Idiopathic Pulmonary Arterial Hypertension Differential Diagnoses

1. [Guideline] Galiè N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A, 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. 2015 Dec. 46(6):903-975. [QxMD MEDLINE Link].

2. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24. 62(25 Suppl):D34-41. [QxMD MEDLINE Link].

3. DRESDALE DT, SCHULTZ M, MICHTOM RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med. 1951 Dec. 11(6):686-705. [QxMD MEDLINE Link].

4. Stiles S. Novel PA-Hypertension Gene Variants Identified, Treatment Approach Hinted. Jul 26 2013. Available at http://www.medscape.com/viewarticle/808479. Accessed: Aug 1 2013.

5. Ma L, Roman-Campos D, Austin ED, Eyries M, Sampson KS, Soubrier F, et al. A novel channelopathy in pulmonary arterial hypertension. N Engl J Med. 2013 Jul 25. 369(4):351-61. [QxMD MEDLINE Link].

6. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24. 62(25 Suppl):D34-41. [QxMD MEDLINE Link].

7. Soon E, Treacy CM, Toshner MR, et al. Unexplained iron deficiency in idiopathic and heritable pulmonary arterial hypertension. Thorax. 2011 Apr. 66(4):326-32. [QxMD MEDLINE Link].

8. Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996 Aug 29. 335(9):609-16. [QxMD MEDLINE Link].

9. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006 May 1. 173(9):1023-30. [QxMD MEDLINE Link].

10. Yigla M, Kramer MR, Bendayan D, Reisner SA, Solomonov A. Unexplained severe pulmonary hypertension in the elderly: report on 14 patients. Isr Med Assoc J. 2004 Feb. 6(2):78-81. [QxMD MEDLINE Link].

11. Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004 Feb 15. 169(4):441-7. [QxMD MEDLINE Link].

12. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010 Jul 13. 122(2):164-72. [QxMD MEDLINE Link].

13. Frost AE, Farber HW, Barst RJ, Miller DP, Elliott CG, McGoon MD. Demographics and Outcomes of Patients Diagnosed With Pulmonary Hypertension With Pulmonary Capillary Wedge Pressures of 16-18 mmHg: Insights From REVEAL. Chest. 2012 May 31. [QxMD MEDLINE Link].

14. Clinical Assessment of Pulmonary Hypertension. Peacock A, Naeije R, Rubin L, eds. Pulmonary Circulation. Boca Raton, Fla: CRC Press; 2016.

15. [Guideline] McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul. 126(1 Suppl):14S-34S. [QxMD MEDLINE Link]. [Full Text].

16. [Guideline] Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul. 126(1 Suppl):4S-6S. [QxMD MEDLINE Link].

17. [Guideline] McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, 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. Circulation. 2009 Apr 28. 119(16):2250-94. [QxMD MEDLINE Link]. [Full Text].

18. Fijalkowska A, Kurzyna M, Torbicki A, Szewczyk G, Florczyk M, Pruszczyk P, et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest. 2006 May. 129(5):1313-21. [QxMD MEDLINE Link].

19. Tolle JJ, Waxman AB, Van Horn TL, Pappagianopoulos PP, Systrom DM. Exercise-induced pulmonary arterial hypertension. Circulation. 2008 Nov 18. 118(21):2183-9. [QxMD MEDLINE Link]. [Full Text].

20. Torbicki A, Kurzyna M, Kuca P, Fijalkowska A, Sikora J, Florczyk M, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003 Aug 19. 108(7):844-8. [QxMD MEDLINE Link].

21. Chakinala M, McGoon M. Pulmonary Hypertension Care Centers. Adv in PH J. Winter 2014. 12(4):175-8. [Full Text].

22. Oudiz RJ, Farber HW. Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am Heart J. 2009 Apr. 157(4):625-35. [QxMD MEDLINE Link].

23. Shapiro SM, Oudiz RJ, Cao T, Romano MA, Beckmann XJ, Georgiou D, et al. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol. 1997 Aug. 30(2):343-9. [QxMD MEDLINE Link].

24. Oudiz RJ, Schilz RJ, Barst RJ, Galié N, Rich S, Rubin LJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. 2004 Aug. 126(2):420-7. [QxMD MEDLINE Link].

25. Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002 Mar 15. 165(6):800-4. [QxMD MEDLINE Link].

26. Olschewski H, Simonneau G, Galiè N, Higenbottam T, Naeije R, Rubin LJ, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002 Aug 1. 347(5):322-9. [QxMD MEDLINE Link].

27. Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galiè N, et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015 Dec 24. 373 (26):2522-33. [QxMD MEDLINE Link].

28. [Guideline] Galiè N, Hinderliter AL, Torbicki A, Fourme T, Simonneau G, Pulido T, et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol. 2003 Apr 16. 41(8):1380-6. [QxMD MEDLINE Link].

29. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002 Mar 21. 346(12):896-903. [QxMD MEDLINE Link].

30. Galiè N, Olschewski H, Oudiz RJ, Torres F, Frost A, Ghofrani HA, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008 Jun 10. 117(23):3010-9. [QxMD MEDLINE Link].

31. Oudiz RJ, Galiè N, Olschewski H, Torres F, Frost A, Ghofrani HA, et al. Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Nov 17. 54(21):1971-81. [QxMD MEDLINE Link].

32. Simonneau G, Rubin LJ, Galiè N, Barst RJ, Fleming TR, Frost AE, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. 2008 Oct 21. 149(8):521-30. [QxMD MEDLINE Link].

33. Galiè N, Brundage BH, Ghofrani HA, Oudiz RJ, Simonneau G, Safdar Z, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009 Jun 9. 119(22):2894-903. [QxMD MEDLINE Link].

34. Ghofrani HA, D'Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med. 2013 Jul 25. 369(4):319-29. [QxMD MEDLINE Link].

35. Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013 Jul 25. 369(4):330-40. [QxMD MEDLINE Link].

36. Wood S. FDA Approves Riociguat for PAH and CTEPH. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/812292. Accessed: October 13, 2013.

37. FDA. FDA approves Adempas to treat pulmonary hypertension [press release]. US Food and Drug Administration. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm370866.htm. Accessed: October 13, 2013.

38. Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013 Aug 29. 369(9):809-18. [QxMD MEDLINE Link].

39. Stiles S. SERAPHIN: Macitentan, Novel Endothelin-Receptor Antagonist, Boosts PAH Outcomes. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/810215. Accessed: September 1, 2013.

40. Wood S. FDA approves macitentan for PAH. Medscape Medical News. October 18, 2013. [Full Text].

41. FDA. FDA approves Opsumit to treat pulmonary arterial hypertension [press release]. US Food and Drug Administration. Available at http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm371362.htm. Accessed: October 29, 2013.

42. Galiè N, Barberà JA, Frost AE, Ghofrani HA, Hoeper MM, McLaughlin VV, et al. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. N Engl J Med. 2015 Aug 27. 373 (9):834-44. [QxMD MEDLINE Link].

43. [Guideline] Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul. 126(1 Suppl):35S-62S. [QxMD MEDLINE Link].

44. [Guideline] Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007 Jun. 131(6):1917-28. [QxMD MEDLINE Link].

45. Taichman DB, Ornelas J, Chung L, et al. Pharmacologic Therapy for Pulmonary Arterial Hypertension in Adults: CHEST Guideline and Expert Panel Report. Chest. 2014 Aug 1. 146(2):449-75. [QxMD MEDLINE Link]. [Full Text].

46. Wirostko BM, Tressler C, Hwang LJ, Burgess G, Laties AM. Ocular safety of sildenafil citrate when administered chronically for pulmonary arterial hypertension: results from phase III, randomised, double masked, placebo controlled trial and open label extension. BMJ. 2012 Feb 21. 344:e554. [QxMD MEDLINE Link]. [Full Text].

47. Oudiz RJ, Brundage BH, Galiè N, Ghofrani HA, Simonneau G, Botros FT, et al. Tadalafil for the treatment of pulmonary arterial hypertension: a double-blind 52-week uncontrolled extension study. J Am Coll Cardiol. 2012 Aug 21. 60(8):768-74. [QxMD MEDLINE Link].

48. Roubinian N, Elliott CG, Barnett CF, Blanc PD, Chen J, De Marco T, et al. Effects of Commercial Air Travel on Patients with Pulmonary Hypertension. Chest. 2012 Apr 5. [QxMD MEDLINE Link].

49. de Man FS, Handoko ML, Groepenhoff H, van 't Hul AJ, Abbink J, Koppers RJ, et al. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J. 2009 Sep. 34(3):669-75. [QxMD MEDLINE Link].

50. Barst RJ, Rubin LJ, McGoon MD, Caldwell EJ, Long WA, Levy PS. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med. 1994 Sep 15. 121(6):409-15. [QxMD MEDLINE Link].

Ronald J Oudiz, MD, FACP, FACC, FCCP Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, American Thoracic Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Actelion, Bayer, Gilead, United Therapeutics<br/>Received research grant from: Actelion, Arena, Gilead, GSK, Liquidia, Reata, United Therapeutics<br/>Received income in an amount equal to or greater than $250 from: Actelion, Complexa, Gilead, Medtronic, Reata, United Therapeutics.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society

Disclosure: Nothing to disclose.

Oleh Wasyl Hnatiuk, MD Program Director, National Capital Consortium, Pulmonary and Critical Care, Walter Reed Army Medical Center; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences

Oleh Wasyl Hnatiuk, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

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