Skip to main content
Top
Published in: Current Hypertension Reports 6/2013

01-12-2013 | Pulmonary Hypertension (Z-C Jing, Section Editor)

Epidemiology of Pulmonary Arterial Hypertension

Authors: Xin Jiang, Zhi-Cheng Jing

Published in: Current Hypertension Reports | Issue 6/2013

Login to get access

Abstract

The epidemiology of pulmonary arterial hypertension (PAH) has changed over the last decade. Remarkable advances in understanding the pathobiology and clinical care required in PAH have resulted in improved quality of life and survival. Despite such important progress, the long-term rate of survival is still unacceptable. The epidemiology of PAH could not be easily generalized globally, due to the fact that nearly all of the present data has been gathered from Western, multicenter, prospective registries. There are potentially marked differences in PAH patients from Western and Eastern populations, and from developed and developing countries. Therefore, it is clear that more registry data will be needed to address novel questions emerging with improved knowledge of PAH.
Literature
1.
go back to reference Galiè N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J. 2010;31(17):2080–6.PubMedCrossRef Galiè N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J. 2010;31(17):2080–6.PubMedCrossRef
2.
go back to reference Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest. 2012;122(12):4306–13.PubMedCrossRef Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest. 2012;122(12):4306–13.PubMedCrossRef
3.
go back to reference McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 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;53(17):1573–619.PubMedCrossRef McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 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;53(17):1573–619.PubMedCrossRef
4.
go back to reference • Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30(20):2493–537. ESC/ERS/ISHLT guidelines for the diagnosis and treatment of pulmonary hypertension.PubMedCrossRef • Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30(20):2493–537. ESC/ERS/ISHLT guidelines for the diagnosis and treatment of pulmonary hypertension.PubMedCrossRef
5.
go back to reference •• Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30(4):394–403. The first meta-analysis of randomized controlled trials performed in patients with PAH suggesting an improvement of survival in the patients treated with the targeted therapies approved for PAH.PubMedCrossRef •• Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30(4):394–403. The first meta-analysis of randomized controlled trials performed in patients with PAH suggesting an improvement of survival in the patients treated with the targeted therapies approved for PAH.PubMedCrossRef
6.
go back to reference •• Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216–23. This first prospective and nationwide registry demonstrated the baseline characteristics of primary pulmonary hypertension.PubMedCrossRef •• Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987;107(2):216–23. This first prospective and nationwide registry demonstrated the baseline characteristics of primary pulmonary hypertension.PubMedCrossRef
7.
go back to reference •• 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;115(5):343–9. The prognostic data in the primary NIH pulmonary hypertension cohort revealed a disastrous long-term survival rate when patients only received conventional therapies, with a median survival rate of only 2.8 years after diagnosis.PubMedCrossRef •• 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;115(5):343–9. The prognostic data in the primary NIH pulmonary hypertension cohort revealed a disastrous long-term survival rate when patients only received conventional therapies, with a median survival rate of only 2.8 years after diagnosis.PubMedCrossRef
8.
go back to reference •• 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;173(9):1023–30. This registry represents the largest population of patients with PAH since the availability of a novel diagnostic classification of the condition and the development of disease-specific therapies, allowing an updated description of this orphan disease in the Western world.PubMedCrossRef •• 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;173(9):1023–30. This registry represents the largest population of patients with PAH since the availability of a novel diagnostic classification of the condition and the development of disease-specific therapies, allowing an updated description of this orphan disease in the Western world.PubMedCrossRef
9.
