Journal of the American College of Cardiology
Volume 54, Issue 1, Supplement , Pages S43-S54 , 30 June 2009

Updated Clinical Classification of Pulmonary Hypertension

  • Gérald Simonneau, MD

      Affiliations

    • Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud Hôpital Antoine Béclère, Clamart, France
    • Corresponding Author InformationReprint requests and correspondence: Dr. Gérald Simonneau, Centre National de Référence des Maladies Vasculaires Pulmonaires, Service de Pneumologie, Hôpital Antoine Béclère, Assistance Publique, Hôpitaux de Paris, Université Paris-Sud (XI), 157, rue de la Porte de Trivaux, 92140 Clamart, France
    • ,
  • Ivan M. Robbins, MD

      Affiliations

    • Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
    • ,
  • Maurice Beghetti, MD

      Affiliations

    • Pediatric Cardiology Unit, Hôpital des Enfants, University Hospital of Geneva, Geneva, Switzerland
    • ,
  • Richard N. Channick, MD

      Affiliations

    • Division of Pulmonary and Critical Care Medicine, UCSD Medical Center, La Jolla, California
    • ,
  • Marion Delcroix, MD, PhD

      Affiliations

    • Center for Pulmonary Vascular Disease, Department of Pneumology, Gasthuisberg University Hospital, Leuven, Belgium
    • ,
  • Christopher P. Denton, MD, PhD

      Affiliations

    • Centre for Rheumatology, Royal Free Hospital, London, United Kingdom
    • ,
  • C. Gregory Elliott, MD

      Affiliations

    • Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, Utah
    • ,
  • Sean P. Gaine, MD, PhD

      Affiliations

    • Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland
    • ,
  • Mark T. Gladwin, MD

      Affiliations

    • Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, Pennsylvania
    • ,
  • Zhi-Cheng Jing, MD

      Affiliations

    • Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
    • ,
  • Michael J. Krowka, MD

      Affiliations

    • Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
    • ,
  • David Langleben, MD

      Affiliations

    • Center for Pulmonary Vascular Disease, Sir Mortimer B. Davis Jewish General Hospital, Montréal, Québec, Canada
    • ,
  • Norifumi Nakanishi, MD, PhD

      Affiliations

    • Division of Cardiology and Pulmonary Circulation, Department of Internal Medicine National Cardiovascular Center, Osaka, Japan
    • ,
  • Rogério Souza, MD

      Affiliations

    • Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil

Received 6 February 2009 ,Accepted 15 April 2009.

