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Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán

2014, Number 1

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Rev Invest Clin 2014; 66 (1)

Pulmonary arterial hypertension, a rare entity

Morales-Blanhir JE, Carmona-Rubio AE, Rosas-Romero MJ, Vergara de MGS, Arbo-Oze-de-Morvil GA

Language: Spanish
References: 100
Page: 65-78
PDF size: 211.21 Kb.


ABSTRACT

Pulmonary arterial hypertension (PAH) is a rare disease. It is characterized by the progressive remodeling of the small pulmonary arteries that lead to an elevation of the pulmonary vascular resistance and right ventricular dysfunction. Early diagnosis and prompt treatment avoid the deterioration of the patient’s quality of life. Statistics show that there are 15 cases per million people worldwide, the majority comprised of young women, with an age at diagnosis of 30 to 40 years. At present, the exact processes that initiate the pathophysiologic changes observed in PAH are unknown; it is thought to be multifactorial. Diagnosis in PAH must be done in a step by step manner. Under the suspicion of pulmonary hypertension (PH), the first thing to do is confirm the diagnosis. The second step is to identify the specific etiology and lastly evaluate the severity by echocardiography, hemodynamic parameters, biomarkers and exercise capacity. PH treatment, particularly PAH, has evolved over the last 2 decades due to the advance in disease knowledge and the availability of agents that act on different pathways. Concerning surgical treatment, it is indicated in PAH when there is not an adequate response to medical treatment. In conclusion, PAH is recognized worldwide as a rare or infrequent disease. The countries that have a registry for the diagnostics, treatment, follow-up and prevalence of PH are mainly in Europe. With the exception of the United States and Canada, there are no registries in America. Recent years have shown an important advance in Latin America.


REFERENCES

  1. Chin KM, Rubin LJ. Pulmonary arterial hypertension. J Am Coll Cardiol 2008; 51(16): 1527-38.

  2. Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USAbased registry for pulmonary arterial hypertension: 1982- 2006. Eur Respir J 2007; 30(6): 1103-10.

  3. Tueller C, Stricker H, Soccal P, Tamm M, Aubert JD, Maggiorini M, et al. Epidemiology of pulmonary hypertension: new data from the Swiss registry. Swiss Med Wkly 2008; 138(25- 26): 379-84.

  4. 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.

  5. Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30(1): 104-09.

  6. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation. Task force on expert consensus documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009; 53(17): 1573-619.

  7. 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 Respir J 2009; 34: 1219-63.

  8. Kovacs G, Beghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy control subjects: a systematic review. Eur Respir J 2009; 34(4): 888-94.

  9. Valerio CJ, Schreiber BE, Handler CE, Denton CP, Coghlan JG. Borderline mean pulmonary artery pressure in patients with systemic sclerosis: Transpulmonary gradient predicts risk of developing pulmonary hypertension. Arthritis and Rheumatism 2013; 65(4): 1074-84.

  10. Pfarr N, Szamalek-Hoegel J, Fischer C, Hinderhofer K, Nagel C, Ehlken N, et al. Hemodynamic and clinical onset in patients with hereditary pulmonary arterial hypertension and BMPR2 mutations. Respiratory Research 2011; 12: 99.

  11. Loyd JE, Phillips JA III. Heritable pulmonary arterial hypertension. 2002 [Updated 2012 Dec 20]. In: Pagon RA, Adam MP, Bird TD, et al. (eds.). GeneReviews™ [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2013. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1485

  12. Rubin LJ, Simonneau G, Badesch D, Galie N, Humbert M, Keogh A, et al. The study of risk in pulmonary arterial hypertension. Eur Respir Rev 2012; 21(125): 234-8.

  13. Rosas-Romero MJ, Campos-Cerda R, Morales-Blanhir JE. Clasificación clínica de la hipertensión arterial pulmonar. Neumol Cir Torax 2006; 65(S4): S19-S27.

  14. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54(1 Suppl.): S43-S45.

  15. O’Callaghan DS, Humbert M. A critical analysis of survival in pulmonary arterial hypertension. Eur Respir Rev 2012; 21(125): 218-22.