go back to reference • Humbert M, Sitbon O, Yaïci A, Montani D, O'Callaghan DS, Jaïs X, et al. French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;36(3):549–55. The first study to reveal a better survival in incident PAH patients compared with those having prevalent PAH.PubMedCrossRef • Humbert M, Sitbon O, Yaïci A, Montani D, O'Callaghan DS, Jaïs X, et al. French Pulmonary Arterial Hypertension Network. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J. 2010;36(3):549–55. The first study to reveal a better survival in incident PAH patients compared with those having prevalent PAH.PubMedCrossRef
10.
go back to reference • Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156–63. This study demonstrated that idiopathic, familial, and anorexigen-associated PAH remains a progressive, fatal disease in the modern management era, with a 3-year rate of survival of only 54.9 % in incident cases.PubMedCrossRef • Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation. 2010;122(2):156–63. This study demonstrated that idiopathic, familial, and anorexigen-associated PAH remains a progressive, fatal disease in the modern management era, with a 3-year rate of survival of only 54.9 % in incident cases.PubMedCrossRef
11.
go back to reference • Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376–87. Baseline characteristics that provided important information on WHO group 1 PAH in the largest PAH registry.PubMedCrossRef • Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137(2):376–87. Baseline characteristics that provided important information on WHO group 1 PAH in the largest PAH registry.PubMedCrossRef
12.
go back to reference 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;139(1):128–37.PubMedCrossRef 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;139(1):128–37.PubMedCrossRef
13.
go back to reference 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;142(2):448–56.PubMedCrossRef 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;142(2):448–56.PubMedCrossRef
14.
go back to reference Escribano-Subias P, Blanco I, López-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J. 2012;40(3):596–603.PubMedCrossRef Escribano-Subias P, Blanco I, López-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J. 2012;40(3):596–603.PubMedCrossRef
15.
go back to reference Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–6.PubMedCrossRef Ling Y, Johnson MK, Kiely DG, Condliffe R, Elliot CA, Gibbs JS, et al. Changing demographics, epidemiology, and survival of incident pulmonary arterial hypertension: results from the pulmonary hypertension registry of the United Kingdom and Ireland. Am J Respir Crit Care Med. 2012;186(8):790–6.PubMedCrossRef
16.
go back to reference Hoeper MM, Huscher D, Ghofrani HA, Delcroix M, Distler O, Schweiger C, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2012 Nov 16. Hoeper MM, Huscher D, Ghofrani HA, Delcroix M, Distler O, Schweiger C, et al. Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol. 2012 Nov 16.
17.
go back to reference Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med. 1951;11(6):686–705.PubMedCrossRef Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med. 1951;11(6):686–705.PubMedCrossRef
18.
go back to reference Loyd JE, Primm RK, Newman JH. Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis. 1984;129(1):194–7.PubMed Loyd JE, Primm RK, Newman JH. Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis. 1984;129(1):194–7.PubMed
19.
go back to reference Loyd JE, Butler MG, Foroud TM, Conneally PM, Phillips 3rd JA, Newman JH. Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Am J Respir Crit Care Med. 1995;152(1):93–7.PubMedCrossRef Loyd JE, Butler MG, Foroud TM, Conneally PM, Phillips 3rd JA, Newman JH. Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Am J Respir Crit Care Med. 1995;152(1):93–7.PubMedCrossRef
20.
go back to reference •• Nichols WC, Koller DL, Slovis B, Foroud T, Terry VH, Arnold ND, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32. Nat Genet. 1997;15(3):277–80. One of the first studies that uncovered the localization of the gene for FPAH.PubMedCrossRef •• Nichols WC, Koller DL, Slovis B, Foroud T, Terry VH, Arnold ND, et al. Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32. Nat Genet. 1997;15(3):277–80. One of the first studies that uncovered the localization of the gene for FPAH.PubMedCrossRef
21.
go back to reference •• Morse JH, Jones AC, Barst RJ, Hodge SE, Wilhelmsen KC, Nygaard TG. Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31-q32. Circulation. 1997;95(12):2603–6. One of the first studies that uncovered the localization of a gene for FPAH.PubMedCrossRef •• Morse JH, Jones AC, Barst RJ, Hodge SE, Wilhelmsen KC, Nygaard TG. Mapping of familial primary pulmonary hypertension locus (PPH1) to chromosome 2q31-q32. Circulation. 1997;95(12):2603–6. One of the first studies that uncovered the localization of a gene for FPAH.PubMedCrossRef