References 

  1. Hatano S, Strasser T. Primary Pulmonary Hypertension (Report on a WHO Meeting). Geneva: World Health Organization; 1975;October 15–17, 1973
  2. Wagenvoort CA, Wagenvoort N. Primary pulmonary hypertension: a pathologic study of the lung vessels in 156 clinically diagnosed cases. Circulation. 1970;42:1163–1184
  3. Fishman AP. Clinical classification of pulmonary hypertension. Clin Chest Med. 2001;22:385–391
  4. Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension clinical and hemodynamic study. Am J Med. 1951;11:686–705
  5. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S
  6. Cogan JD, Pauciulo MW, Batchman AP, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174:590–598
  7. Aldred MA, Vijayakrishnan J, James V, et al. BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. Hum Mutat. 2006;27:212–213
  8. Sztrymf B, Coulet F, Girerd B, et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008;177:1377–1383
  9. Elliott CG, Glissmeyer EW, Havlena GT, et al. Relationship of BMPR2 mutations to vasoreactivity in pulmonary arterial hypertension. Circulation. 2006;113:2509–2515
  10. Rosenzweig EB, Morse JH, Knowles JA, et al. Clinical implications of determining BMPR2 mutation status in a large cohort of children and adults with pulmonary arterial hypertension. J Heart Lung Transplant. 2008;27:668–674
  11. Machado RD, Aldred MA, James V, et al. Mutations of the TGF-β type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006;27:121–132
  12. Thomson JR, Machado RD, Pauciulo MW, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-β family. J Med Genet. 2000;37:741–745
  13. Chaouat A, Coulet F, Favre C, et al. Endoglin germline mutation in a patient with hereditary haemorrhagic telangiectasia and dexfenfluramine associated pulmonary arterial hypertension. Thorax. 2004;59:446–448
  14. Trembath RC, Thomson JR, Machado RD, et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2001;345:325–334
  15. McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:14S–34S
  16. Souza R, Humbert M, Sztrymf B, et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J. 2008;31:343–348
  17. Walker AM, Langleben D, Korelitz JJ, et al. Temporal trends and drug exposures in pulmonary hypertension: an American experience. Am Heart J. 2006;152:521–526
  18. Chambers CD, Hernandez-Diaz S, Van Marter LJ, et al. Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. N Engl J Med. 2006;354:579–587
  19. Chin KM, Channick RN, Rubin LJ. Is methamphetamine use associated with idiopathic pulmonary arterial hypertension?. Chest. 2006;130:1657–1663
  20. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005;52:3792–3800
  21. Mukerjee D, St George D, Coleiro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62:1088–1093
  22. Launay D, Mouthon L, Hachulla E, et al. Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol. 2007;34:1005–1011
  23. de Groote P, Gressin V, Hachulla E, et al. Evaluation of cardiac abnormalities by Doppler echocardiography in a large nationwide multicentric cohort of patients with systemic sclerosis. Ann Rheum Dis. 2008;67:31–36
  24. Meune C, Avouac J, Wahbi K, et al. Cardiac involvement in systemic sclerosis assessed by tissue-doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum. 2008;58:1803–1809
  25. Tanaka E, Harigai M, Tanaka M, Kawaguchi Y, Hara M, Kamatani N. Pulmonary hypertension in systemic lupus erythematosus: evaluation of clinical characteristics and response to immunosuppressive treatment. J Rheumatol. 2002;29:282–287
  26. Asherson RA, Higenbottam TW, Dinh Xuan AT, Khamashta MA, Hughes GR. Pulmonary hypertension in a lupus clinic: experience with twenty-four patients. J Rheumatol. 1990;17:1292–1298
  27. Burdt MA, Hoffman RW, Deutscher SL, Wang GS, Johnson JC, Sharp GC. Long-term outcome in mixed connective tissue disease: longitudinal clinical and serologic findings. Arthritis Rheum. 1999;42:899–909
  28. Jaïs X, Launay D, Yaici A, et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum. 2008;58:521–531
