Rubin LJ: Primary pulmonary hypertension. N Engl J Med. 1997, 336 (2): 111-7.
CAS
PubMed
Google Scholar
Simonneau G, et al.: Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009, 54 (1 Suppl): S43-54.
PubMed
Google Scholar
Wagenvoort CA, Wagenvoort N: Primary pulmonary hypertension: a pathological study of the lung vessels in 156 clinically diagnosed cases. Circulation. 1970, 42: 1163-84.
Google Scholar
Hatano S, Strasser T: Primary Pulmonary Hypertension. Report on a WHO meeting. October 15–17, 1973. Geneva: WHO 1975.
Google Scholar
Fishman AP: Clinical classification of pulmonary hypertension. Clin Chest Med. 2001, 22 (3): 385-91. vii
CAS
PubMed
Google Scholar
Cogan JD, 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.
CAS
PubMed
Google Scholar
Aldred MA, et al.: BMPR2 gene rearrangements account for a significant proportion of mutations in familial and idiopathic pulmonary arterial hypertension. Hum Mutat. 2006, 27 (2): 212-3.
PubMed
Google Scholar
Shintani M, et al.: A new nonsense mutation of SMAD8 associated with pulmonary arterial hypertension. J Med Genet. 2009, 46 (5): 331-7.
CAS
PubMed
Google Scholar
Nasim MT, et al.: Molecular genetic characterization of SMAD signaling molecules in pulmonary arterial hypertension. Hum Mutat. 2011, 2011: 2011.
Google Scholar
Austin ED, 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.
CAS
PubMed
Google Scholar
Machado RD, et al.: Mutations of the TGF-beta type II receptor BMPR2 in pulmonary arterial hypertension. Hum Mutat. 2006, 27 (2): 121-32.
CAS
PubMed
Google Scholar
Thomson JR, et al.: Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-ß family. J Med Genet. 2000, 37: 741-5.
CAS
PubMed
Google Scholar
Chaouat A, et al.: Endoglin germline mutation in a patient with hereditary haemorrhagic telangiectasia and dexfenfluramine associated pulmonary arterial hypertension. Thorax. 2004, 59 (5): 446-8.
CAS
PubMed
Google Scholar
Trembath RC, et al.: Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med. 2001, 345 (5): 325-34.
CAS
PubMed
Google Scholar
Galie N, et al.: Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009, 34 (6): 1219-63.
CAS
PubMed
Google Scholar
Simonneau G, et al.: Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004, 43 (12 Suppl S): 5S-12S.
PubMed
Google Scholar
Souza R, et al.: Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases. Eur Respir J. 2008, 31 (2): 343-8.
CAS
PubMed
Google Scholar
Rich S, et al.: Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest. 2000, 117 (3): 870-4.
CAS
PubMed
Google Scholar
Walker AM, et al.: Temporal trends and drug exposures in pulmonary hypertension: an American experience. Am Heart J. 2006, 152 (3): 521-6.
CAS
PubMed
Google Scholar
Frachon I, et al.: Benfluorex and unexplained valvular heart disease: a case–control study. PLoS One. 2010, 5 (4): e10128.
PubMed
Google Scholar
Savale L, et al.: Pulmonary hypertension associated with benfluorex exposure. Eur Respir J. 2012, 40 (5): 1164-72.
PubMed
Google Scholar
Chin KM, Channick RN, Rubin LJ: Is methamphetamine use associated with idiopathic pulmonary arterial hypertension?. Chest. 2006, 130 (6): 1657-63.
CAS
PubMed
Google Scholar
Montani D, et al.: Pulmonary arterial hypertension in patients treated by dasatinib. Circulation. 2012, 125 (17): 2128-37.
CAS
PubMed
Google Scholar
Hachulla E, et al.: Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005, 52 (12): 3792-800.
PubMed
Google Scholar
Mukerjee D, 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.
CAS
PubMed
Google Scholar
Launay D, et al.: Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol. 2007, 34 (5): 1005-11.
PubMed
Google Scholar
de Groote P, 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 (1): 31-6.
CAS
PubMed
Google Scholar
Meune C, 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 (6): 1803-9.
PubMed
Google Scholar
Kim KK, Factor SM: Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome. Hum Pathol. 1987, 18 (12): 1293-6.
CAS
PubMed
Google Scholar
Mehta NJ, et al.: HIV-Related pulmonary hypertension: analytic review of 131 cases. Chest. 2000, 118 (4): 1133-41.
CAS
PubMed
Google Scholar
Opravil M, et al.: HIV-associated primary pulmonary hypertension. A case control study. Swiss HIV Cohort Study. Am J Respir Crit Care Med. 1997, 155 (3): 990-5.
CAS
PubMed
Google Scholar
Sitbon O, 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.
PubMed
Google Scholar
Herve P, et al.: Pulmonary vascular disorders in portal hypertension. Eur Respir J. 1998, 11 (5): 1153-66.
CAS
PubMed
Google Scholar
Rodriguez-Roisin R, et al.: Pulmonary-Hepatic vascular Disorders (PHD). Eur Respir J. 2004, 24 (5): 861-880.
CAS
PubMed
Google Scholar
Hadengue A, et al.: Pulmonary hypertension complicating portal hypertension: prevalence and relation to splanchnic hemodynamics. Gastroenterology. 1991, 100 (2): 520-8.
CAS
PubMed
Google Scholar
Krowka MJ, et al.: Portopulmonary hypertension: Results from a 10-year screening algorithm. Hepatology. 2006, 44 (6): 1502-10.
PubMed
Google Scholar
Eisenmenger V: Die angeborene defecte der kammersheidewand des herzen. Z Klin Med. 1897, 132-1.
Google Scholar
Wood P: The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958, 2: 701-712.
CAS
PubMed
Google Scholar
Daliento L, et al.: Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998, 19 (12): 1845-55.
