Respiratory Medicine
Volume 104, Issue 11 , Pages 1588-1607 , November 2010

Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: A systematic review of the literature

  • John Robert Swiston

      Affiliations

    • Division of Respirology, University of British Columbia, Vancouver General Hospital, 7th floor – 2775 Laurel Street, Vancouver BC V5Z 1M9, Canada
    • Corresponding Author InformationCorresponding author. Tel.: +1 604 875 4122; fax: +1 604 875 4695.
    • ,
  • Sindhu R. Johnson

      Affiliations

    • University of Toronto, Canada
    • ,
  • John T. Granton

      Affiliations

    • University of Toronto, Canada

Received 26 April 2010 ,Accepted 9 August 2010.

References 

  1. 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. J Am Coll Cardiol. 2009 Apr 28;53(17):1573–1619
  2. Badesch DB, Champion HC, Sanchez MA, Hoeper MM, Loyd JE, Manes A, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl.):S55–S66
  3. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl.):S43–S54
  4. 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):343–349
  5. Barst RJ, Gibbs JS, Ghofrani HA, Hoeper MM, McLaughlin VV, Rubin LJ, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl.):S78–S84
  6. Nootens M, Freels S, Kaufmann E, Levy PS, Rich S. Timing of single lung transplantation for primary pulmonary hypertension. J Heart Lung Transpl. 1994 Mar–Apr;13(2):276–281
  7. Glanville AR, Burke CM, Theodore J, Robin ED. Primary pulmonary hypertension. Length of survival in patients referred for heart-lung transplantation. Chest. 1987 May;91(5):675–681
  8. Keogh AM, Mayer E, Benza RL, Corris P, Dartevelle PG, Frost AE, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl.):S67–S77
  9. Badesch DB, McLaughlin VV, Delcroix M, Vizza CD, Olschewski H, Sitbon O, et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl. S):56S–61S
  10. Gomberg-Maitland M, Olschewski H. Prostacyclin therapies for the treatment of pulmonary arterial hypertension. Eur Respir J. 2008 Apr;31(4):891–901
  11. Galie N, Seeger W, Naeije R, Simonneau G, Rubin LJ. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl. S):81S–88S
  12. Humbert M, McLaughlin VV. The 4th World symposium on pulmonary hypertension. Introduction. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl.):S1–S2
  13. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1 Suppl.):4S–6S
  14. Nagaya N, Nishikimi T, Uematsu M, Satoh T, Kyotani S, Sakamaki F, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000 Aug 22;102(8):865–870
  15. Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. August 21, 2002;40(4):780–788
  16. Kanemoto N. Electrocardiogram in primary pulmonary hypertension–with special reference to prognosis. Tokai J Exp Clin Med. 1987 Sep;12(3):173–179
  17. Chapman PJ, Bateman ED, Benatar SR. Prognostic and therapeutic considerations in clinical primary pulmonary hypertension. Respir Med. 1990 Nov;84(6):489–494
  18. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol. 2002 Apr 3;39(7):1214–1219
  19. van Wolferen SA, Marcus JT, Boonstra A, Marques KM, Bronzwaer JG, Spreeuwenberg MD, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007 May;28(10):1250–1257
  20. Rozkovec A, Montanes P, Oakley CM. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J. 1986 May;55(5):449–458
  21. Wensel R, Opitz CF, Anker SD, Winkler J, Hoffken G, Kleber FX, et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation. 2002 Jul 16;106(3):319–324
  22. Rich S, Brundage BH, Levy PS. The effect of vasodilator therapy on the clinical outcome of patients with primary pulmonary hypertension. Circulation. 1985 Jun;71(6):1191–1196
  23. Paciocco G, Martinez FJ, Bossone E, Pielsticker E, Gillespie B, Rubenfire M. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J. April 1, 2001;17(4):647–652
