Severe pulmonary arterial hypertension: Treatment options and the bridge to transplantation

European Respiratory Review

Volumn 23, Issue 134, 2014, Pages 488-497

  Corris, Paul ^a   Degano, Bruno ^b  

^a NEWCASTLE UPON TYNE HOSPITALS NHS FOUNDATION TRUST   (United Kingdom)

^b Bâtiment Socrate   (France)

Author keywords

[No Author keywords available]

Indexed keywords

IMATINIB; PROSTACYCLIN; TEZOSENTAN; TREPROSTINIL; ANTIHYPERTENSIVE AGENT;

ALGORITHM; AORTOPULMONARY SHUNT; ATRIAL SEPTOSTOMY; DISEASE SEVERITY; DRUG DELIVERY SYSTEM; DRUG EFFICACY; EXTRACORPOREAL OXYGENATION; HEART SURGERY; HUMAN; LUNG ASSIST DEVICE; LUNG PERFUSION; LUNG TRANSPLANTATION; MORTALITY; PATIENT ASSESSMENT; PATIENT IDENTIFICATION; PULMONARY HYPERTENSION; REVIEW; SIDE TO SIDE ANASTOMOSIS; TREATMENT INDICATION; TREATMENT PLANNING; DISEASE COURSE; DISEASE MANAGEMENT; HYPERTENSION, PULMONARY; MULTIMODALITY CANCER THERAPY; PATIENT SELECTION; PHYSIOTHERAPY; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME;

ANTIHYPERTENSIVE AGENTS; COMBINED MODALITY THERAPY; DISEASE MANAGEMENT; DISEASE PROGRESSION; HUMANS; HYPERTENSION, PULMONARY; LUNG TRANSPLANTATION; PATIENT SELECTION; PHYSICAL THERAPY MODALITIES; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME;

EID: 84914178496     PISSN: 09059180     EISSN: 16000617     Source Type: Journal    
DOI: 10.1183/09059180.00007214     Document Type: Review

References (85)