22.
go back to reference Lane KB, Machado RD, Pauciulo MW, Thomson JR, Phillips III JA, Loyd JE, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-b receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet. 2000;26(1):81–4. The first study that confirmed that BMPR2 mutation could cause familial primary pulmonary hypertension.PubMedCrossRef Lane KB, Machado RD, Pauciulo MW, Thomson JR, Phillips III JA, Loyd JE, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-b receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet. 2000;26(1):81–4. The first study that confirmed that BMPR2 mutation could cause familial primary pulmonary hypertension.PubMedCrossRef
23.
go back to reference Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000;67(3):737–44.PubMedCrossRef Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet. 2000;67(3):737–44.PubMedCrossRef
24.
go back to reference Machado RD, Eickelberg O, Elliott CG, Geraci MW, Hanaoka M, Loyd JE, et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1 suppl):S32–42.PubMedCrossRef Machado RD, Eickelberg O, Elliott CG, Geraci MW, Hanaoka M, Loyd JE, et al. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1 suppl):S32–42.PubMedCrossRef
25.
go back to reference • Sztrymf B, Coulet F, Girerd B, Yaici A, Jais X, Sitbon O, et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008;177(12):1377–83. The first study to reveal that the BMPR2 mutation carriers with PAH present approximately 10 years earlier than noncarriers, with a more severe hemodynamic compromise at diagnosis.PubMedCrossRef • Sztrymf B, Coulet F, Girerd B, Yaici A, Jais X, Sitbon O, et al. Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008;177(12):1377–83. The first study to reveal that the BMPR2 mutation carriers with PAH present approximately 10 years earlier than noncarriers, with a more severe hemodynamic compromise at diagnosis.PubMedCrossRef
26.
go back to reference Rosenzweig EB, Morse JH, Knowles JA, Knowles JA, Chada KK, Khan AM, 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(6):668–74.PubMedCrossRef Rosenzweig EB, Morse JH, Knowles JA, Knowles JA, Chada KK, Khan AM, 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(6):668–74.PubMedCrossRef
27.
go back to reference Aldred MA, Machado RD, James V, Morrell NW, Trembath RC. Characterization of the BMPR2 5’-untranslated region and a novel mutation in pulmonary hypertension. Am J Respir Crit Care Med. 2007;176(8):819–24.PubMedCrossRef Aldred MA, Machado RD, James V, Morrell NW, Trembath RC. Characterization of the BMPR2 5’-untranslated region and a novel mutation in pulmonary hypertension. Am J Respir Crit Care Med. 2007;176(8):819–24.PubMedCrossRef
28.
go back to reference Cogan JD, Pauciulo MW, Batchman AP, Prince MA, Robbins IM, Hedges LK, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174(5):590–8.PubMedCrossRef Cogan JD, Pauciulo MW, Batchman AP, Prince MA, Robbins IM, Hedges LK, et al. High frequency of BMPR2 exonic deletions/duplications in familial pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006;174(5):590–8.PubMedCrossRef
29.
go back to reference Machado RD, Aldred MA, James V, Harrison RE, Patel B, Schwalbe EC, et al. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006;27(2):121–32.PubMedCrossRef Machado RD, Aldred MA, James V, Harrison RE, Patel B, Schwalbe EC, et al. Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006;27(2):121–32.PubMedCrossRef
30.
go back to reference Girerd B, Montani D, Coulet F, Sztrymf B, Yaici A, Jaïs X, et al. Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation. Am J Respir Crit Care Med. 2010;181(8):851–61.PubMedCrossRef Girerd B, Montani D, Coulet F, Sztrymf B, Yaici A, Jaïs X, et al. Clinical outcomes of pulmonary arterial hypertension in patients carrying an ACVRL1 (ALK1) mutation. Am J Respir Crit Care Med. 2010;181(8):851–61.PubMedCrossRef
31.
go back to reference McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE, et al. Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 1994;8(4):345–51.PubMedCrossRef McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE, et al. Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 1994;8(4):345–51.PubMedCrossRef
32.
go back to reference Johnson DW, Berg JN, Baldwin MA, Gallione CJ, Marondel I, Yoon SJ, et al. Mutations in the activin receptorlike kinase 1 gene in hereditary haemorrhagic telangiectasia type 2. Nat Genet. 1996;13(2):189–95.PubMedCrossRef Johnson DW, Berg JN, Baldwin MA, Gallione CJ, Marondel I, Yoon SJ, et al. Mutations in the activin receptorlike kinase 1 gene in hereditary haemorrhagic telangiectasia type 2. Nat Genet. 1996;13(2):189–95.PubMedCrossRef
