Pulmonary Arterial Hypertension Questions & Answers

1. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30. 54(1 Suppl):S43-54. [QxMD MEDLINE Link].

2. Takatsuki S, Ivy DD. Current challenges in pediatric pulmonary hypertension. Semin Respir Crit Care Med. 2013 Oct. 34 (5):627-44. [QxMD MEDLINE Link].

3. Humbert M, Morrell NW, Archer SL, Stenmark KR, MacLean MR, Lang IM, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16. 43 (12 Suppl S):13S-24S. [QxMD MEDLINE Link].

4. 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].

5. Fernandes CJ, Jardim CV, Hovnanian A, Hoette S, Morinaga LK, Souza R. Schistosomiasis and pulmonary hypertension. Expert Rev Respir Med. 2011 Oct. 5 (5):675-81. [QxMD MEDLINE Link].

6. Lapa M, Dias B, Jardim C, Fernandes CJ, Dourado PM, Figueiredo M, et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation. 2009 Mar 24. 119 (11):1518-23. [QxMD MEDLINE Link].

7. Ferreira RC, Domingues AL, Bandeira AP, Markman Filho B, Albuqerque Filho ES, Correiade de Araújo AC, et al. Prevalence of pulmonary hypertension in patients with schistosomal liver fibrosis. Ann Trop Med Parasitol. 2009 Mar. 103 (2):129-43. [QxMD MEDLINE Link].

8. McGoon MD, Benza RL, Escribano-Subias P, Jiang X, Miller DP, Peacock AJ, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol. 2013 Dec 24. 62 (25 Suppl):D51-9. [QxMD MEDLINE Link].

9. Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med. 2008 Jan 1. 177(1):108-13. [QxMD MEDLINE Link].

10. Speich R, Jenni R, Opravil M, Pfab M, Russi EW. Primary pulmonary hypertension in HIV infection. Chest. 1991 Nov. 100(5):1268-71. [QxMD MEDLINE Link].

11. Battle RW, Davitt MA, Cooper SM, et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest. 1996 Dec. 110(6):1515-9. [QxMD MEDLINE Link].

12. Frost AE, Badesch DB, Barst RJ, Benza RL, Elliott CG, Farber HW, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest. 2011 Jan. 139 (1):128-37. [QxMD MEDLINE Link].

13. Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012 Aug. 142 (2):448-456. [QxMD MEDLINE Link].

14. Sitbon O, Benza RL, Badesch DB, Barst RJ, Elliott CG, Gressin V, et al. Validation of two predictive models for survival in pulmonary arterial hypertension. Eur Respir J. 2015 Jul. 46 (1):152-64. [QxMD MEDLINE Link].

15. McLaughlin V. Managing pulmonary arterial hypertension and optimizing treatment options: prognosis of pulmonary artery hypertension. Am J Cardiol. 2013 Apr 16. 111 (8 Suppl):10C-5C. [QxMD MEDLINE Link].

16. Tonelli AR, Arelli V, Minai OA, Newman J, Bair N, Heresi GA, et al. Causes and circumstances of death in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2013 Aug 1. 188 (3):365-9. [QxMD MEDLINE Link].

17. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991 Sep 1. 115 (5):343-9. [QxMD MEDLINE Link].

18. Demerouti EA, Manginas AN, Athanassopoulos GD, Karatasakis GT. Complications leading to sudden cardiac death in pulmonary arterial hypertension. Respir Care. 2013 Jul. 58 (7):1246-54. [QxMD MEDLINE Link].

19. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Rev Esp Cardiol (Engl Ed). 2016 Feb. 69 (2):177. [QxMD MEDLINE Link].

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

21. Arkles JS, Opotowsky AR, Ojeda J, Rogers F, Liu T, Prassana V, et al. Shape of the right ventricular Doppler envelope predicts hemodynamics and right heart function in pulmonary hypertension. Am J Respir Crit Care Med. 2011 Jan 15. 183(2):268-76. [QxMD MEDLINE Link].

22. Maron BA, Galiè N. Diagnosis, Treatment, and Clinical Management of Pulmonary Arterial Hypertension in the Contemporary Era: A Review. JAMA Cardiol. 2016 Dec 1. 1 (9):1056-1065. [QxMD MEDLINE Link].