  24. Bossone E, Paciocco G, Iarussi D, Agretto A, Iacono A, Gillespie BW, et al. The prognostic role of the ECG in primary pulmonary hypertension. Chest. 2002 Feb;121(2):513–518
  25. 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):1313–1321
  26. Kanemoto N. Natural history of pulmonary hemodynamics in primary pulmonary hypertension. Am Heart J. 1987 Aug;114(2):407–413
  27. Sandoval J, Bauerle O, Palomar A, Gomez A, Martinez-Guerra ML, Beltran M, et al. Survival in primary pulmonary hypertension. Validation of a prognostic equation. Circulation. 1994 Apr;89(4):1733–1744
  28. Chun KJ, Kim SH, An BJ, Kim SH, Ha JK, Hong TJ, et al. Survival and prognostic factors in patients with primary pulmonary hypertension. Korean J Intern Med. 2001 Jun;16(2):75–79
  29. Appelbaum L, Yigla M, Bendayan D, Reichart N, Fink G, Priel I, et al. Primary pulmonary hypertension in Israel: a national survey. Chest. 2001 Jun;119(6):1801–1806
  30. Okada O, Tanabe N, Yasuda J, Yoshida Y, Katoh K, Yamamoto T, et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980–1990. Intern Med. 1999 Jan;38(1):12–16
  31. Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M. Contemporary survival in patients with pulmonary arterial hypertension: a reappraisal of the National Institutes of Health Risk Stratification Equation. Eur Respir J. 2009 Dec 23;
  32. Miyamoto S, Nagaya N, Satoh T, Kyotani S, Sakamaki F, Fujita M, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):487–492
  33. McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. February 1, 2005;25(2):244–249
  34. Paggiaro PL, Chanez P, Holz O, Ind PW, Djukanovic R, Maestrelli P, et al. Sputum induction. Eur Respir J Suppl. 2002 Sep;37:3s–8s
  35. Provencher S, Sitbon O, Humbert M, Cabrol S, Jais X, Simonneau G. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J. 2006 Mar;27(5):589–595
  36. Mahapatra S, Nishimura RA, Sorajja P, Cha S, McGoon MD. Relationship of pulmonary arterial capacitance and mortality in idiopathic pulmonary arterial hypertension. J Am Coll Cardiol. 2006 Feb 21;47(4):799–803
  37. McLaughlin VV, Presberg KW, Doyle RL, Abman SH, McCrory DC, Fortin T, et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004 Jul;126(1 Suppl.):78S–92S
  38. Raeside DA, Smith A, Brown A, Patel KR, Madhok R, Cleland J, et al. Pulmonary artery pressure measurement during exercise testing in patients with suspected pulmonary hypertension. Eur Respir J. 2000 Aug;16(2):282–287
  39. Rajasekhar D, Balakrishnan KG, Venkitachalam CG, Tharakan JA, Titus T, Subramanian R, et al. Primary pulmonary hypertension: natural history and prognostic factors. Indian Heart J. 1994 May–Jun;46(3):165–170
  40. Davis KK, Lilienfeld DE, Doyle RL. Increased mortality in African Americans with idiopathic pulmonary arterial hypertension. J Natl Med Assoc. 2008 Jan;100(1):69–72
  41. Braman SS, Eby E, Kuhn C, Rounds S. Primary pulmonary hypertension in the elderly. Arch Intern Med. 1991 Dec;151(12):2433–2438
  42. Dolara A, Camerini F, Menotti A, Thiene G. Primary pulmonary hypertension: an Italian multicenter study. A retrospective epidemiological survey in the period 1975–1985. G Ital Cardiol. 1988 Feb;18(2):115–120
  43. Barst RJ, Galie N, Naeije R, Simonneau G, Jeffs R, Arneson C, et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J. 2006 Dec;28(6):1195–1203
  44. Jing ZC, Xu XQ, Han ZY, Wu Y, Deng KW, Wang H, et al. Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest. 2007 Aug;132(2):373–379
  45. Kanemoto N, Sasamoto H. Arrhythmias in primary pulmonary hypertension. Jpn Heart J. 1979;20(6):765–775
  46. Rich S, Levy PS. Characteristics of surviving and nonsurviving patients with primary pulmonary hypertension. Am J Med. 1984 Apr;76(4):573–578
  47. Eysmann SB, Palevsky HI, Reichek N, Hackney K, Douglas PS. Two-dimensional and Doppler-echocardiographic and cardiac catheterization correlates of survival in primary pulmonary hypertension. Circulation. 1989 Aug;80(2):353–360