1. Seferian A, Simonneau G. Therapies for pulmonary arterial hypertension: where are we today, where do we go tomorrow? Eur Respir Rev 2013; 22: 217-226.
2. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France. Results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023-1030.
3. Hsu CH, Glassner C, Foreman AJ, et al. Treadmill testing improves survival prediction models in pulmonary arterial hypertension. Am Heart J 2011; 162: 1011-1017.
4. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-induced pulmonary arterial hypertension in the modern management era. Circulation 2010; 122: 156-163.
5. Lee WT, Ling Y, Sheares KK, et al. Predicting survival in pulmonary arterial hypertension in the UK. Eur Respir J 2012; 40: 604-611.
6. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425-1436.
7. Galiè N, Corris PA, Frost AE, et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013; 62: Suppl. 25, D60-D72
8. Hartert M, Senbaklavaci O, Bohrbandt B, et al. Lung transplantation: a treatment option in end-stage lung disease. Dtsch Arztebl Int 2014; 111: 107-116.
9. Orens JB, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update - a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2006; 25: 745-755.
10. McGoon MD, Benza RL, Escribano-Subias P, et al. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62: Suppl. 25, D51-D59
11. Barst RJ, Chung L, Zamanian RT, et al. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL registry. Chest 2013; 144: 160-168.
12. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780-788.
13. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996; 334: 296-301.
14. Galiè N, Olschewski H, Oudiz RJ, 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: 3010-3019.
15. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.
16. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809-818.
17. Ghofrani HA, Galiè N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369: 330-340.
18. Galiè N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148-2157.
19. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2002; 165: 800-804.
20. McLaughlin VV, Benza RL, Rubin LJ, et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010; 55: 1915-1922.
21. Galiè N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119: 2894-2903.
22. Hiremath J, Thankikachalam S, Parikh K, et al. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 2010; 29: 137-149.
23. Olschewski H, Simonneau G, Galiè N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002; 347: 322-329.
24. Hoeper MM, Markevych E, Splekerkoetter E, et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26: 858-863.
25. Hoeper MM, Barst RJ, Bourge RC, et al. Imatinib mesylate as add-on therapy for pulmonary arterial hypertension: results of the randomized IMPRES study. Circulation 2013; 127: 1128-1138.
26. Ghofrani H-A, Humbert M. The role of combination therapy in managing pulmonary arterial hypertension. Eur Respir Rev 2014; 23: 469-475.
27. Sitbon O, Jaïs X, Savale L, et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691-1697.
28. Sitbon O, Bertoletti L. Connective tissue disease associated with pulmonary arterial hypertension: management of a patient with severe haemodynamic impairment. Eur Respir Rev 2014; 23: 505-509.
29. McLaughlin VV, Langer A, Tan M, et al. Contemporary trends in the diagnosis and management of pulmonary arterial hypertension: an initiative to close the care gap. Chest 2013; 143: 324-332.
30. Farber HW, Miller DP, Meltzer LA, et al. Treatment of patients with pulmonary arterial hypertension at the time of death or deterioration to functional class IV: insights from the REVEAL Registry. J Heart Lung Transplant 2013; 31: 1114-1122.
31. emc. Flolan 1.5 mg Powder and Solvent for Solution for Infusion. www.medicines.org.uk/emc/medicine/7173 Date last updated: May 28, 2014. Date last accessed: August 29, 2014
32. GlaxoSmithKline. Flolan prescribing information. www.gsksource.com/gskprm/htdocs/documents/FLOLAN.PDF Date last updated: March 2011. Date last accessed: August 29, 2014
33. López-Medrano F, Fernández-Ruiz M, Ruiz-Cano MJ, et al. High incidence of bloodstream infection due to Gramnegative bacilli in patients with pulmonary hypertension receiving intravenous treprostinil. Arch Bronconeumol 2012; 48: 443-447.
34. Actelion Pharmaceuticals US, Inc. Highlights of prescribing information. www.veletri.com/pdf/veletri_full_ prescribing_information_2nd_gen.pdf Date last updated: June 2012. Date last accessed: August 29, 2014
35. Sitbon O, Delcroix M, Bergot E, et al. EPITOME-2: an open-label study assessing the transition to a new formulation of intravenous epoprostenol in patients with pulmonary arterial hypertension. Am Heart J 2014; 167: 210-217.
36. Tapson VF, Jing ZC, Xu KF, et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial. Chest 2013; 144: 952-958.
37. Tapson VF, Torres F, Kermeen F, 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: 1383-1390.
38. Jing ZC, Parikh K, Pulido T, et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension. Circulation 2013; 127: 624-633.
39. Selexipag (ACT-293897) in pulmonary arterial hypertension, GRIPHON Trial. NCT01106014. http://clinicaltrials. gov/ct2/show/NCT01106014?term5GRIPHON&rank51 Last updated: September 25, 2014. Date last accessed: August 29, 2014.
40. Yusen RD, Christie JD, Edwards LB, et al. The Registry of the International Society for Heart and Lung Transplantation: thirtieth adult lung and heart-lung transplant report - 2013; focus theme: age. J Heart Lung Transplant 2013; 32: 965-978.
41. Lindstedt S, Eyjolfsson A, Koul B, et al. How to recondition ex vivo initially rejected donor lungs for clinical transplantation: clinical experience from Lund University Hospital. J Transplant 2011; 2011: 754383.
42. Mariani AW, Pêgo-Fernandes PM, Abdalla LG, et al. Ex vivo lung reconditioning: a new era for lung transplantation. J Bras Pneumol 2012; 38: 776-785.
43. Cypel M, Yeung JC, Liu M, et al. Normothermic ex vivo lung perfusion in clinical lung transplantation. N Engl J Med 2011; 364: 1431-1440.
44. Chen H, Shiboski SC, Golden JA, et al. Impact of the lung allocation score on lung transplantation for pulmonary arterial hypertension. Am J Respir Crit Care Med 2009; 180: 468-474.