CAS
PubMed
Google Scholar
Besterman E: Atrial Septal Defect with Pulmonary Hypertension. Br Heart J. 1961, 23 (5): 587-598.
CAS
PubMed
Google Scholar
Hoffman JI, Rudolph AM: The natural history of ventricular septal defects in infancy. Am J Cardiol. 1965, 16 (5): 634-53.
CAS
PubMed
Google Scholar
Lapa MS, et al.: [Clinical characteristics of pulmonary hypertension patients in two reference centers in the city of Sao Paulo]. Rev Assoc Med Bras. 2006, 52 (3): 139-43.
PubMed
Google Scholar
Chaves E: The pathology of the arterial pulmonary vasculature in manson's schistosomiasis. Dis Chest. 1966, 50 (1): 72-7.
CAS
PubMed
Google Scholar
de Cleva R, et al.: Prevalence of pulmonary hypertension in patients with hepatosplenic Mansonic schistosomiasis–prospective study. Hepatogastroenterology. 2003, 50 (54): 2028-30.
PubMed
Google Scholar
Lapa M, et al.: Cardiopulmonary manifestations of hepatosplenic schistosomiasis. Circulation. 2009, 119 (11): 1518-23.
PubMed
Google Scholar
Castro O, Hoque M, Brown BD: Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood. 2003, 101 (4): 1257-61.
CAS
PubMed
Google Scholar
Gladwin MT, et al.: Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease. N Engl J Med. 2004, 350 (9): 886-895.
CAS
PubMed
Google Scholar
Aessopos A, et al.: Pulmonary hypertension and right heart failure in patients with beta-thalassemia intermedia. Chest. 1995, 107 (1): 50-3.
CAS
PubMed
Google Scholar
Smedema JP, Louw VJ: Pulmonary arterial hypertension after splenectomy for hereditary spherocytosis. Cardiovasc J Afr. 2007, 18 (2): 84-9.
CAS
PubMed
Google Scholar
Jais X, et al.: An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation. Hemoglobin. 2003, 27 (3): 139-47.
CAS
PubMed
Google Scholar
Stuard ID, Heusinkveld RS, Moss AJ: Microangiopathic hemolytic anemia and thrombocytopenia in primary pulmonary hypertension. N Engl J Med. 1972, 287 (17): 869-70.
CAS
PubMed
Google Scholar
Parent F, et al.: A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011, 365 (1): 44-53.
CAS
PubMed
Google Scholar
Montani D, 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.
Google Scholar
Lantuejoul S, et al.: Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases. Am J Surg Pathol. 2006, 30 (7): 850-7.
PubMed
Google Scholar
Resten A, et al.: Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 2004, 183 (1): 65-70.
PubMed
Google Scholar
Holcomb BW, et al.: Pulmonary veno-occlusive disease: a case series and new observations. Chest. 2000, 118 (6): 1671-9.
PubMed
Google Scholar
Dufour B, et al.: High-resolution CT of the chest in four patients with pulmonary capillary hemangiomatosis or pulmonary venoocclusive disease. AJR Am J Roentgenol. 1998, 171 (5): 1321-4.
CAS
PubMed
Google Scholar
Montani D, et al.: Pulmonary veno-occlusive disease. Eur Respir J. 2009, 33 (1): 189-200.
CAS
PubMed
Google Scholar
Rabiller A, et al.: Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J. 2006, 27 (1): 108-13.
CAS
PubMed
Google Scholar
Oudiz RJ: Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med. 2007, 28 (1): 233-41.
PubMed
Google Scholar
Abramson SV, et al.: Pulmonary hypertension predicts mortality and morbidity in patients with dilated cardiomyopathy. Ann Intern Med. 1992, 116 (11): 888-95.
CAS
PubMed
Google Scholar
Zener JC, et al.: Regression of extreme pulmonary hypertension after mitral valve surgery. Am J Cardiol. 1972, 30 (8): 820-6.
CAS
PubMed
Google Scholar
Braunwald E, et al.: Effects of Mitral-Valve Replacement on the Pulmonary Vascular Dynamics of Patients with Pulmonary Hypertension. N Engl J Med. 1965, 273: 509-14.
CAS
PubMed
Google Scholar
Delgado JF, et al.: Pulmonary vascular remodeling in pulmonary hypertension due to chronic heart failure. Eur J Heart Fail. 2005, 7 (6): 1011-6.
PubMed
Google Scholar
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 (14): 1718-23.
CAS
PubMed
Google Scholar
Fraser KL, et al.: Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia. Chest. 1999, 115 (5): 1321-8.
CAS
PubMed
Google Scholar
Cottin V, et al.: Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur Respir J. 2005, 26 (4): 586-93.
CAS
PubMed
Google Scholar
Weitzenblum E, et al.: Prognostic value of pulmonary artery pressure in chronic obstructive pulmonary disease. Thorax. 1981, 36 (10): 752-8.
CAS
PubMed
Google Scholar
Thabut G, et al.: Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest. 2005, 127 (5): 1531-6.
PubMed
Google Scholar
Chaouat A, et al.: Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med. 2005, 172 (2): 189-94.
PubMed
Google Scholar
Tapson VF, Humbert M: Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006, 3 (7): 564-7.
PubMed
Google Scholar
Pengo V, et al.: Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004, 350 (22): 2257-64.
CAS
PubMed
Google Scholar
Kim NH: Assessment of operability in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006, 3 (7): 584-8.
PubMed
Google Scholar
Dartevelle P, et al.: Chronic thromboembolic pulmonary hypertension. Eur Respir J. 2004, 23 (4): 637-48.
CAS
PubMed
Google Scholar
Jamieson SW, et al.: Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg. 2003, 76 (5): 1457-62. discussion 1462–4
PubMed
Google Scholar
Suntharalingam J, et al.: Long-term use of sildenafil in inoperable chronic thromboembolic pulmonary hypertension. Chest. 2008, 134 (2): 229-36.