  48. Ewert R, Wensel R, Opitz C, Habedank D, Lodziewski S, Hummel M, et al. Prognosis in patients with primary pulmonary hypertension awaiting lung transplantation. Transpl Proc. 2001 Nov-Dec;33(7–8):3574–3575
  49. Henkens IR, Van Wolferen SA, Gan CT, Boonstra A, Swenne CA, Twisk JW, et al. Relation of resting heart rate to prognosis in patients with idiopathic pulmonary arterial hypertension. Am J Cardiol. 2009 May 15;103(10):1451–1456
  50. Kanemoto N. Electrocardiogram in primary pulmonary hypertension. Eur J Cardiol. 1981;12(3–4):181–193
  51. Kanemoto N, Sasamoto H. P wave in primary pulmonary hypertension. Tokai J Exp Clin Med. 1979;4(4):265–272
  52. Kanemoto N, Sasamoto H. Pulmonary hemodynamics in primary pulmonary hypertension. Jpn Heart J. 1979 Jul;20(4):395–405
  53. Nakonechnicov S, Gabbasov Z, Chazova I, Popov E, Belenkov Y. Platelet aggregation in patients with primary pulmonary hypertension. Blood Coagul Fibrinolysis. 1996 Mar;7(2):225–227
  54. Shitrit D, Bendayan D, Bar-Gil-Shitrit A, Huerta M, Rudensky B, Fink G, et al. Significance of a plasma D-dimer test in patients with primary pulmonary hypertension. Chest. 2002 Nov;122(5):1674–1678
  55. Kawut SM, Horn EM, Berekashvili KK, Widlitz AC, Rosenzweig EB, Barst RJ. von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension. Chest. 2005 Oct;128(4):2355–2362
  56. Kielstein JT, Bode-Boger SM, Hesse G, Martens-Lobenhoffer J, Takacs A, Fliser D, et al. Asymmetrical dimethylarginine in idiopathic pulmonary arterial hypertension. Arterioscler Thromb Vasc Biol. 2005 Jul;25(7):1414–1418
  57. Nagaya N, Uematsu M, Satoh T, Kyotani S, Sakamaki F, Nakanishi N, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med. 1999 Aug;160(2):487–492
  58. Bendayan D, Shitrit D, Ygla M, Huerta M, Fink G, Kramer MR. Hyperuricemia as a prognostic factor in pulmonary arterial hypertension. Respir Med. 2003 Feb;97(2):130–133
  59. Ogawa Y, Nishimura T, Hayashida K, Uehara T, Shimonagata T. Perfusion lung scintigraphy in primary pulmonary hypertension. Br J Radiol. 1993 Aug;66(788):677–680
  60. Sakamaki F, Satoh T, Nagaya N, Kyotani S, Oya H, Nakanishi N, et al. Correlation between severity of pulmonary arterial hypertension and 123I-metaiodobenzylguanidine left ventricular imaging. J Nucl Med. 2000 Jul;41(7):1127–1133
  61. Quarck R, Nawrot T, Meyns B, Delcroix M. C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Apr 7;53(14):1211–1218
  62. Hinderliter AL, PWt Willis, Long W, Clarke WR, Ralph D, Caldwell EJ, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Primary pulmonary hypertension. Am J Cardiol. 1999 Aug 15;84(4):481–484A10
  63. Tei C, Dujardin KS, Hodge DO, Bailey KR, McGoon MD, Tajik AJ, et al. Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr. 1996 Nov–Dec;9(6):838–847
  64. Shigematsu Y, Hamada M, Kokubu T. Significance of systolic time intervals in predicting prognosis of primary pulmonary hypertension. J Cardiol. 1988 Dec;18(4):1109–1114
  65. Loogen F, Worth H, Schwan G, Goeckenjan G, Losse B, Horstkotte D. Long-term follow-up of pulmonary hypertension in patients with and without anorectic drug intake. Cor Vasa. 1985;27(2–3):111–124
  66. Rodes-Cabau J, Domingo E, Roman A, Majo J, Lara B, Padilla F, et al. Intravascular ultrasound of the elastic pulmonary arteries: a new approach for the evaluation of primary pulmonary hypertension. Heart. 2003 Mar;89(3):311–315
  67. Raffy O, Azarian R, Brenot F, Parent F, Sitbon O, Petitpretz P, et al. Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension. Circulation. 1996 Feb 1;93(3):484–488

☆ This work was performed at the University of British Columbia and the University of Toronto.

PII: S0954-6111(10)00348-3

doi: 10.1016/j.rmed.2010.08.003

Respiratory Medicine
Volume 104, Issue 11 , Pages 1588-1607 , November 2010

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