  16. Morales-Blanhir JE, Carmona-Rubio AE, Rosas-Romero MJ, Vergara GS, Dennis M, Ota AJS. Hipertensión pulmonar, perspectiva en Latinoamérica. Revista Española de Hipertensión Pulmonar 2011; 1(3): 65-8.

  17. Londoño A. Aproximación al diagnóstico clínico de hipertensión pulmonar: signos y síntomas. Rev Col Neum 2010; 22(1 Suppl.): 11-13.

  18. Morales-Blanhir JE, Campos-Cerda R, Rosas-Romero MJ. Proceso diagnóstico en la hipertensión arterial pulmonar. Neumol Cir Torax 2006; 65(S4): S28-S42.

  19. Fisher MR, Forfia PR, Chamera E, Housten-Harris T, Champion HC, Girgis RE. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179(7): 615-21.

  20. Vonk MC, Sander MH, van den Hoogen FH, van Riel PL, Verheugt FW, van Dijk AP. Right ventricle Tei-index: a tool to increase the accuracy of non-invasive detection of pulmonary arterial hypertension in connective tissue diseases. Eur J Echocardiogr 2007; 8(5): 317-21.

  21. Bleeker GB, Steendijk P, Holman ER, Yu CM, Breithardt OA, Kaandorp TA, et al. Acquired right ventricular dysfunction. Heart 2006; 92(1 Suppl.): 14-8. 76 Morales-Blanhir JE, et al. Hipertensión arterial pulmonar. Rev Invest Clin 2014; 66 (1): 65-78

  22. Forfia PR, Fisher MR, Mathai SC, Housten-Harris T, Hemnes AR, Borlaug BA, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J of Respir Crit Care Med 2006; 174(9): 1034-41.

  23. Freed BH, Patel AR, Lang RM. Redefining the role of cardiovascular imaging in patients with pulmonary arterial hypertension. Curr Cardiol Rep 2012; 14(3): 366-73.

  24. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am J Roentgenol 2004; 183(1): 65-70.

  25. Kadowaki T, Yano S, Kobayashi K, Araki K, Tokushima T, Yamadori I. Pulmonary capillary hemangiomatosis-like foci detected by high resolution computed tomography. Intern Med 2010; 49(2): 175-8.

  26. Nistal MA, Martín MT. Imaging tests in chronic thromboembolic pulmonary hypertension. Arch Bronconeumol 2009; 45(6 Suppl.): 21-9.

  27. Sanz J, Fernández-Friera L, Moral S. Imaging techniques and the evaluation of the right heart and the pulmonary circulation. Rev Esp Cardiol 2010; 63(2): 209-23.

  28. Tunariu N, Gibbs SJ, Win Z, Gin-Sing W, Graham A, Gishen P, 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.

  29. Blanco I, Barberá JA. Clinical and hemodynamic diagnosis in chronic thromboembolic pulmonary hypertension. Arch Bronconeumol 2009; 45(6 Suppl.): 15-20.

  30. Armigliato M, Paolini R, Aggio S, Zamboni S, Galasso MP, Zonzin P, et al. Hyperthyroidism as a cause of pulmonary arterial hypertension: a prospective study. Angiology 2006; 57(5): 600-06.

  31. 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.

  32. Uribe M, Morales-Blanhir JE, Rosas-Romero MJ, Campos-Cerda R, Poo JL. Epidemiología, fisiopatología y diagnóstico de la hipertensión arterial pulmonar (HAP) en el cirrótico. Gaceta Médica de México 2012; 148: 153-61.

  33. Porres-Aguilar M, Gallegos-Orozco JF, Garcia H, Aguirre J, Macías-Rodriguez RU, Torre-Delgadillo A. Pulmonary vascular complications in portal hypertension and liver disease: A concise review. Revista de Gastroenterología de México 2013; 78(1): 35-44.

  34. Kawut SM, Krowka MJ, Trotter JF, Roberts KE, Benza RL, Badesch DB, et al. Clinical risk factors for portopulmonary hypertension. Hepatology 2008; 48(1): 196-203.

  35. Escribano SP, Barberà JA, Suberviola V. current diagnostic and prognostic assessment of pulmonary hypertension. Rev Esp Cardiol 2010; 63(5): 583-96.