  29. Launay D, Hachulla E, Hatron PY, Jaïs X, Simonneau G, Humbert M. Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore). 2007;86:299–315
  30. Bunch TW, Tancredi RG, Lie JT. Pulmonary hypertension in polymyositis. Chest. 1981;79:105–107
  31. Dawson JK, Goodson NG, Graham DR, Lynch MP. Raised pulmonary artery pressures measured with Doppler echocardiography in rheumatoid arthritis patients. Rheumatology (Oxford). 2000;39:1320–1325
  32. Kim KK, Factor SM. Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome. Hum Pathol. 1987;18:1293–1296
  33. Mehta NJ, Khan IA, Mehta RN, Sepkowitz DA. HIV-related pulmonary hypertension: analytic review of 131 cases. Chest. 2000;118:1133–1141
  34. Opravil M, Pechère M, Speich R, et al. HIV-associated primary pulmonary hypertension: a case control study (Swiss HIV Cohort Study). Am J Respir Crit Care Med. 1997;155:990–995
  35. 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;177:108–113
  36. Nunes H, Humbert M, Sitbon O, et al. Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2003;167:1433–1439
  37. Sitbon O, Gressin V, Speich R, et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2004;170:1212–1217
  38. Degano B, Yaïci A, Le Pavec J, et al. Long-term effects of bosentan in patients with HIV-associated pulmonary arterial hypertension. Eur Respir J. 2008;33:92–98
  39. Hervé P, Lebrec D, Brenot F, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J. 1998;11:1153–1166
  40. Rodríguez-Roisin R, Krowka MJ, Hervé P, Fallon MB ERS Task Force Pulmonary-Hepatic Vascular Disorders Scientific Committee. Pulmonary-hepatic vascular disorders. Eur Respir J. 2004;24:861–880
  41. Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991;100:520–528
  42. Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: results from a 10-year screening algorithm. Hepatology. 2006;44:1502–1510
  43. Kawut SM, Krowka MJ, Trotter JF, et al. Clinical risk factors for portopulmonary hypertension. Hepatology. 2008;48:196–203
  44. Le Pavec J, Souza R, Herve P, et al. Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med. 2008;178:637–643
  45. Eisenmenger V. Die angeborene defecte der kammersheidewand das herzen. Z Klin Med. 1897;132:131
  46. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958;2:701–709
  47. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome: factors relating to deterioration and death. Eur Heart J. 1998;19:1845–1855
  48. Besterman E. Atrial septal defect with pulmonary hypertension. Br Heart J. 1961;23:587–598
  49. Hoffman JI, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965;16:634–653
  50. Galiè N, Manes A, Palazzini M, et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome. Drugs. 2008;68:1049–1066
  51. Shaw AP, Ghareed A. The pathogenesis of pulmonary schistosomiasis in Egypt with special reference to Ayerza's disease. J Pathol Bacteriol. 1938;46:401–424
  52. Lapa MS, Ferreira EV, Jardim C, Martins Bdo C, Arakaki JS, Souza R. Clinical characteristics of pulmonary hypertension patients in two reference centers in the city of Sao Paulo. Rev Assoc Med Bras. 2006;52:139–143
  53. Chaves E. The pathology of the arterial pulmonary vasculature in Manson's schistosomiasis. Chest. 1966;50:72–77
  54. de Cleva R, Herman P, Pugliese V, et al. Prevalence of pulmonary hypertension in patients with hepatosplenic Mansonic schistosomiasis—prospective study. Hepatogastroenterology. 2003;50:2028–2030
  55. Lapa M, Dias B, Jardim C, et al. Cardio-pulmonary manifestations of hepatosplenic schistosomiasis. Circulation. 2009;119:1518–1523
  56. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003;101:1257–1261
  57. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004;350:886–895
  58. Aessopos A, Stamatelos G, Skoumas V, Vassilopoulos G, Mantzourani M, Loukopoulos D. Pulmonary hypertension and right heart failure in patients with β-thalassemia intermedia. Chest. 1995;107:50–53
  59. Smedema JP, Louw VJ. Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. Cardiovasc J Afr. 2007;18:84–89
  60. Jaïs X, Till SJ, Cynober T, et al. An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation. Hemoglobin. 2003;27:139–147