33.
go back to reference Chen YJ, Yang QH, Liu D, Liu QQ, Eyries M, Wen L, et al. Clinical and genetic characteristics of Chinese patients with hereditary haemorrhagic telangiectasia-associated pulmonary hypertension. Eur J Clin Invest. 2013 Jul 10. Chen YJ, Yang QH, Liu D, Liu QQ, Eyries M, Wen L, et al. Clinical and genetic characteristics of Chinese patients with hereditary haemorrhagic telangiectasia-associated pulmonary hypertension. Eur J Clin Invest. 2013 Jul 10.
34.
go back to reference Shintani M, Yagi H, Nakayama T, Saji T, Matsuoka R. A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension. J Med Genet. 2009;46(5):331–7.PubMedCrossRef Shintani M, Yagi H, Nakayama T, Saji T, Matsuoka R. A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension. J Med Genet. 2009;46(5):331–7.PubMedCrossRef
35.
go back to reference Austin ED, Ma L, LeDuc C, Berman Rosenzweig E, Borczuk A, Phillips 3rd JA, et al. Whole exome sequencing to identify a novel gene (caveolin-1) associated with human pulmonary arterial hypertension. Circ Cardiovasc Genet. 2012;5(3):336–43.PubMedCrossRef Austin ED, Ma L, LeDuc C, Berman Rosenzweig E, Borczuk A, Phillips 3rd JA, et al. Whole exome sequencing to identify a novel gene (caveolin-1) associated with human pulmonary arterial hypertension. Circ Cardiovasc Genet. 2012;5(3):336–43.PubMedCrossRef
36.
go back to reference • 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;369(4):351–61. The results of this study indicated that in some patients, the KCNK3 mutation might be regarded as a kind of channelopathy.PubMedCrossRef • 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;369(4):351–61. The results of this study indicated that in some patients, the KCNK3 mutation might be regarded as a kind of channelopathy.PubMedCrossRef
37.
go back to reference Liu D, Wu WH, Mao YM, Yuan P, Zhang R, Ju FL, et al. BMPR2 mutations influence phenotype more obviously in male patients with pulmonary arterial hypertension. Circ Cardiovasc Genet. 2012;5(5):511–8.PubMedCrossRef Liu D, Wu WH, Mao YM, Yuan P, Zhang R, Ju FL, et al. BMPR2 mutations influence phenotype more obviously in male patients with pulmonary arterial hypertension. Circ Cardiovasc Genet. 2012;5(5):511–8.PubMedCrossRef
38.
go back to reference • Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151–7. The first prospective, large-scale registry of CTD-PAH in the modern therapeutic era demonstrated that the survival of patients with SSc-PAH in the modern treatment era is better than in the historical series, and that SLE-PAH has a better prognosis than SSc-PAH.PubMedCrossRef • Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;179(2):151–7. The first prospective, large-scale registry of CTD-PAH in the modern therapeutic era demonstrated that the survival of patients with SSc-PAH in the modern treatment era is better than in the historical series, and that SLE-PAH has a better prognosis than SSc-PAH.PubMedCrossRef
39.
go back to reference Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild- moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol. 2006;33(2):269–74.PubMed Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild- moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol. 2006;33(2):269–74.PubMed
40.
go back to reference Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, et al. Isolated pulmonary hypertension in scleroderma. Intern Med J. 2005;35(1):28–33.PubMedCrossRef Cox SR, Walker JG, Coleman M, Rischmueller M, Proudman S, Smith MD, et al. Isolated pulmonary hypertension in scleroderma. Intern Med J. 2005;35(1):28–33.PubMedCrossRef
41.
go back to reference Hachulla E, Launay D, Mouthon L, Sitbon O, Berezne A, Guillevin L, et al. French PAH-SSc Network. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;136(5):1211–9.PubMedCrossRef Hachulla E, Launay D, Mouthon L, Sitbon O, Berezne A, Guillevin L, et al. French PAH-SSc Network. Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;136(5):1211–9.PubMedCrossRef
42.
go back to reference • Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138(6):1383–94. SSc-PAH was further identified as a unique phenotype due to much worse long-term prognosis compared with other forms of CTD-PAH.PubMedCrossRef • Chung L, Liu J, Parsons L, Hassoun PM, McGoon M, Badesch DB, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype. Chest. 2010;138(6):1383–94. SSc-PAH was further identified as a unique phenotype due to much worse long-term prognosis compared with other forms of CTD-PAH.PubMedCrossRef
43.
go back to reference Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;54(9):3043–50.PubMedCrossRef Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;54(9):3043–50.PubMedCrossRef