  36. Hoeper MM, Lee SH, Voswinckel R, Palazzini M, Jais X, Marinelli A, 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.

  37. Morales-Blanhir J, Chaires LG, Rosas-Romero MJ. Cateterismo cardiaco derecho y test de reactividad vascular pulmonar. Revista Española de Hipertensión Pulmonar 2013; 3(2): 112-21.

  38. Morales-Blanhir JE, Santos S, de Jover L, Sala E, Pare C, Roca J, Rodríguez-Roisin R, et al. Clinical value of vasodilator test with inhaled nitric oxide for predicting long-term response to oral vasodilators in pulmonary hypertension. Respir Med 2004; 98: 225-34.

  39. Marcus JT, Gan CT, Zwanenburg JJ, Boonstra A, Allaart CP, Götte MJ, 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.

  40. Van Wolferen SA, Marcus JT, Boonstra A, Marques KM, Bronzwaer JG, Spreeuwenberg MD, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007; 28(10): 1250-7.

  41. Badano LP, Ginghina C, Easaw J, Muraru D, Grillo MT, Lancellotti P, et al. Right ventricle in pulmonary arterial hypertension: haemodynamics, structural changes, imaging, and proposal of a study protocol aimed to assess remodelling and treatment effects. Eur J Echocardiogr 2010; 11(1): 27-37.

  42. Early detection and management of pulmonary arterial hypertension. Eur Respir Rev 2012; 21(126): 306-12.

  43. Taichman DB, Mandel J. Epidemiology of pulmonary arterial hypertension. Clin Chest Med 2007; 28(1): 1-22.

  44. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106(12): 1477-82.

  45. Opravil M, Sereni D. Natural history of HIV-associated pulmonary arterial hypertension: trends in the HAART era. AIDS 2008; 22(3 Suppl.): S35-S40.

  46. Humbert M, Sitbon O, Yaïci A, Montani D, O’Callaghan DS, Jaïs X, et al. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010; 36(3): 549-55.

  47. Rasekaba T, Lee AL, Naughton MT, Williams TJ, Holland AE. The six-minute walk test: a useful metric for the cardiopulmonary patient. Intern Med J 2009; 39(8): 495-501.

  48. Groepenhoff H, Vonk-Noordegraaf A, Boonstra A, Spreeuwenberg MD, Postmus PE, Bogaard HJ. Exercise testing to estimate survival in pulmonary hypertension. Med Sci Sports Exerc 2008; 40(10): 1725-32.

  49. Hoeper MM, Pletz MW, Golpon H, Welte T. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2007; 29(5): 944-50.

  50. Sztrymf B, Souza R, Bertoletti L, Jaïs X, Sitbon O, Price LC, et al. Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 2010; 35(6): 1286-93.

  51. Leuchte HH, Holzapfel M, Baumgartner RA, Ding I, Neurohr C, Vogeser M, et al. Clinical significance of brain natriuretic peptide in primary pulmonary hypertension. J Am Coll Cardiol 2004; 43(5): 764-70.

  52. Takeda Y, Takeda Y, Tomimoto S, Tani T, Narita H, Kimura G. Bilirubin as a prognostic marker in patients with pulmonary arterial hypertension. BMC Pulm Med 2010; 22; 10:22.

  53. Bendayan D, Shitrit D, Ygla M, Huerta M, Fink G, Kramer MR. Hyperuricemia as a prognostic factor in pulmonary arterial hypertension. Respir Med 2003; 97(2): 130-3.

  54. Tang WH, Aytekin M, Hammel J, Hazen SL, Dweik RA. Sensitive cardiac troponin I predicts poor outcomes in pulmonary arterial hypertension. Eur Respir J 2012; 39(4): 939-44.

  55. Miura Y, Fukumoto Y, Sugimura K, Oikawa M, Nakano M, Tatebe S, et al. Identification of new prognostic factors of pulmonary hypertension. Circ J 2010; 74(9): 1965-71

  56. Galié N, Palazzini M, Leci E, Manes A. Current therapeutic approaches to pulmonary arterial hypertension. Rev Esp Cardiol 2010; 63(6): 708-24.

  57. 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.