CAS
PubMed
Google Scholar
Jais X, 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.
PubMed
Google Scholar
Rubin LJ, et al.: Current and future management of chronic thromboembolic pulmonary hypertension: from diagnosis to treatment responses. Proc Am Thorac Soc. 2006, 3 (7): 601-7.
PubMed
Google Scholar
Dingli D, et al.: Unexplained pulmonary hypertension in chronic myeloproliferative disorders. Chest. 2001, 120 (3): 801-8.
CAS
PubMed
Google Scholar
Guilpain P, et al.: Pulmonary Hypertension Associated with Myeloproliferative Disorders: A Retrospective Study of Ten Cases. Respiration. 2008, 76: 295-302.
CAS
PubMed
Google Scholar
Marvin KS, Spellberg RD: Pulmonary hypertension secondary to thrombocytosis in a patient with myeloid metaplasia. Chest. 1993, 103 (2): 642-4.
CAS
PubMed
Google Scholar
Nand S, Orfei E: Pulmonary hypertension in polycythemia vera. Am J Hematol. 1994, 47 (3): 242-4.
CAS
PubMed
Google Scholar
Peacock AJ: Pulmonary hypertension after splenectomy: a consequence of loss of the splenic filter or is there something more?. Thorax. 2005, 60 (12): 983-4.
CAS
PubMed
Google Scholar
Gluskowski J, et al.: Pulmonary haemodynamics at rest and during exercise in patients with sarcoidosis. Respiration. 1984, 46 (1): 26-32.
CAS
PubMed
Google Scholar
Bourbonnais JM, Samavati L: Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J. 2008, 32 (2): 296-302.
CAS
PubMed
Google Scholar
Handa T, et al.: Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006, 129 (5): 1246-52.
PubMed
Google Scholar
Nunes H, et al.: Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax. 2006, 61 (1): 68-74.
CAS
PubMed
Google Scholar
Dauriat G, et al.: Lung transplantation for pulmonary langerhans' cell histiocytosis: a multicenter analysis. Transplantation. 2006, 81 (5): 746-50.
PubMed
Google Scholar
Fartoukh M, et al.: Severe pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med. 2000, 161 (1): 216-23.
CAS
PubMed
Google Scholar
Harari S, et al.: Advanced pulmonary histiocytosis X is associated with severe pulmonary hypertension. Chest. 1997, 111 (4): 1142-4.
CAS
PubMed
Google Scholar
Cottin V, et al.: Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients. Eur Respir J. 2012, 40 (3): 630-40.
PubMed
Google Scholar
Montani D, et al.: Pulmonary hypertension in patients with neurofibromatosis type I. Medicine (Baltimore). 2011, 90 (3): 201-11.
Google Scholar
Hamaoka K, et al.: Pulmonary hypertension in type I glycogen storage disease. Pediatr Cardiol. 1990, 11 (1): 54-6.
CAS
PubMed
Google Scholar
Inoue S, et al.: [Pulmonary hypertension due to glycogen storage disease type II (Pompe's disease): a case report]. J Cardiol. 1989, 19 (1): 323-32.
CAS
PubMed
Google Scholar
Humbert M, et al.: Pulmonary arterial hypertension and type-I glycogen-storage disease: the serotonin hypothesis. Eur Respir J. 2002, 20 (1): 59-65.
CAS
PubMed
Google Scholar
Pizzo CJ: Type I glycogen storage disease with focal nodular hyperplasia of the liver and vasoconstrictive pulmonary hypertension. Pediatrics. 1980, 65 (2): 341-3.
CAS
PubMed
Google Scholar
Elstein D, et al.: Echocardiographic assessment of pulmonary hypertension in Gaucher's disease. Lancet. 1998, 351 (9115): 1544-6.
CAS
PubMed
Google Scholar
Lee R, Yousem S: The frequency and type of lung involvement in patients with Gaucher's disease. Lab Invest. 1998, 58: 54-A (Abstract)
Google Scholar
Theise ND, Scheuer PJ, Grundy JE: Cytomegalovirus and autoimmune liver disease. J Clin Pathol. 1989, 42 (12): 1310-1.
CAS
PubMed
Google Scholar
Chu JW, et al.: High prevalence of autoimmune thyroid disease in pulmonary arterial hypertension. Chest. 2002, 122 (5): 1668-73.
PubMed
Google Scholar
Mayer E, et al.: Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2001, 121 (1): 77-82.
CAS
PubMed
Google Scholar
Anderson MB, et al.: Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg. 1995, 59 (6): 1487-90.
CAS
PubMed
Google Scholar
Mussot S, et al.: Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg. 2013, 43 (4): 787-93.
PubMed
Google Scholar
Yigla M, et al.: Pulmonary hypertension in patients with end-stage renal disease. Chest. 2003, 123 (5): 1577-82.
PubMed
Google Scholar
Rich S, et al.: Primary pulmonary hypertension. A national prospective study. Ann Intern Med. 1987, 107 (2): 216-23.
CAS
PubMed
Google Scholar
D'Alonzo GE, et al.: Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991, 115 (5): 343-9.
PubMed
Google Scholar
Humbert M, Sitbon O, Simonneau G: Treatment of pulmonary arterial hypertension. N Engl J Med. 2004, 351 (14): 1425-36.
CAS
PubMed
Google Scholar
Humbert M, et al.: Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006, 173 (9): 1023-30.
PubMed
Google Scholar
Humbert M, 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.
PubMed
Google Scholar
Benza RL, et al.: Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010, 122 (2): 164-72.
PubMed
Google Scholar
Humbert M, et al.: Bone morphogenetic protein receptor 2 germline mutations in pulmonary arterial hypertension associated with fenfluramine derivatives. Eur Respir J. 2002, 20 (3): 518-523.
CAS
PubMed
Google Scholar
Machado RD, et al.: Genetic association of the serotonin transporter in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006, 173 (7): 793-7.