44.
go back to reference Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11):1088–93.PubMedCrossRef Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003;62(11):1088–93.PubMedCrossRef
45.
go back to reference Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123(2):344–50.PubMedCrossRef Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest. 2003;123(2):344–50.PubMedCrossRef
46.
go back to reference Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413–25.PubMedCrossRef Mathai SC, Hassoun PM. Pulmonary arterial hypertension in connective tissue diseases. Heart Fail Clin. 2012;8(3):413–25.PubMedCrossRef
47.
go back to reference Yang X, Mardekian J, Sanders KN, Mychaskiw MA, Thomas J 3rd. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clin Rheumatol. 2013 Jun 20. Yang X, Mardekian J, Sanders KN, Mychaskiw MA, Thomas J 3rd. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clin Rheumatol. 2013 Jun 20.
48.
go back to reference Prabu A, Patel K, Yee CS, Nightingale P, Situnayake RD, Thickett DR, et al. Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford). 2009;48(12):1506–11.CrossRef Prabu A, Patel K, Yee CS, Nightingale P, Situnayake RD, Thickett DR, et al. Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus. Rheumatology (Oxford). 2009;48(12):1506–11.CrossRef
49.
go back to reference Lau CS, Yin G, Mok MY. Ethnic and geographical differences in systemic lupus erythematosus: an overview. Lupus. 2006;15(11):715–9.PubMedCrossRef Lau CS, Yin G, Mok MY. Ethnic and geographical differences in systemic lupus erythematosus: an overview. Lupus. 2006;15(11):715–9.PubMedCrossRef
50.
go back to reference •• Jiang X, Humbert M, Jing ZC. Idiopathic pulmonary arterial hypertension and its prognosis in the modern management era in developed and developing countries. In: Humbert M, Souza R, Simonneau G, editors. Pulmonary Vascular Disorders. Prog Respir Res. Basel: Karger; 2012;85–93. The first description of the similarities and differences of baseline characteristics in Chinese PAH patients compared with those data from Western registries. •• Jiang X, Humbert M, Jing ZC. Idiopathic pulmonary arterial hypertension and its prognosis in the modern management era in developed and developing countries. In: Humbert M, Souza R, Simonneau G, editors. Pulmonary Vascular Disorders. Prog Respir Res. Basel: Karger; 2012;85–93. The first description of the similarities and differences of baseline characteristics in Chinese PAH patients compared with those data from Western registries.
51.
go back to reference Jeon CH, Chai JY, Seo YI, Jun JB, Koh EM, Lee SK. Pulmonary hypertension study group of Korean College of Rheumatology. Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the Korean registry. Int J Rheum Dis. 2012;15(5):e80–9.PubMedCrossRef Jeon CH, Chai JY, Seo YI, Jun JB, Koh EM, Lee SK. Pulmonary hypertension study group of Korean College of Rheumatology. Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the Korean registry. Int J Rheum Dis. 2012;15(5):e80–9.PubMedCrossRef
52.
go back to reference Gunnarsson R, Andreassen AK, Molberg Ø, Lexberg ÅS, Time K, Dhainaut AS, et al. Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature. Rheumatology (Oxford). 2013;52(7):1208–13.CrossRef Gunnarsson R, Andreassen AK, Molberg Ø, Lexberg ÅS, Time K, Dhainaut AS, et al. Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature. Rheumatology (Oxford). 2013;52(7):1208–13.CrossRef
53.
go back to reference Jais X, Launay D, Yaici A, Le Pavec J, Tchérakian C, Sitbon O, 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(2):521–31.PubMedCrossRef Jais X, Launay D, Yaici A, Le Pavec J, Tchérakian C, Sitbon O, 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(2):521–31.PubMedCrossRef
54.
go back to reference Launay D, Hachulla E, Hatron PY, Jais 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(5):299–315.CrossRef Launay D, Hachulla E, Hatron PY, Jais 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(5):299–315.CrossRef
55.
go back to reference van Loon RL, Roofthooft MT, Hillege HL, ten Harkel AD, van Osch-Gevers M, Delhaas T, et al. Pediatric pulmonary hypertension in the Netherlands. Epidemiology and characterization during the period 1991 to 2005. Circulation. 2011;124(16):1755–64.PubMedCrossRef van Loon RL, Roofthooft MT, Hillege HL, ten Harkel AD, van Osch-Gevers M, Delhaas T, et al. Pediatric pulmonary hypertension in the Netherlands. Epidemiology and characterization during the period 1991 to 2005. Circulation. 2011;124(16):1755–64.PubMedCrossRef
56.