  58. Ortega H. Tratamiento con bloqueadores de los canales de calcio. Rev Col Neum 2010; 22(1 Suppl.): 50-2. Morales-Blanhir JE, et al. Hipertensión arterial pulmonar. Rev Invest Clin 2014; 66 (1): 65-78 77

  59. Cabrol S, Souza R, Jais X, Fadel E, Ali RH, Humbert M, et al. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2007; 26(4): 357-62.

  60. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106(12): 1477-82.

  61. Lang I, Gómez-Sánchez M, Kneussl M, Naeije R, Escribano P, Skoro-Sajer N, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest 2006; 129(6): 1636-43.

  62. Benza RL, Seeger W, McLaughlin VV, Channick RN, Voswinckel R, Tapson VF, et al. Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: the treprostinil sodium inhalation used in the management of pulmonary arterial hypertension (TRIUMPH) study openlabel extension. J Heart Lung Transplant 2011; 30(12): 1327-33.

  63. Tapson VF, Torres F, Kermeen F, Keogh AM, Allen RP, Frantz RP, Badesch DB, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (The FREEDOM-C Study): A randomized controlled trial. Chest 2012; 142(6): 1383-90.

  64. Olschewski H, Hoeper MM, Behr J, Ewert R, Meyer A, Borst MM, et al. Long-term therapy with inhaled iloprost in patients with pulmonary hypertension. Respir Med 2010; 104(5): 731-40.

  65. Galié N, Humbert M, Vachiéry JL, Vizza CD, Kneussl M, Manes A, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trial. J Am Coll Cardiol 2002; 39(9): 1496-502.

  66. Barst RJ, McGoon M, McLaughlin V, Tapson V, Rich S, Rubin L, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41(12): 2119-25.

  67. Kunieda T, Nakanishi N, Matsubara H, Ohe T, Okano Y, Kondo H, et al. Effects of long-acting beraprost sodium (TRK- 100STP) in Japanese patients with pulmonary arterial hypertension. Int Heart J 2009; 50(4): 513-29.

  68. Simonneau G, Torbicki A, Hoeper MM, Delcroix M, Karlócai K, Galié N, et al. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012; 40(4): 874-80.

  69. Morrison K, Studer R, Ernst R, Haag F, Kauser K, Clozel M. Differential effects of Selexipag [corrected] and prostacyclin analogs in rat pulmonary artery. J Pharmacol Exp Ther 2012; 343(3): 547-55.

  70. Sitbon O, Morrell NW. Pathways in pulmonary arterial hypertension: the future is here. Eur Respir Rev 2012; 21(126): 321-7.

  71. Keogh A, McNeil K, Williams TJ, Gabbay E, Proudman S, Weintraub RG, et al. The bosentan patient registry: Long-term survival in pulmonary arterial hypertension. Intern Med J 2011; 41(3): 227-34.

  72. McGoon MD, Frost AE, Oudiz RJ, Badesch DB, Galie N, Olschewski H, et al. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest 2009; 135(1): 122-9.

  73. Bruderer S, Marjason J, Sidharta PN, Dingemanse J. Pharmacokinetics of macitentan in caucasian and japanese subjects: The influence of ethnicity and sex. Pharmacology 2013; 91(5-6): 331-8.

  74. Raghu G, Million-Rousseau R, Morganti A, Perchenet L, Behr J. MUSIC study group. Macitentan for the treatment of idiopathic pulmonary fibrosis: the randomised controlled MUSIC trial. Eur Respir J 2013 [Epub ahead of print].

  75. Pulido T, Adzerikho I, Channick RN, Delcroix M, Galié N, Ghofrani HA, et al. Macicentan and morbity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369(6): 809-918.

  76. Galié N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353(20): 2148-57.

  77. Rubin LJ, Badesch DB, Fleming TR, Galiè N, Simonneau G, Ghofrani HA, et al. Long-term treatment with sildenafil citrate in pulmonary arterial hypertension: the SUPER-2 study. Chest 2011; 140(5): 1274-83.

  78. Blanco I, Gimeno E, Munoz PA, Pizarro S, Gistau C, Rodriguez- Roisin R, et al. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease and pulmonary hypertension. Am J Respir Crit Care Med 2010; 181(3): 270-8.