  61. Stuard ID, Heusinkveld RS, Moss AJ. Microangiopathic hemolytic anemia and thrombocytopenia in primary pulmonary hypertension. N Engl J Med. 1972;287:869–870
  62. Haque AK, Gokhale S, Rampy BA, Adegboyega P, Duarte A, Saldana MJ. Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases. Hum Pathol. 2002;33:1037–1043
  63. Parent F, Egels S, Stzrymf B, et al. Haemodynamic characteristics of patients with sickle cell disease and suspected pulmonary hypertension on the basis of a tricuspid regurgitation jet velocity >2.5 m/s on doppler echocardiography. Eur Respir J. 2006;28:544s
  64. Anthi A, Machado RF, Jison ML, et al. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. Am J Respir Crit Care Med. 2007;175:1272–1279
  65. Reiter CD, Wang X, Tanus-Santos JE, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med. 2002;8:1383–1389
  66. Gladwin MT, Lancaster JR, Freeman BA, Schechter AN. Nitric oxide's reactions with hemoglobin: a view through the SNO-storm. Nat Med. 2003;9:496–500
  67. Montani D, Price LC, Dorfmüller P, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33:189–200
  68. Dorfmüller P, Humbert M, Perros F, et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol. 2007;38:893–902
  69. Escamilla R, Hermant C, Berjaud J, Mazerolles C, Daussy X. Pulmonary veno-occlusive disease in a HIV-infected intravenous drug abuser. Eur Respir J. 1995;8:1982–1984
  70. Ruchelli ED, Nojadera G, Rutstein RM, Rudy B. Pulmonary veno-occlusive disease: another vascular disorder associated with human immunodeficiency virus infection?. Arch Pathol Lab Med. 1994;118:664–666
  71. Montani D, Achouh L, Dorfmüller P, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87:220–233
  72. Runo JR, Vnencak-Jones CL, Prince M, et al. Pulmonary veno-occlusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003;167:889–894
  73. Lantuejoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. Am J Surg Pathol. 2006;30:850–857
  74. Resten A, Maitre S, Humbert M, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 2004;183:65–70
  75. Holcomb BW, Loyd JE, Ely EW, Johnson J, Robbins IM. Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000;118:1671–1679
  76. Dufour B, Maitre S, Humbert M, Capron F, Simonneau G, Musset D. High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. AJR Am J Roentgenol. 1998;171:1321–1324
  77. Rabiller A, Jaïs X, Hamid A, et al. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J. 2006;27:108–113
  78. Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med. 2007;28:233–241
  79. Costard-Jackle A, Fowler MB. Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol. 1992;19:48–54
  80. Abramson SV, Burke JF, Kelly JJ, et al. Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann Intern Med. 1992;116:888–895
  81. Zener JC, Hancock EW, Shumway NE, Harrison DC. Regression of extreme pulmonary hypertension after mitral valve surgery. Am J Cardiol. 1972;30:820–826
  82. Braunwald E, Braunwald NS, Ross J, Morrow AG. Effects of mitral-valve replacement on the pulmonary vascular dynamics of patients with pulmonary hypertension. N Engl J Med. 1965;273:509–514
  83. Delgado JF, Conde E, Sánchez V, et al. Pulmonary vascular remodeling in pulmonary hypertension due to chronic heart failure. Eur J Heart Fail. 2005;7:1011–1016
  84. Moraes DL, Colucci WS, Givertz MM. Secondary pulmonary hypertension in chronic heart failure: the role of the endothelium in pathophysiology and management. Circulation. 2000;102:1718–1723
  85. Fraser KL, Tullis DE, Sasson Z, Hyland RH, Thornley KS, Hanly PJ. Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia. Chest. 1999;115:1321–1328
  86. Cottin V, Nunes H, Brillet PY, et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005;26:586–593
  87. Weitzenblum E, Hirth C, Ducolone A, Mirhom R, Rasaholinjanahary J, Ehrhart M. Prognostic value of pulmonary artery pressure in chronic obstructive pulmonary disease. Thorax. 1981;36:752–758
  88. Thabut G, Dauriat G, Stern JB, et al. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest. 2005;127:1531–1536