go back to reference •• Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012;379(9815):537–46. Baseline data from the TOPP registry, the first international, observational study that focused on pediatric pulmonary hypertension.PubMedCrossRef •• Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. 2012;379(9815):537–46. Baseline data from the TOPP registry, the first international, observational study that focused on pediatric pulmonary hypertension.PubMedCrossRef
57.
go back to reference Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007;115(8):1039–50.PubMedCrossRef Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007;115(8):1039–50.PubMedCrossRef
58.
go back to reference Mulder BJ. Changing demographics of pulmonary arterial hypertension in congenital heart disease. Eur Respir Rev. 2010;19(118):308–13.PubMedCrossRef Mulder BJ. Changing demographics of pulmonary arterial hypertension in congenital heart disease. Eur Respir Rev. 2010;19(118):308–13.PubMedCrossRef
59.
go back to reference •• Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120(2):198–204. This national registry gave us some new insights on the changing epidemiology of PAH related to congenital heart disease in Western countries.PubMedCrossRef •• Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120(2):198–204. This national registry gave us some new insights on the changing epidemiology of PAH related to congenital heart disease in Western countries.PubMedCrossRef
60.
go back to reference Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128(9):745–55.PubMedCrossRef Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128(9):745–55.PubMedCrossRef
61.
go back to reference Diller GP, Dimopoulos K, Broberg CS, Kaya MG, Naghotra US, Uebing A, et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J. 2006;27(14):1737–42.PubMedCrossRef Diller GP, Dimopoulos K, Broberg CS, Kaya MG, Naghotra US, Uebing A, et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J. 2006;27(14):1737–42.PubMedCrossRef
62.
go back to reference Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011;58(5):538–46.PubMedCrossRef Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011;58(5):538–46.PubMedCrossRef
63.
go back to reference Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31(10):1220–9.PubMedCrossRef Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, van Dijk AP, et al. Mortality in adult congenital heart disease. Eur Heart J. 2010;31(10):1220–9.PubMedCrossRef
64.
go back to reference •• Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010;121(1):20–5. The first study identified the long-term benefits in patients with Eisenmenger’s syndrome from the PAH specific therapies.PubMedCrossRef •• Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010;121(1):20–5. The first study identified the long-term benefits in patients with Eisenmenger’s syndrome from the PAH specific therapies.PubMedCrossRef
65.
go back to reference •• Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2013 Mar 1. The first study to compare the long-term survival among four types of PAH related to congenital heart disease. Interestingly, patients with left to right shunts and Eisenmenger’s syndrome have a much better 20-year rate of survival compared to that of patients with repaired congenital heart disease. •• Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2013 Mar 1. The first study to compare the long-term survival among four types of PAH related to congenital heart disease. Interestingly, patients with left to right shunts and Eisenmenger’s syndrome have a much better 20-year rate of survival compared to that of patients with repaired congenital heart disease.
66.
go back to reference Speich R, Jenni R, Opravil M, Pfab M, Russi EW. Primary pulmonary hypertension in HIV infection. Chest. 1991;100(80):1268–71.PubMedCrossRef Speich R, Jenni R, Opravil M, Pfab M, Russi EW. Primary pulmonary hypertension in HIV infection. Chest. 1991;100(80):1268–71.PubMedCrossRef
67.
go back to reference Sitbon O, Lascoux-Combe C, Delfraissy JF, Yeni PG, Raffi F, De Zuttere D, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med. 2008;177(1):108–13.PubMedCrossRef Sitbon O, Lascoux-Combe C, Delfraissy JF, Yeni PG, Raffi F, De Zuttere D, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med. 2008;177(1):108–13.PubMedCrossRef
68.
go back to reference Opravil M, Sereni D. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. AIDS. 2008;22 suppl 3:S35–40.PubMedCrossRef Opravil M, Sereni D. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. AIDS. 2008;22 suppl 3:S35–40.PubMedCrossRef
69.
go back to reference Opravil M, Pechere M, Speich R, Joller-Jemelka HI, Jenni R, Russi EW, et al. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. Am J Respir Crit Care Med. 1997;155(3):990–5.PubMedCrossRef Opravil M, Pechere M, Speich R, Joller-Jemelka HI, Jenni R, Russi EW, et al. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. Am J Respir Crit Care Med. 1997;155(3):990–5.PubMedCrossRef