CAS
PubMed
Google Scholar
Thomson J, et al.: Familial and sporadic primary pulmonary hypertension is caused by BMPR2 gene mutations resulting in haploinsufficiency of the bone morphogenetic protein type II receptor. J Heart Lung Transplant. 2001, 20 (2): 149.
PubMed
Google Scholar
Sztrymf B, et al.: Clinical outcomes of pulmonary arterial hypertension in carriers of BMPR2 mutation. Am J Respir Crit Care Med. 2008, 177 (12): 1377-83.
CAS
PubMed
Google Scholar
Loyd J, et al.: Genetic anticipation and abnormal gender ratio at birth in familial primary pulmonary hypertension. Am J Respir Crit Care Med. 1995, 152: 93-97.
CAS
PubMed
Google Scholar
Loyd JE, Primm RK, Newman JH: Familial primary pulmonary hypertension: clinical patterns. Am Rev Respir Dis. 1984, 129 (1): 194-7.
CAS
PubMed
Google Scholar
Deng Z, 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: 737-744.
CAS
PubMed
Google Scholar
Lane KB, et al.: Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet. 2000, 26 (1): 81-4.
CAS
PubMed
Google Scholar
Machado RD, et al.: Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol. 2009, 54 (1 Suppl): S32-42.
CAS
PubMed
Google Scholar
Girerd B, 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.
CAS
PubMed
Google Scholar
Rosenzweig EB, 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.
PubMed
Google Scholar
Aldred MA, et al.: 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.
CAS
PubMed
Google Scholar
Humbert M: Update in pulmonary hypertension 2008. Am J Respir Crit Care Med. 2009, in press
Google Scholar
Trembath R, et al.: Clinical and molecular genetic features of pulmonary hypertension in hereditary hemorrhagic telangiectasia. N Engl J Med. 2001, 345: 325-334.
CAS
PubMed
Google Scholar
Abdalla SA, et al.: Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia. Eur Respir J. 2004, 23 (3): 373-7.
CAS
PubMed
Google Scholar
Harrison RE, et al.: Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia. J Med Genet. 2003, 40 (12): 865-71.
CAS
PubMed
Google Scholar
Harrison RE, et al.: Transforming growth factor-beta receptor mutations and pulmonary arterial hypertension in childhood. Circulation. 2005, 111 (4): 435-41.
CAS
PubMed
Google Scholar
Fujiwara M, et al.: Implications of mutations of activin receptor-like kinase 1 gene (ALK1) in addition to bone morphogenetic protein receptor II gene (BMPR2) in children with pulmonary arterial hypertension. Circ J. 2008, 72 (1): 127-33.
CAS
PubMed
Google Scholar
Smoot LB, et al.: Clinical Features of Pulmonary Arterial Hypertension in Young People with an ALK1 Mutation and Hereditary Hemorrhagic Telangiectasia. Arch Dis Child. 2009, 94 (7): 506-11.
CAS
PubMed
Google Scholar
Cracowski JL, et al.: Évaluation pronostique de biomarqueurs dans l'hypertension artérielle pulmonaire. Rev Mal Respir. 2004, 21 (6 Pt 1): 1137-43.
CAS
PubMed
Google Scholar
Morrell N, et al.: Altered growth responses of pulmonary artery smooth muscle cells from patients with primary pulmonary hypertension to transforming growth factor-beta1 and bone morphogenetic proteins. Circulation. 2001, 104: 790-795.
CAS
PubMed
Google Scholar
Masri F, et al.: Hyperproliferative apoptosis-resistant endothelial cells in idiopathic pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2007, 293 (3): L548-54.
CAS
PubMed
Google Scholar
Sztrymf B, et al.: Genes and pulmonary arterial hypertension. Respiration. 2007, 74 (2): 123-32.
CAS
PubMed
Google Scholar
Girerd B, et al.: Absence of influence of gender and BMPR2 mutation type on clinical phenotypes of pulmonary arterial hypertension. Respir Res. 2010, 11: 73.
PubMed
Google Scholar
West J, et al.: Gene expression in BMPR2 mutation carriers with and without evidence of pulmonary arterial hypertension suggests pathways relevant to disease penetrance. BMC Med Genomics. 2008, 1: 45.
PubMed
Google Scholar
Austin ED, et al.: Alterations in oestrogen metabolism: implications for higher penetrance of familial pulmonary arterial hypertension in females. Eur Respir J. 2009, 34 (5): 1093-9.
CAS
PubMed
Google Scholar
O'Callaghan DS, et al.: Treatment of pulmonary arterial hypertension with targeted therapies. Nat Rev Cardiol. 2011, 8 (9): 526-38.
PubMed
Google Scholar
Stenmark KR, et al.: Hypoxic activation of adventitial fibroblasts: role in vascular remodeling. Chest. 2002, 122 (6 Suppl): 326S-334S.
CAS
PubMed
Google Scholar
Davie NJ, et al.: Hypoxia-induced pulmonary artery adventitial remodeling and neovascularization: contribution of progenitor cells. Am J Physiol Lung Cell Mol Physiol. 2004, 286 (4): L668-78.
CAS
PubMed
Google Scholar
Cool CD, et al.: Three-dimensional reconstruction of pulmonary arteries in plexiform pulmonary hypertension using cell-specific markers. Evidence for a dynamic and heterogeneous process of pulmonary endothelial cell growth. Am J Pathol. 1999, 155 (2): 411-9.
CAS
PubMed
Google Scholar
Montani D, et al.: C-Kit Positive Cells Accumulate in Remodeled Vessels of Idiopathic Pulmonary Arterial Hypertension. Am J Respir Crit Care Med. 2011, 184 (1): 116-23.
PubMed
Google Scholar
Gambaryan N, et al.: Circulating fibrocytes and pulmonary arterial hypertension. Eur Respir J. 2012, 39 (1): 210-2.