  89. Chaouat A, Bugnet AS, Kadaoui N, et al. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005;172:189–194
  90. Tapson VF, Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006;3:564–567
  91. Pengo V, Lensing AW, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350:2257–2264
  92. Kim NH. Assessment of operability in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006;3:584–588
  93. Dartevelle P, Fadel E, Mussot S, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004;23:637–648
  94. Jamieson SW, Kapelanski DP, Sakakibara N, et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg. 2003;76:1457–1462
  95. Suntharalingam J, Treacy CM, Doughty NJ, et al. Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest. 2008;134:229–236
  96. Jaïs X, D'Armini AM, Jansa P, et al. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension; BENEFiT (bosentan effects in inoperable forms of chronic thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol. 2008;16:2127–2134
  97. Rubin LJ, Hoeper MM, Klepetko W, Galiè N, Lang IM, Simonneau G. Current and future management of chronic thromboembolic pulmonary hypertension: from diagnosis to treatment responses. Proc Am Thorac Soc. 2006;3:601–607
  98. Dingli D, Utz JP, Krowka MJ, Oberg AL, Tefferi A. Unexplained pulmonary hypertension in chronic myeloproliferative disorders. Chest. 2001;120:801–808
  99. Marvin KS, Spellberg RD. Pulmonary hypertension secondary to thrombocytosis in a patient with myeloid metaplasia. Chest. 1993;103:642–644
  100. Nand S, Orfei E. Pulmonary hypertension in polycythemia vera. Am J Hematol. 1994;47:242–244
  101. Peacock AJ. Pulmonary hypertension after splenectomy: a consequence of loss of the splenic filter or is there something more?. Thorax. 2005;60:983–984
  102. Guilpain P, Montani D, Damaj G, et al. Pulmonary hypertension associated with myeloproliferative disorders: a retrospective study of ten cases. Respiration. 2008;76:295–302
  103. Jaïs X, Ioos V, Jardim C, et al. Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax. 2005;60:1031–1034
  104. Hoeper MM, Niedermeyer J, Hoffmeyer F, Flemming P, Fabel H. Pulmonary hypertension after splenectomy?. Ann Intern Med. 1999;130:506–509
  105. Handa T, Nagai S, Miki S, et al. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006;129:1246–1252
  106. Gluskowski J, Hawrylkiewicz I, Zych D, Wojtczak A, Zielinski J. Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis. Respiration. 1984;46:26–32
  107. Shorr AF, Helman DL, Davies DB, Nathan SD. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J. 2005;25:783–788
  108. Bourbonnais JM, Samavati L. Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J. 2008;32:296–302
  109. Nunes H, Humbert M, Capron F, et al. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006;61:68–74
  110. Dauriat G, Mal H, Thabut G, et al. Lung transplantation for pulmonary Langerhans' cell histiocytosis: a multicenter analysis. Transplantation. 2006;81:746–750
  111. Fartoukh M, Humbert M, Capron F, et al. Severe pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med. 2000;161:216–223
  112. Taveira-DaSilva AM, Hathaway OM, Sachdev V, Shizukuda Y, Birdsall CW, Moss J. Pulmonary artery pressure in lymphangioleiomyomatosis: an echocardiographic study. Chest. 2007;132:1573–1578
  113. Harari S, Simonneau G, De Juli E, et al. Prognostic value of pulmonary hypertension in patients with chronic interstitial lung disease referred for lung or heart-lung transplantation. J Heart Lung Transplant. 1997;16:460–463
  114. Simeoni S, Puccetti A, Chilosi M, et al. Type 1 neurofibromatosis complicated by pulmonary artery hypertension: a case report. J Med Invest. 2007;54:354–358
  115. Engel PJ, Baughman RP, Menon SG, Kereiakes DJ, Taylor L, Scott M. Pulmonary hypertension in neurofibromatosis. Am J Cardiol. 2007;99:1177–1178
  116. Aoki Y, Kodama M, Mezaki T, et al. von Recklinghausen disease complicated by pulmonary hypertension. Chest. 2001;119:1606–1608