70.
go back to reference Degano B, Guillaume M, Savale L, Montani D, Jais X, Yaici A, et al. HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS. 2010;24(1):67–75.PubMedCrossRef Degano B, Guillaume M, Savale L, Montani D, Jais X, Yaici A, et al. HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS. 2010;24(1):67–75.PubMedCrossRef
71.
go back to reference McDonnell PJ, Toye PA, Hutchins GM. Primary pulmonary hypertension and cirrhosis: are they related? Am Rev Respir Dis. 1983;127(4):437–41.PubMed McDonnell PJ, Toye PA, Hutchins GM. Primary pulmonary hypertension and cirrhosis: are they related? Am Rev Respir Dis. 1983;127(4):437–41.PubMed
72.
go back to reference Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991;100(2):520–8.PubMed Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991;100(2):520–8.PubMed
73.
go back to reference Le Pavec J, Souza R, Herve P, Lebrec D, Savale L, Tcherakian C, et al. Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med. 2008;178(6):637–43.PubMedCrossRef Le Pavec J, Souza R, Herve P, Lebrec D, Savale L, Tcherakian C, et al. Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med. 2008;178(6):637–43.PubMedCrossRef
74.
go back to reference Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: results from a 10-year screening algorithm. Hepatology. 2006;44(6):1502–10.PubMedCrossRef Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: results from a 10-year screening algorithm. Hepatology. 2006;44(6):1502–10.PubMedCrossRef
75.
go back to reference Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg. 1997;3(5):494–500.PubMedCrossRef Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg. 1997;3(5):494–500.PubMedCrossRef
76.
go back to reference Colle IO, Moreau R, Godinho E, Belghiti J, Ettori F, Cohen-Solal A, et al. Diagnosis of portopulmnonary hypertension in candidates for liver transplantation: a prospective study. Hepatology. 2003;37(2):401–9.PubMedCrossRef Colle IO, Moreau R, Godinho E, Belghiti J, Ettori F, Cohen-Solal A, et al. Diagnosis of portopulmnonary hypertension in candidates for liver transplantation: a prospective study. Hepatology. 2003;37(2):401–9.PubMedCrossRef
77.
go back to reference • Krowka MJ, Miller DP, Barst RJ, Taichman D, Dweik RA, Badesch DB, et al. Portopulmonary hypertension: results from the US-based REVEAL Registry. Chest. 2012;141(4):906–15. Important clinical and prognostic information on PoPH in the modern management era was obtained from this prospective, multicenter registry.PubMedCrossRef • Krowka MJ, Miller DP, Barst RJ, Taichman D, Dweik RA, Badesch DB, et al. Portopulmonary hypertension: results from the US-based REVEAL Registry. Chest. 2012;141(4):906–15. Important clinical and prognostic information on PoPH in the modern management era was obtained from this prospective, multicenter registry.PubMedCrossRef
78.
go back to reference Krowka MJ, Plevak DJ, Findlay JY, Rosen CB, Wiesner RH, Krom RA. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl. 2000;6(4):443–50.PubMedCrossRef Krowka MJ, Plevak DJ, Findlay JY, Rosen CB, Wiesner RH, Krom RA. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transpl. 2000;6(4):443–50.PubMedCrossRef
79.
go back to reference Greiser E. Epidemiologic studies on the relation between use of appetite depressants and primary vascular pulmonary hypertension (in German). Internist (Berl). 1973;14(9):437–42. Greiser E. Epidemiologic studies on the relation between use of appetite depressants and primary vascular pulmonary hypertension (in German). Internist (Berl). 1973;14(9):437–42.
80.
go back to reference 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):111–24.PubMed 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):111–24.PubMed
81.
go back to reference Brenot F, Hervé P, Petitpretz P, Parent F, Duroux P, Simonneau G. Primary pulmonary hypertension and fenfluramine use. Br Heart J. 1993;70(6):537–41.PubMedCrossRef Brenot F, Hervé P, Petitpretz P, Parent F, Duroux P, Simonneau G. Primary pulmonary hypertension and fenfluramine use. Br Heart J. 1993;70(6):537–41.PubMedCrossRef
82.
go back to reference • 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;335(9):609–16. One of the most important studies that investigated the potential risk of primary pulmonary hypertension after exposure to appetite-suppressant drugs.PubMedCrossRef • 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;335(9):609–16. One of the most important studies that investigated the potential risk of primary pulmonary hypertension after exposure to appetite-suppressant drugs.PubMedCrossRef
83.