  79. Barst RJ, Oudiz RJ, Beardsworth A, Brundage BH, Simonneau G, Ghofrani HA, 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.

  80. Jing ZC, Yu ZX, Shen JY, Wu BX, Xu KF, Zhu XY, 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.

  81. Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Zhi-Cheng J, Keogh AM, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-40.

  82. Ghofrani HA, D’Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013; 369: 319-29.

  83. Hoeper MM, Halank M, Wilkens H, et al. Riociguat for interstitial lung disease and pulmonary hypertension a pilot trial. Eur Respir J 2013; 41: 853-60.

  84. Bonderman D, Ghio S, Felix SB, Ghofrani HA, Michelakis ED, Mitrovic V, et al. On behalf of the left ventricular systolic dysfunction associated with pulmonary hypertension riociguat Trial (LEPHT) study group. Riociguat for patients with pulmonary hypertension due to systolic left ventricular dysfunction: A phase IIb double-blind, randomized, placebo-controlled, dose-ranging hemodynamic study. Circulation 2013; 128(5): 502-11.

  85. Rubin LJ. Treatment of pulmonary hypertension due to left heart failure with PAH-specific therapies: lessons from the right and LEPHT. Circularion 2013; 128 (5): 475-6

  86. Provencher S, Jais X, Yaici A, Sitbon O, Humbert M, Simonneau G. Clinical challenges in pulmonary hypertension: Roger S. Mitchell lecture. Chest 2005; 128(6 Suppl.): 622S-628S.

  87. Humbert M, Barst RJ, Robbins IM, Channick RN, Galiè N, Boonstra A, et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24(3): 353-9.

  88. McLaughlin VV, Oudiz RJ, Frost A, Tapson VF, Murali S, Channick RN, 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.

  89. Porhownik NR, Al-Sharif H, Bshouty Z. Addition of sildenafil in patients with pulmonary arterial hypertension with inadequate response to bosentan monotherapy. Can Respir J 2008; 15(8): 427-30.

  90. Galiè N, Rubin LJ, Hoeper M, Jansa P, Al-Hiti H, Meyer G, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double78 Morales-Blanhir JE, et al. Hipertensión arterial pulmonar. Rev Invest Clin 2014; 66 (1): 65-78 blind, randomised controlled trial. Lancet 2008; 371(9630): 2093-100.

  91. Simonneau G, Rubin LJ, Galiè N, Barst RJ, Fleming TR, Frost AE, 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.

  92. McLaughlin VV, Benza RL, Rubin LJ, Channick RN, Voswinckel R, Tapson VF, 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.

  93. Abraham T, Wu G, Vastey F, Rapp J, Saad N, Balmir E. Role of combination therapy in the treatment of pulmonary arterial hypertension. Pharmacotherapy 2010; 30(4): 390-404.

  94. Rhodes CJ, Davidson A, Gibbs JS, Wharton J, Wilkins MR. Therapeutic targets in pulmonary arterial hypertension. Pharmacol Ther 2009; 121(1): 69-88.

  95. Rubin LJ, Santana LA. Treatment of pulmonary arterial hypertension: A look to the future. Avances Cardiol 2009; 29(2): 124-9.

  96. Souza R, Sitbon O, Parent F, Simonneau G, Humbert M. Long term imatinib treatment in pulmonary arterial hypertension. Thorax 2006;61(8):736.

  97. Reynolds PN. Gene therapy for pulmonary hypertension: prospects and challenges. Expert Opin Biol Ther 2011; 11:133-43.

  98. Carmona-Rubio AE, Vergara GS, Morales-Blanhir JE. Hipertensión arterial pulmonar, ¿hacia dónde vamos en el tratamiento? Revista Española de Hipertensión Pulmonar 2013; (3) 1:10-13

  99. Law MA, Grifka RG, Mullins CE, Nihill MR. Atrial septostomy improves survival in select patients with pulmonary hypertension. Am Heart J 2007;153(5):779-84.

  100. Fadel E, Mercier O, Mussot S, Leroy-Ladurie F, Cerrina J, Chapelier A, et al. Long-term outcome of double-lung and heart-lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients. Eur J Cardiothorac Surg 2010;38(3):277-84




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