  117. Samuels N, Berkman N, Milgalter E, Bar-Ziv J, Amir G, Kramer MR. Pulmonary hypertension secondary to neurofibromatosis: intimal fibrosis versus thromboembolism. Thorax. 1999;54:858–859
  118. Stewart DR, Cogan JD, Kramer MR, et al. Is pulmonary arterial hypertension in neurofibromatosis type 1 secondary to a plexogenic arteriopathy?. Chest. 2007;132:798–808
  119. Launay D, Souza R, Guillevin L, et al. Pulmonary arterial hypertension in ANCA-associated vasculitis. Sarcoidosis Vasc Diffuse Lung Dis. 2006;23:223–228
  120. Hamaoka K, Nakagawa M, Furukawa N, Sawada T. Pulmonary hypertension in type I glycogen storage disease. Pediatr Cardiol. 1990;11:54–56
  121. Inoue S, Nakamura T, Hasegawa K, et al. Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report. J Cardiol. 1989;19:323–332
  122. Humbert M, Labrune P, Sitbon O, et al. Pulmonary arterial hypertension and type-I glycogen-storage disease: the serotonin hypothesis. Eur Respir J. 2002;20:59–65
  123. Pizzo CJ. Type I glycogen storage disease with focal nodular hyperplasia of the liver and vasoconstrictive pulmonary hypertension. Pediatrics. 1980;65:341–343
  124. Elstein D, Klutstein MW, Lahad A, Abrahamov A, Hadas-Halpern I, Zimran A. Echocardiographic assessment of pulmonary hypertension in Gaucher's disease. Lancet. 1998;351:1544–1546
  125. Lee R, Yousem S. The frequency and type of lung involvement in patients with Gaucher's disease. Lab Invest. 1998;58:54A
  126. Theise ND, Ursell PC. Pulmonary hypertension and Gaucher's disease: logical association or mere coincidence?. Am J Pediatr Hematol Oncol. 1990;12:74–76
  127. Li JH, Safford RE, Aduen JF, Heckman MG, Crook JE, Burger CD. Pulmonary hypertension and thyroid disease. Chest. 2007;132:793–797
  128. Ferris A, Jacobs T, Widlitz A, Barst RJ, Morse JH. Pulmonary arterial hypertension and thyroid disease. Chest. 2001;119:1980–1981
  129. Mercé J, Ferrás S, Oltra C, et al. Cardiovascular abnormalities in hyperthyroidism: a prospective Doppler echocardiographic study. Am J Med. 2005;118:126–131
  130. Kokturk N, Demir N, Demircan S, et al. Pulmonary veno-occlusive disease in a patient with a history of Hashimoto's thyroiditis. Indian J Chest Dis Allied Sci. 2005;47:289–292
  131. Chu JW, Kao PN, Faul JL, Doyle RL. High prevalence of autoimmune thyroid disease in pulmonary arterial hypertension. Chest. 2002;122:1668–1673
  132. Mayer E, Kriegsmann J, Gaumann A, et al. Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2001;121:77–82
  133. Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg. 1995;59:1487–1490
  134. Kim HK, Choi YS, Kim K, et al. Surgical treatment for pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2008;33:712–716
  135. Ishiguro T, Kasahara K, Matsumoto I, et al. Primary pulmonary artery sarcoma detected with a pulmonary infarction. Intern Med. 2007;46:601–604
  136. Roberts KE, Hamele-Bena D, Saqi A, Stein CA, Cole RP. Pulmonary tumor embolism: a review of the literature. Am J Med. 2003;115:228–232
  137. Dot JM, Sztrymf B, Yaici A, et al. Pulmonary arterial hypertension due to tumor emboli. Rev Mal Respir. 2007;24:359–366
  138. Davis AM, Pierson RN, Loyd JE. Mediastinal fibrosis. Semin Respir Infect. 2001;16:119–130
  139. Loyd JE, Tillman BF, Atkinson JB, Des Prez RM. Mediastinal fibrosis complicating histoplasmosis. Medicine (Baltimore). 1988;67:295–310
  140. Goodwin RA, Nickell JA, Des Prez RM. Mediastinal fibrosis complicating healed primary histoplasmosis and tuberculosis. Medicine (Baltimore). 1972;51:227–246
  141. Yigla M, Nakhoul F, Sabag A, et al. Pulmonary hypertension in patients with end-stage renal disease. Chest. 2003;123:1577–1582
  142. Nakhoul F, Yigla M, Gilman R, Reisner SA, Abassi Z. The pathogenesis of pulmonary hypertension in haemodialysis patients via arterio-venous access. Nephrol Dial Transplant. 2005;20:1686–1692

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PII: S0735-1097(09)01216-9

doi: 10.1016/j.jacc.2009.04.012

Journal of the American College of Cardiology
Volume 54, Issue 1, Supplement , Pages S43-S54 , 30 June 2009

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