go back to reference Rich S, Rubin L, Walker AM, Schneeweiss S, Abenhaim L. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest. 2000;117(3):870–4.PubMedCrossRef Rich S, Rubin L, Walker AM, Schneeweiss S, Abenhaim L. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest. 2000;117(3):870–4.PubMedCrossRef
84.
go back to reference Souza R, Humbert M, Sztrymf B, Jaïs X, Yaïci A, Le Pavec J, et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J. 2008;31(2):343–8.PubMedCrossRef Souza R, Humbert M, Sztrymf B, Jaïs X, Yaïci A, Le Pavec J, et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J. 2008;31(2):343–8.PubMedCrossRef
85.
go back to reference Rich S, Shillington A, McLaughlin V. Comparison of survival in patients with pulmonary hypertension associated with fenfluramine to patients with primary pulmonary hypertension. Am J Cardiol. 2003;92(11):1366–8.PubMedCrossRef Rich S, Shillington A, McLaughlin V. Comparison of survival in patients with pulmonary hypertension associated with fenfluramine to patients with primary pulmonary hypertension. Am J Cardiol. 2003;92(11):1366–8.PubMedCrossRef
86.
go back to reference Montani D, Bergot E, Günther S, Savale L, Bergeron A, Bourdin A, et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation. 2012;125(17):2128–37.PubMedCrossRef Montani D, Bergot E, Günther S, Savale L, Bergeron A, Bourdin A, et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation. 2012;125(17):2128–37.PubMedCrossRef
87.
go back to reference Seferian A, Chaumais MC, Savale L, Günther S, Tubert-Bitter P, Humbert M, et al.. Drugs induced pulmonary arterial hypertension. Presse Med. 2013 Aug 21. Seferian A, Chaumais MC, Savale L, Günther S, Tubert-Bitter P, Humbert M, et al.. Drugs induced pulmonary arterial hypertension. Presse Med. 2013 Aug 21.
88.
go back to reference Lapa MDB, Jardim C, Fernandes CJ, Dourado PM, Figueiredo M, Farias A, et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation. 2009;119(11):1518–23.PubMedCrossRef Lapa MDB, Jardim C, Fernandes CJ, Dourado PM, Figueiredo M, Farias A, et al. Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation. 2009;119(11):1518–23.PubMedCrossRef
89.
go back to reference Lapa MSFE, 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 (in Portuguese). Rev Assoc Med Bras. 2006;52(3):139–43.PubMedCrossRef Lapa MSFE, 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 (in Portuguese). Rev Assoc Med Bras. 2006;52(3):139–43.PubMedCrossRef
90.
go back to reference dos Santos Fernandes CJ, Jardim CV, Hovnanian A, Hoette S, Dias BA, Souza S, et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol. 2010;56(9):715–20.PubMedCrossRef dos Santos Fernandes CJ, Jardim CV, Hovnanian A, Hoette S, Dias BA, Souza S, et al. Survival in schistosomiasis-associated pulmonary arterial hypertension. J Am Coll Cardiol. 2010;56(9):715–20.PubMedCrossRef
91.
go back to reference Wagenvoort CA, Beetstra A, Spijker J. Capillary haemangiomatosis of the lungs. Histopathology. 1978;2(6):401–6.PubMedCrossRef Wagenvoort CA, Beetstra A, Spijker J. Capillary haemangiomatosis of the lungs. Histopathology. 1978;2(6):401–6.PubMedCrossRef
92.
go back to reference Montani D, Price LC, Dorfmuller P, Achouh L, Jaïs X, Yaïci A, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33(1):189–200.PubMedCrossRef Montani D, Price LC, Dorfmuller P, Achouh L, Jaïs X, Yaïci A, et al. Pulmonary veno-occlusive disease. Eur Respir J. 2009;33(1):189–200.PubMedCrossRef
93.
go back to reference Montani D, Achouh L, Dorfmüller P, Le Pavec J, Sztrymf B, Tchérakian C, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87(4):220–33.CrossRef Montani D, Achouh L, Dorfmüller P, Le Pavec J, Sztrymf B, Tchérakian C, et al. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore). 2008;87(4):220–33.CrossRef
94.
go back to reference 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;132(2):373–9.PubMedCrossRef 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;132(2):373–9.PubMedCrossRef
Metadata
Title
Epidemiology of Pulmonary Arterial Hypertension
Authors
Xin Jiang
Zhi-Cheng Jing
Publication date
01-12-2013
Publisher
Springer US
Published in
Current Hypertension Reports / Issue 6/2013
Print ISSN: 1522-6417
Electronic ISSN: 1534-3111
DOI
https://doi.org/10.1007/s11906-013-0397-5

Other articles of this Issue 6/2013

Current Hypertension Reports 6/2013 Go to the issue

Hypertension and the Brain (M Banach and A Zanchetti, Section Editors)

Hypertension, Brain Damage and Cognitive Decline

Pulmonary Hypertension (Z-C Jing, Section Editor)

Physiology of the Pulmonary Circulation and the Right Heart

Hypertension and the Brain (M Banach and A Zanchetti, Section Editors)

Blood Pressure Levels and Stroke: J-curve Phenomenon?