23. Sitbon O, Humbert M, Jagot JL, et al. Inhaled nitric oxide as a screening agent for safely identifying responders to oral calcium-channel blockers in primary pulmonary hypertension. Eur Respir J. 1998 Aug. 12(2):265-70. [QxMD MEDLINE Link].

24. Wiener RS, Ouellette DR, Diamond E, Fan VS, Maurer JR, Mularski RA, et al. An official American Thoracic Society/American College of Chest Physicians policy statement: the Choosing Wisely top five list in adult pulmonary medicine. Chest. 2014 Jun. 145 (6):1383-1391. [QxMD MEDLINE Link].

25. Ventetuolo CE, Klinger JR. WHO Group 1 pulmonary arterial hypertension: current and investigative therapies. Prog Cardiovasc Dis. 2012 Sep-Oct. 55 (2):89-103. [QxMD MEDLINE Link].

26. Taichman DB, Ornelas J, Chung L, Klinger JR, Lewis S, Mandel J, et al. Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report. Chest. 2014 Aug. 146 (2):449-475. [QxMD MEDLINE Link].

27. Johnson SR, Granton JT, Mehta S. Thrombotic arteriopathy and anticoagulation in pulmonary hypertension. Chest. 2006 Aug. 130(2):545-52. [QxMD MEDLINE Link].

28. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J. 2006 Nov. 28(5):999-1004. [QxMD MEDLINE Link].

29. 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].

30. Mathai SC, Girgis RE, Fisher MR, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007 Mar. 29(3):469-75. [QxMD MEDLINE Link].

31. Simonneau G, Rubin LJ, Galie N, 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].

32. López-Meseguer M, Quezada CA, Ramon MA, Lázaro M, Dos L, Lara A, et al. Lung and heart-lung transplantation in pulmonary arterial hypertension. PLoS One. 2017. 12 (11):e0187811. [QxMD MEDLINE Link].

33. Mereles D, Ehlken N, Kreuscher S, Ghofrani S, Hoeper MM, Halank M, et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006 Oct 3. 114 (14):1482-9. [QxMD MEDLINE Link].

34. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992 Jul 9. 327(2):76-81. [QxMD MEDLINE Link].

35. Sitbon O, Humbert M, Jaïs X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005 Jun 14. 111(23):3105-11. [QxMD MEDLINE Link].

36. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996 Feb 1. 334(5):296-302. [QxMD MEDLINE Link].

37. Rubin LJ, Mendoza J, Hood M, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med. 1990 Apr 1. 112(7):485-91. [QxMD MEDLINE Link].

38. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002 Aug 21. 40(4):780-8. [QxMD MEDLINE Link].

39. Aguilar RV, Farber HW. Epoprostenol (prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med. 2000 Nov. 162(5):1846-50. [QxMD MEDLINE Link].

40. Simonneau G, Barst RJ, Galie N, 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].

41. Lang I, Gomez-Sanchez M, Kneussl M, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest. 2006 Jun. 129(6):1636-43. [QxMD MEDLINE Link].

42. McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol. 2010 May 4. 55(18):1915-22. [QxMD MEDLINE Link].

43. Waxman A, Restrepo-Jaramillo R, Thenappan T, Ravichandran A, Engel P, Bajwa A, et al. Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. N Engl J Med. 2021 Jan 28. 384 (4):325-334. [QxMD MEDLINE Link].

44. Jing ZC, Parikh K, Pulido T, Jerjes-Sanchez C, White RJ, Allen R, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation. 2013 Feb 5. 127(5):624-33. [QxMD MEDLINE Link].

45. Tapson VF, Torres F, Kermeen F, Keogh AM, Allen RP, Frantz RP, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial. Chest. 2012 Dec. 142(6):1383-90. [QxMD MEDLINE Link].

46. Tapson VF, Jing ZC, Xu KF, Pan L, Feldman J, Kiely DG, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial. Chest. 2013 Sep. 144(3):952-8. [QxMD MEDLINE Link].

47. Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002 Aug 1. 347(5):322-9. [QxMD MEDLINE Link].