CAS
PubMed
Google Scholar
Gambaryan N, et al.: Targeting of c-kit + haematopoietic progenitor cells prevents hypoxic pulmonary hypertension. Eur Respir J. 2011, 37 (6): 1392-9.
CAS
PubMed
Google Scholar
Kherbeck N, et al.: The Role of Inflammation and Autoimmunity in the Pathophysiology of Pulmonary Arterial Hypertension. Clin Rev Allergy Immunol. 2013, 44 (1): 31-8.
CAS
PubMed
Google Scholar
Balabanian K, et al.: CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002, 165 (10): 1419-25.
PubMed
Google Scholar
Itoh T, et al.: Increased plasma monocyte chemoattractant protein-1 level in idiopathic pulmonary arterial hypertension. Respirology. 2006, 11 (2): 158-63.
PubMed
Google Scholar
Nicolls MR, et al.: Autoimmunity and pulmonary hypertension: a perspective. Eur Respir J. 2005, 26 (6): 1110-8.
CAS
PubMed
Google Scholar
Perros F, et al.: Dendritic cell recruitment in lesions of human and experimental pulmonary hypertension. Eur Respir J. 2007, 29 (3): 462-8.
CAS
PubMed
Google Scholar
Herve P, et al.: Pathobiology of pulmonary hypertension. The role of platelets and thrombosis. Clin Chest Med. 2001, 22 (3): 451-8.
CAS
PubMed
Google Scholar
Herve P, et al.: Increased plasma serotonin in primary pulmonary hypertension. Am J Med. 1995, 99: 249-254.
CAS
PubMed
Google Scholar
Huertas A, et al.: Leptin and regulatory T lymphocytes in idiopathic pulmonary arterial hypertension. Eur Respir J. 2012, 40 (4): 895-904.
CAS
PubMed
Google Scholar
Perros F, et al.: Pulmonary lymphoid neogenesis in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2012, 185 (3): 311-21.
PubMed
Google Scholar
Humbert M, et al.: Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004, 43 (12 Suppl S): 13S-24S.
CAS
PubMed
Google Scholar
Christman BW, et al.: An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med. 1992, 327 (2): 70-5.
CAS
PubMed
Google Scholar
Montani D, et al.: Phosphodiesterase type 5 inhibitors in pulmonary arterial hypertension. Adv Ther. 2009, 26 (9): 813-25.
CAS
PubMed
Google Scholar
Petkov V, et al.: Vasoactive intestinal peptide as a new drug for treatment of primary pulmonary hypertension. J Clin Invest. 2003, 111 (9): 1339-46.
CAS
PubMed
Google Scholar
Jeffery T, Morrell NW: Molecular and cellular basis of pulmonary vascular remodeling in pulmonary hypertension. Prog Cardiovasc Dis. 2002, 45: 173-202.
CAS
PubMed
Google Scholar
Giaid A, et al.: Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med. 1993, 328 (24): 1732-9.
CAS
PubMed
Google Scholar
Yuan XJ, et al.: Attenuated K + channel gene transcription in primary pulmonary hypertension. Lancet. 1998, 351 (9104): 726-7.
CAS
PubMed
Google Scholar
Yuan JX, et al.: Dysfunctional voltage-gated K + channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation. 1998, 98 (14): 1400-6.
CAS
PubMed
Google Scholar
Weir EK, et al.: Anorexic Agents Aminorex, Fenfluramine, and Dexfenfluramine Inhibit Potassium Current in Rat Pulmonary Vascular Smooth Muscle and Cause Pulmonary Vasoconstriction. Circulation. 1996, 94 (9): 2216-2220.
CAS
PubMed
Google Scholar
MacLean MR, et al.: 5-hydroxytryptamine and the pulmonary circulation: receptors, transporters and relevance to pulmonary arterial hypertension. Br J Pharmacol. 2000, 131 (2): 161-8.
CAS
PubMed
Google Scholar
Marcos E, et al.: Serotonin transporter inhibitors protect against hypoxic pulmonary hypertension. Am J Respir Crit Care Med. 2003, 168 (4): 487-93.
PubMed
Google Scholar
Keegan A, et al.: Contribution of the 5-HT(1B) receptor to hypoxia-induced pulmonary hypertension: converging evidence using 5-HT(1B)-receptor knockout mice and the 5-HT(1B/1D)-receptor antagonist GR127935. Circ Res. 2001, 89 (12): 1231-9.
CAS
PubMed
Google Scholar
Guilluy C, et al.: Inhibition of RhoA/Rho kinase pathway is involved in the beneficial effect of sildenafil on pulmonary hypertension. Br J Pharmacol. 2005, 146 (7): 1010-8.
CAS
PubMed
Google Scholar
Nagaoka T, et al.: Inhaled Rho kinase inhibitors are potent and selective vasodilators in rat pulmonary hypertension. Am J Respir Crit Care Med. 2005, 171 (5): 494-9.
PubMed
Google Scholar
Guilluy C, et al.: RhoA and Rho kinase activation in human pulmonary hypertension: role of 5-HT signaling. Am J Respir Crit Care Med. 2009, 179 (12): 1151-8.
CAS
PubMed
Google Scholar
Semenza GL: HIF-1 and mechanisms of hypoxia sensing. Curr Opin Cell Biol. 2001, 13 (2): 167-71.
CAS
PubMed
Google Scholar
Semenza GL: Involvement of hypoxia-inducible factor 1 in pulmonary pathophysiology. Chest. 2005, 128 (6 Suppl): 592S-594S.
CAS
PubMed
Google Scholar
Tuder RM, et al.: Expression of angiogenesis-related molecules in plexiform lesions in severe pulmonary hypertension: evidence for a process of disordered angiogenesis. J Pathol. 2001, 195 (3): 367-74.
CAS
PubMed
Google Scholar
Remillard CV, Yuan JX: High altitude pulmonary hypertension: role of K + and Ca2+ channels. High Alt Med Biol. 2005, 6 (2): 133-46.
