

18. Tuder RM, Archer SL, Dorfmüller P, et al. Relevant issues in the pathology and pathobiology of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D4–D12.

19. Humbert M. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology. Eur Respir Rev. 2010;19(115):59–63.

20. Yuan JX, Rubin LJ. Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation. 2005;111(5):534–538.

21. Vonk Noordegraaf A, Galiè N. The role of the right ventricle in pulmonary arterial hypertension. Eur Respir Rev. 2011;20(122):243–253.

22. Agency for Healthcare Research and Quality. Pulmonary arterial hypertension: screening, management, and treatment: executive summary. April 25, 2013. http://effectivehealthcare.ahrq.gov/index.cfm/search-for-guides-reviews-and-reports/?productid=1479&pageaction=displayproduct. Accessed August 18, 2015.

23. Janda S, Shahidi N, Gin K, Swiston J. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis [published correction appears in Heart. 2011;97(13):1112]. Heart. 2011;97(8):612–622.

24. Farber HW, Foreman AJ, Miller DP, McGoon MD. REVEAL registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension. Congest Heart Fail. 2011;17(2):56–64.

25. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 suppl):D92–D99.

26. Halpern SD, Becker D, Curtis JR, et al. An official American Thoracic Society/American Association of Critical-Care Nurses/American College of Chest Physicians/Society of Critical Care Medicine policy statement: the Choosing Wisely® top 5 list in critical care medicine. Am J Respir Crit Care Med. 2014;190(7):818–826.

27. Simonneau G, Torbicki A, Hoeper MM, et al. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J. 2012;40(4):874–880.

28. Global Initiative for Chronic Obstructive Lung Disease. Global strategy for diagnosis, management, and prevention of COPD—2016. http://goldcopd.org/global-strategy-diagnosis-management-prevention-copd-2016/. Accessed June 10, 2016.

29. Vestbo J, Hurd SS, Agustí AG, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med. 2013;187(4):347–365.

30. Arias MA, García-Río F, Alonso-Fernández A, Martínez I, Villamor J. Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Eur Heart J. 2006;27(9):1106–1113.

31. Kim DK, Bridges CB, Harriman KH. Advisory Committee on Immunization Practices recommended immunization schedule for adults aged 19 years or older—United States, 2016. MMWR Morb Mortal Wkly Rep. 2016;65(4):88–90.

32. Tomczyk S, Bennett NM, Stoecker C, et al.; Centers for Disease Control and Prevention (CDC). Use of 13-valent pneumococcal conjugate vaccine and 23-valent pneumococcal polysaccharide vaccine among adults aged ≥65 years: recommendations of the Advisory Committee on Immunization Practices (ACIP). MMWR Morb Mortal Wkly Rep. 2014;63(37):822–825.

33. Klinger JR, Frantz RP, eds. Diagnosis and Management of Pulmonary Hypertension. New York, NY: Humana Press; 2015:437–464.

34. Olsson KM, Jais X. Birth control and pregnancy management in pulmonary hypertension. Semin Respir Crit Care Med. 2013;34(5):681–688.

35. Kingman M, Hinzmann B, Sweet O, Vachiéry JL. Living with pulmonary hypertension: unique insights from an international ethnographic study. BMJ Open. 2014;4(5):e004735.

36. Stringham R, Shah NR. Pulmonary arterial hypertension: an update on diagnosis and treatment. Am Fam Physician. 2010;82(4):370–377.



 

Copyright © 2016 by the American Academy of Family Physicians.
This content is owned by the AAFP. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Contact afpserv@aafp.org for copyright questions and/or permission requests.

Want to use this article elsewhere? Get Permissions

CME Quiz

More in AFP


Related Content


More in Pubmed

Email Alerts

Don't miss a single issue. Sign up for the free AFP email table of contents.

Sign Up Now

Navigate this Article