48. Opitz CF, Wensel R, Winkler J, et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J. 2005 Sep. 26(18):1895-902. [QxMD MEDLINE Link].

49. 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].

50. Li HH, Hsu HH, Chang GJ, Chen IC, Ho WJ, Hsu PC, et al. Prostanoid EP4 Agonist L-902,688 Activates PPARγ and Attenuates Pulmonary Arterial Hypertension. Am J Physiol Lung Cell Mol Physiol. 2017 Nov 16. ajplung.00245.2017. [QxMD MEDLINE Link].

51. Furukawa A, Tamura Y, Iwahori H, Goto M, Ohashi N, Okabe T, et al. Successful transition from Treprostinil to Selexipag in patient with severe pulmonary arterial hypertension. BMC Pulm Med. 2017 Oct 26. 17 (1):135. [QxMD MEDLINE Link].

52. Hughes RJ, Jais X, Bonderman D, et al. The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. Eur Respir J. 2006 Jul. 28(1):138-43. [QxMD MEDLINE Link].

53. Kenyon KW, Nappi JM. Bosentan for the treatment of pulmonary arterial hypertension. Ann Pharmacother. 2003 Jul-Aug. 37(7-8):1055-62. [QxMD MEDLINE Link].

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

55. Sharma S, Kashour T, Philipp R. Secondary pulmonary arterial hypertension: treated with endothelin receptor blockade. Tex Heart Inst J. 2005. 32(3):405-10. [QxMD MEDLINE Link].

56. Galiè N, Olschewski H, Oudiz RJ, 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].

57. 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].

58. Stiles S. SERAPHIN: Macitentan, Novel Endothelin-Receptor Antagonist, Boosts PAH Outcomes. Medscape Medical News. Available at http://www.medscape.com/viewarticle/810215. Accessed: October 23, 2013.

59. Aypar E, Alehan D, Karagöz T, Aykan HH, Ertugrul İ. Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension. Cardiol Young. 2017 Dec 13. 1-6. [QxMD MEDLINE Link].

60. Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005 Nov 17. 353(20):2148-57. [QxMD MEDLINE Link].

61. Lee AJ, Chiao TB, Tsang MP. Sildenafil for pulmonary hypertension. Ann Pharmacother. 2005 May. 39(5):869-84. [QxMD MEDLINE Link].

62. Reichenberger F, Voswinckel R, Enke B, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J. 2007 Nov. 30(5):922-7. [QxMD MEDLINE Link].

63. Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J. 2006 Apr. 151(4):851.e1-5. [QxMD MEDLINE Link].

64. Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009 Jun 9. 119(22):2894-903. [QxMD MEDLINE Link].

65. Jing ZC, Yu ZX, Shen JY, et al. Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med. 2011 Jun 15. 183(12):1723-9. [QxMD MEDLINE Link].

66. 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].

67. 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].

Kristin E Schwab, MD Fellow in Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, Los Angeles, David Geffen School of Medicine

Disclosure: Nothing to disclose.

Nader Kamangar, MD, FACP, FCCP, FCCM Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Chief, Division of Pulmonary and Critical Care Medicine, Vice-Chair, Department of Medicine, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: Academy of Persian Physicians, American Academy of Sleep Medicine, American Association for Bronchology and Interventional Pulmonology, American College of Chest Physicians, American College of Critical Care Medicine, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, Association of Pulmonary and Critical Care Medicine Program Directors, Association of Specialty Professors, California Sleep Society, California Thoracic Society, Clerkship Directors in Internal Medicine, Society of Critical Care Medicine, Trudeau Society of Los Angeles, World Association for Bronchology and Interventional Pulmonology

Disclosure: Nothing to disclose.

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.

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba Faculty of Medicine; Site Director, Respiratory Medicine, St Boniface General Hospital, Canada

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, World Medical Association

Disclosure: Nothing to disclose.

Kelvin Chan, MD Resident Physician, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Olive View-UCLA Medical Center

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.

Shahriar Pirouz, MD Resident Physician, Department of Internal Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Reference Salary Employment

• Print this section
• Print the entire contents of
• Print the entire contents of article