CAS
PubMed
Google Scholar
Cool CD, et al.: Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection. Hum Pathol. 1997, 28 (4): 434-42.
CAS
PubMed
Google Scholar
Pietra GG, et al.: Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol. 2004, 43 (12 Suppl S): 25S-32S.
PubMed
Google Scholar
Bjornsson J, Edwards WD: Primary pulmonary hypertension: a histopathologic study of 80 cases. Mayo Clin Proc. 1985, 60 (1): 16-25.
CAS
PubMed
Google Scholar
Heath D, Edwards JE: The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958, 18 (4 Part 1): 533-47.
CAS
PubMed
Google Scholar
Dorfmuller P, et al.: Inflammation in pulmonary arterial hypertension. Eur Respir J. 2003, 22 (2): 358-63.
CAS
PubMed
Google Scholar
Dorfmuller P, et al.: Chemokine RANTES in severe pulmonary arterial hypertension. Am J Respir Crit Care Med. 2002, 165 (4): 534-9.
PubMed
Google Scholar
Dorfmuller P, et al.: Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol. 2007, 38 (6): 893-902.
PubMed
Google Scholar
Overbeek MJ, et al.: Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. Eur Respir J. 2009, 34 (2): 371-9.
CAS
PubMed
Google Scholar
Golde DW, et al.: Occult pulmonary haemorrhage in leukaemia. Br Med J. 1975, 2 (5964): 166-8.
CAS
PubMed
Google Scholar
Tron V, et al.: Pulmonary capillary hemangiomatosis. Hum Pathol. 1986, 17 (11): 1144-50.
CAS
PubMed
Google Scholar
Runo JR, 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 (6): 889-94.
PubMed
Google Scholar
Barst RJ, et al.: Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2004, 43 (12 Suppl S): 40S-47S.
PubMed
Google Scholar
Elliott CG, et al.: Pulmonary veno-occlusive disease associated with severe reduction of single-breath carbon monoxide diffusing capacity. Respiration. 1988, 53 (4): 262-6.
CAS
PubMed
Google Scholar
Groepenhoff H, et al.: Exercise testing to estimate survival in pulmonary hypertension. Med Sci Sports Exerc. 2008, 40 (10): 1725-32.
PubMed
Google Scholar
Wensel R, et al.: Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation. 2002, 106 (3): 319-24.
PubMed
Google Scholar
McQuillan BM, et al.: Clinical correlates and reference intervals for pulmonary artery systolic pressure among echocardiographically normal subjects. Circulation. 2001, 104 (23): 2797-802.
CAS
PubMed
Google Scholar
Tunariu N, et al.: Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007, 48 (5): 680-4.
PubMed
Google Scholar
Ley S, et al.: Diagnostic performance of state-of-the-art imaging techniques for morphological assessment of vascular abnormalities in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol. 2012, 22 (3): 607-16.
PubMed
Google Scholar
Marcus JT, et al.: Interventricular mechanical asynchrony in pulmonary arterial hypertension: left-to-right delay in peak shortening is related to right ventricular overload and left ventricular underfilling. J Am Coll Cardiol. 2008, 51 (7): 750-7.
PubMed
Google Scholar
Haddad F, et al.: Right ventricular function in cardiovascular disease, part I: Anatomy, physiology, aging, and functional assessment of the right ventricle. Circulation. 2008, 117 (11): 1436-48.
PubMed
Google Scholar
Galie N, 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.
PubMed
Google Scholar
van Wolferen SA, et al.: Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007, 28 (10): 1250-7.
PubMed
Google Scholar
Naeije R: Hepatopulmonary syndrome and portopulmonary hypertension. Swiss Med Wkly. 2003, 133 (11–12): 163-9.
PubMed
Google Scholar
Hoeper MM, et al.: Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006, 48 (12): 2546-52.
PubMed
Google Scholar
Benza RL, et al.: The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension. Chest. 2012, 141 (2): 354-62.
PubMed
Google Scholar
Sitbon O, Humbert M, Simonneau G: Primary pulmonary hypertension: Current therapy. Prog Cardiovasc Dis. 2002, 45 (2): 115-28.
CAS
PubMed
Google Scholar
Bonnin M, et al.: Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology. 2005, 102 (6): 1133-7. discussion 5A-6A
PubMed
Google Scholar
Price LC, et al.: Non-cardiothoracic non-obstetric surgery in mild-moderate pulmonary hypertension: Perioperative management of 28 consecutive individual cases. Eur Respir J. 2010, 35 (6): 1294-302.
CAS
PubMed
Google Scholar
Provencher S, et al.: Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur Respir J. 2006, 27 (1): 114-20.
CAS
PubMed
Google Scholar
Sitbon O, et al.: Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol. 2002, 40 (4): 780-8.
CAS
PubMed
Google Scholar
Fuster V, et al.: Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation. 1984, 70 (4): 580-7.
CAS
PubMed
Google Scholar
Rich S, Kaufmann E, Levy PS: The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992, 327 (2): 76-81.
CAS
PubMed
Google Scholar
Rich S, et al.: The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest. 1998, 114 (3): 787-92.
CAS
PubMed
Google Scholar
Sitbon O, et al.: Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005, 111 (23): 3105-11.
CAS
PubMed
Google Scholar
Moncada S, et al.: An enzyme isolated from arteries transforms prostaglandin endoperoxides to an unstable substance that inhibits platelet aggregation. Nature. 1976, 263 (5579): 663-5.
CAS
PubMed
Google Scholar
Rubin LJ, et al.: Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med. 1990, 112 (7): 485-91.
CAS
PubMed
Google Scholar
O'Callaghan DS, et al.: Treatment of pulmonary arterial hypertension with targeted therapies. Nat Rev Cardiol. 2011, 8 (9): 526-38.
PubMed
Google Scholar
Higenbottam T, et al.: Long-term treatment of primary pulmonary hypertension with continuous intravenous epoprostenol (prostacyclin). Lancet. 1984, 1 (8385): 1046-7.
CAS
PubMed
Google Scholar
Robbins IM, et al.: A survey of diagnostic practices and the use of epoprostenol in patients with primary pulmonary hypertension. Chest. 1998, 114 (5): 1269-75.
CAS
PubMed
Google Scholar
Barst RJ, et al.: A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996, 334 (5): 296-302.
CAS
PubMed
Google Scholar
Badesch DB, et al.: Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000, 132 (6): 425-34.
CAS
PubMed
Google Scholar
Barst RJ, et al.: Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med. 1994, 121 (6): 409-15.
CAS
PubMed
Google Scholar
McLaughlin VV, Shillington A, Rich S: Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002, 106 (12): 1477-82.
CAS
PubMed
Google Scholar
Rosenzweig EB, Kerstein D, Barst RJ: Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999, 99 (14): 1858-65.
CAS
PubMed
Google Scholar
Nunes H, et al.: Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med. 2003, 167 (10): 1433-9.
PubMed
Google Scholar
Cabrol S, et al.: Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant. 2007, 26 (4): 357-62.
PubMed
Google Scholar
McLaughlin VV, et al.: Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998, 338 (5): 273-7.
CAS
PubMed
Google Scholar
Shapiro SM, et al.: Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol. 1997, 30 (2): 343-9.
CAS
PubMed
Google Scholar
Laliberte K, et al.: Pharmacokinetics and steady-state bioequivalence of treprostinil sodium (Remodulin) administered by the intravenous and subcutaneous route to normal volunteers. J Cardiovasc Pharmacol. 2004, 44 (2): 209-14.
CAS
PubMed
Google Scholar
Simonneau G, et al.: Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002, 165 (6): 800-4.
PubMed
Google Scholar
McLaughlin VV, et al.: Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol. 2010, 55 (18): 1915-22.
CAS
PubMed
Google Scholar
Olschewski H, et al.: Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002, 347 (5): 322-9.
CAS
PubMed
Google Scholar
Hoeper MM, et al.: Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med. 2000, 342 (25): 1866-70.
CAS
PubMed
Google Scholar
Channick RN, et al.: Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet. 2001, 358 (9288): 1119-23.
CAS
PubMed
Google Scholar
Rubin LJ, et al.: Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002, 346 (12): 896-903.
CAS
PubMed
Google Scholar
Humbert M, et al.: Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J. 2004, 24 (3): 353-9.
CAS
PubMed
Google Scholar
Galie N, et al.: Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008, 371 (9630): 2093-100.
CAS
PubMed
Google Scholar
Sitbon O, et al.: Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest. 2003, 124 (1): 247-54.
CAS
PubMed
Google Scholar
McLaughlin VV, et al.: Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005, 25 (2): 244-9.
CAS
PubMed
Google Scholar
Sitbon O, et al.: Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax. 2005, 60 (12): 1025-30.
CAS
PubMed
Google Scholar
Galie N, et al.: Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008, 117 (23): 3010-9.
CAS
PubMed
Google Scholar
Klinger JR, et al.: Long-term pulmonary hemodynamic effects of ambrisentan in pulmonary arterial hypertension. Am J Cardiol. 2011, 108 (2): 302-7.
CAS
PubMed
Google Scholar
Ghofrani HA, et al.: Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial. Lancet. 2002, 360 (9337): 895-900.
CAS
PubMed
Google Scholar
Schermuly RT, et al.: Chronic sildenafil treatment inhibits monocrotaline-induced pulmonary hypertension in rats. Am J Respir Crit Care Med. 2004, 169 (1): 39-45.
PubMed
Google Scholar
Galie N, et al.: Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005, 353 (20): 2148-57.
CAS
PubMed
Google Scholar
Galie N, et al.: Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009, 119 (22): 2894-903.
CAS
PubMed
Google Scholar
Barst RJ, et al.: Tadalafil monotherapy and as add-on to background bosentan in patients with pulmonary arterial hypertension. J Heart Lung Transplant. 2011, 30 (6): 632-43.
PubMed
Google Scholar
Simonneau G, et al.: Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. 2008, 149 (8): 521-30.
PubMed
Google Scholar
McLaughlin VV, et al.: Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med. 2006, 174 (11): 1257-63.
CAS
PubMed
Google Scholar
Hoeper MM, et al.: Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids. Eur Respir J. 2003, 22 (2): 330-4.
CAS
PubMed
Google Scholar
Ghofrani HA, et al.: Oral sildenafil as long-term adjunct therapy to inhaled iloprost in severe pulmonary arterial hypertension. J Am Coll Cardiol. 2003, 42 (1): 158-64.
CAS
PubMed
Google Scholar
Gomberg-Maitland M, et al.: Efficacy and safety of sildenafil added to treprostinil in pulmonary hypertension. Am J Cardiol. 2005, 96 (9): 1334-6.
CAS
PubMed
Google Scholar
Hoeper MM, et al.: Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J. 2004, 24 (6): 1007-10.
CAS
PubMed
Google Scholar
Mathai SC, et al.: Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J. 2007, 29 (3): 469-75.
CAS
PubMed
Google Scholar
Rubin L, et al.: Effect of Macitentan on Morbidity and Mortality in Pulmonary Arterial Hypertension (PAH): Results From the SERAPHIN Trial. Chest. 2012, 142: 1026A-1026A. 10.1378/chest.1456207.
Google Scholar
Iglarz M, et al.: Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J Pharmacol Exp Ther. 2008, 327 (3): 736-45.
CAS
PubMed
Google Scholar
Sidharta PN, et al.: Macitentan: entry-into-humans study with a new endothelin receptor antagonist. Eur J Clin Pharmacol. 2011, 67 (10): 977-84.
CAS
PubMed
Google Scholar
Jing ZC, et al.: Vardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med. 2011, 183 (12): 1723-9.
CAS
PubMed
Google Scholar
Ghofrani HA, Grimminger F: Soluble guanylate cyclase stimulation: an emerging option in pulmonary hypertension therapy. Eur Respir Rev. 2009, 18 (111): 35-41.
CAS
PubMed
Google Scholar
Grimminger F, et al.: First acute haemodynamic study of soluble guanylate cyclase stimulator riociguat in pulmonary hypertension. Eur Respir J. 2009, 33 (4): 785-92.
CAS
PubMed
Google Scholar
Ghofrani HA, et al.: Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study. Eur Respir J. 2010, 36 (4): 792-9.
CAS
PubMed
Google Scholar
Schermuly RT, et al.: Riociguat for the treatment of pulmonary hypertension. Expert Opin Investig Drugs. 2011, 20 (4): 567-76.
CAS
PubMed
Google Scholar
Ghio S, et al.: Left ventricular systolic dysfunction associated with pulmonary hypertension riociguat trial (LEPHT): rationale and design. Eur J Heart Fail. 2012, 14 (8): 946-53.
CAS
PubMed
Google Scholar
Hoeper MM, et al.: Riociguat for interstitial lung disease and pulmonary hypertension: a pilot trial. Eur Respir J. 2013, 41 (4): 853-60.
PubMed
Google Scholar
Ghofrani H, et al.: Riociguat for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2013, 369 (4): 330-40.
CAS
PubMed
Google Scholar
Asaki T, et al.: Structure-activity studies on diphenylpyrazine derivatives: a novel class of prostacyclin receptor agonists. Bioorg Med Chem. 2007, 15 (21): 6692-704.
CAS
PubMed
Google Scholar
Kuwano K, et al.: 2-[4-[(5,6-diphenylpyrazin-2-yl)(isopropyl)amino]butoxy]-N-(methylsulfonyl)acetam ide (NS-304), an orally available and long-acting prostacyclin receptor agonist prodrug. J Pharmacol Exp Ther. 2007, 322 (3): 1181-8.
CAS
PubMed
Google Scholar
Kuwano K, et al.: A long-acting and highly selective prostacyclin receptor agonist prodrug, 2-{4-[(5,6-diphenylpyrazin-2-yl)(isopropyl)amino]butoxy}-N-(methylsulfonyl)acetam ide (NS-304), ameliorates rat pulmonary hypertension with unique relaxant responses of its active form, {4-[(5,6-diphenylpyrazin-2-yl)(isopropyl)amino]butoxy}acetic acid (MRE-269), on rat pulmonary artery. J Pharmacol Exp Ther. 2008, 326 (3): 691-9.
CAS
PubMed
Google Scholar
Morrison K, et al.: Differential effects of selexipag and prostacyclin analogs in rat pulmonary artery. J Pharmacol Exp Ther. 2012, 343 (3): 547-55.
CAS
PubMed
Google Scholar
Simonneau G, et al.: Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J. 2012, 40 (4): 874-80.
CAS
PubMed
Google Scholar
NIH: ClinicalTrials.gov. ACT-BR;293987 in pulmonary arterial hypertension [online]. 2011
Google Scholar
Barst RJ: PDGF signaling in pulmonary arterial hypertension. J Clin Invest. 2005, 115 (10): 2691-4.
CAS
PubMed
Google Scholar
Perros F, et al.: Platelet-derived growth factor expression and function in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med. 2008, 178 (1): 81-8.
CAS
PubMed
Google Scholar
Panos RJ, Baker SK: Mediators, cytokines, and growth factors in liver-lung interactions. Clin Chest Med. 1996, 17 (1): 151-69.
CAS
PubMed
Google Scholar
Schermuly RT, et al.: Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest. 2005, 115 (10): 2811-21.
CAS
PubMed
Google Scholar
Ghofrani H, et al.: Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy. Am J Respir Crit Care Med. 2010, 182 (9): 1171-7.
CAS
PubMed
Google Scholar
Hoeper MM, et al.: Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation. 2013, 127 (10): 1128-38.
CAS
PubMed
Google Scholar
Kerkela R, et al.: Cardiotoxicity of the cancer therapeutic agent imatinib mesylate. Nat Med. 2006, 12 (8): 908-16.
PubMed
Google Scholar
McLaughlin VV, et al.: Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004, 126 (1 Suppl): 78S-92S.
PubMed
Google Scholar
Sandoval J, et al.: Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment. J Am Coll Cardiol. 1998, 32 (2): 297-304.
CAS
PubMed
Google Scholar
Rothman A, et al.: Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol. 1999, 84 (6): 682-6.
CAS
PubMed
Google Scholar
Kerstein D, et al.: Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation. 1995, 91 (7): 2028-35.
CAS
PubMed
Google Scholar
Kurzyna M, et al.: Atrial septostomy in treatment of end-stage right heart failure in patients with pulmonary hypertension. Chest. 2007, 131 (4): 977-83.
PubMed
Google Scholar
Hosenpud JD, et al.: The Registry of the International Society for Heart and Lung Transplantation: eighteenth Official Report-2001. J Heart Lung Transplant. 2001, 20 (8): 805-15.
CAS
PubMed
Google Scholar
Levine SM, et al.: Single lung transplantation for primary pulmonary hypertension. Chest. 1990, 98 (5): 1107-15.
CAS
PubMed
Google Scholar
Pielsticker EJ, Martinez FJ, Rubenfire M: Lung and heart-lung transplant practice patterns in pulmonary hypertension centers. J Heart Lung Transplant. 2001, 20 (12): 1297-304.
CAS
PubMed
Google Scholar
Mendeloff EN, et al.: Lung transplantation for pulmonary vascular disease. Ann Thorac Surg. 2002, 73 (1): 209-17. discussion 217–9
PubMed
Google Scholar
Reitz BA, et al.: Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med. 1982, 306 (10): 557-64.
CAS
PubMed
Google Scholar