45. Egan TM, Murray S, Bustami RT, et al. Development of the new lung allocation system in the United States. Am J Transplant 2006; 6: 1212-1227.
46. Gomberg-Maitland M, Tapson VF, Benza RL, et al. Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension. Am J Respir Crit Care Med 2005; 172: 1586-1589.
47. Benza RL, Miller DP, Gomberg-Maitland M, 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: 164-172.
48. George MP, Champion HC, Pilewski JM. Lung transplantation for pulmonary hypertension. Pulm Circ 2011; 1: 182-191.
49. Vandervest KM, Zamora MR. Recipient risk factors and lung transplant outcomes. Curr Opin Organ Transplant 2013; 18: 531-536.
50. Keogh A, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol 2009; 54: Suppl. 1, S67-S77
51. Lordan JL, Corris PA. Pulmonary arterial hypertension and lung transplantation. Expert Rev Respir Med 2011; 5: 441-454.
52. Escribano-Subias P, Blanco I, López-Meseguer M, et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012; 40: 596-603.
53. Benza RL, Miller DP, Barst RJ, et al. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL registry. Chest 2012; 142: 448-456.
54. Zhang R, Dai LZ, Xie WP, et al. Survival of Chinese patients with pulmonary arterial hypertension in the modern management era. Chest 2011; 140: 301-309.
55. Thenappan T, Shah SJ, Rich S, et al. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification. Eur Respir J 2010; 35: 1079-1087.
56. Montani D, Jaïs X, Price LC, et al. Cautious epoprostenol therapy is a safe bridge to lung transplantation in pulmonary veno-occlusive disease. Eur Respir J 2009; 34: 1348-1356.
57. Palmer S, Robinson LJ, Wang A, et al. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 1998; 113: 237-240.
58. Dellgren G, Riise GC, Swärd K, et al. Extracorporeal membrane oxygenation as a bridge to lung transplantation: a long-term study. Eur J Cardiothorac Surg 2014 [In press DOI: 10.1093/ejcts/ezu112].
59. Sandoval J, Gaspar J, Pulido T, et al. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. J Am Coll Cardiol 1998; 32: 297-304.
60. Rich S, Dodin E, McLaughlin VV. Usefulness of atrial septostomy as a treatment for primary pulmonary hypertension and guidelines for its application. Am J Cardiol 1997; 80: 369-371.
61. Nihill MR, O’Laughlin MP, Mullins CE. Effects of atrial septostomy in patients with terminal cor pulmonale due to pulmonary vascular disease. Cathet Cardiovasc Diagn 1991; 24: 166-172.
62. Kerstein D, Levy PS, Hsu DT, et al. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995; 91: 2028-2035.
63. Rothman A, Sklansky MS, Lucas VW, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999; 84: 682-686.
64. Sandoval J, Gaspar J, Peña H, et al. Effect of atrial septostomy on the survival of patients with severe pulmonary arterial hypertension. Eur Respir J 2011; 38: 1343-1348.
65. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med 2001; 22: 547-560.
66. Reichenberger F, Pepke-Zaba J, McNeil K, et al. Atrial septostomy in the treatment of severe pulmonary arterial hypertension. Thorax 2003; 58: 797-800.
67. Rich S, Lam W. Atrial septostomy as palliative therapy for refractory primary pulmonary hypertension. Am J Cardiol 1983; 51: 1560-1561.
68. Fischer S, Simon AR, Welte T, et al. Bridge to lung transplantation with the novel pumpless interventional lung assist device NovaLung. J Thorac Cardiovasc Surg 2006; 131: 719-723.
69. Olsson KM, Simon A, Strueber M, et al. Extracorporeal membrane oxygenation in nonintubated patients as bridge to lung transplantation. Am J Transplant 2010; 10: 2173-2178.
70. Del Sorbo L, Boffini M, Rinaldi M, et al. Bridging to lung transplantation by extracorporeal support. Minerva Anestesiol 2012; 78: 243-250.
71. Strueber M, Hoeper MM, Fischer A, et al. Bridge to thoracic organ transplantation in patients with pulmonary arterial hypertension using a pumpless lung assist device. Am J Transplant 2009; 9: 853-857.
72. Javidfar J, Bacchetta M. Bridge to lung transplantation with extracorporeal membrane oxygen support. Curr Opin Organ Transplant 2012; 17: 496-502.
73. Abrams DC, Brodie D, Rosenzweig EB, et al. Upper-body extracorporeal membrane oxygenation as a strategy in decompensated pulmonary arterial hypertension. Pulm Circ 2013; 3: 432-435.
74. Rosenzweig EB, Brodie D, Abrams DC, et al. Extracorporeal membrane oxygenation as a novel bridging strategy for acute right heart failure in Group 1 pulmonary arterial hypertension. ASAIO J 2014; 60: 129-133.
75. Garcia JP, Iacono A, Kon ZN, et al. Ambulatory extracorporeal membrane oxygenation: a new approach for bridgeto- lung transplantation. J Thorac Cardiovasc Surg 2010; 139: e137-e139.
76. Martin JT, Zwischenberger JB. Artificial and novel devices for respiratory support. Semin Thorac Cardiovasc Surg 2013; 25: 70-75.
77. Petersen C, Helvind M, Jensen T, et al. Potts shunt in a child with end-stage pulmonary hypertension after late repair of ventricular septal defect. World J Pediatr Congenit Heart Surg 2013; 4: 286-289.
78. Esch JJ, Shah PB, Cockrill BA, et al. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant 2013; 32: 381-387.
79. Granton J, Mercier O, De Perrot M. Management of severe pulmonary arterial hypertension. Semin Respir Crit Care Med 2013; 34: 700-713.
80. Katz WE, Gasior TA, Quinlan JJ, et al. Immediate effects of lung transplantation on right ventricular morphology and function in patients with variable degrees of pulmonary hypertension. J Am Coll Cardiol 1996; 27: 384-391.
81. McNeil K, Glanville AR, Wahlers T, et al. Comparison of mycophenolate mofetil and azathioprine for prevention of bronchiolitis obliterans syndrome in de novo lung transplant recipients. Transplantation 2006; 81: 998-1003.
82. Christie JD, Edwards LB, Aurora P, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-sixth official adult lung and heart-lung transplantation report - 2009. J Heart Lung Transplant 2009; 28: 1031-1049.
83. de Perrot M, Granton J, McRae K, et al. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single center experience. J Thorac Cardiovasc Surg 2012; 143: 910-918.
84. Toyoda Y, Thacker J, Santos R, et al. Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension. Ann Thorac Surg 2008; 86: 1116-1122.
85. Fadel E, Mercier O,Mussot S, 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: 277-284.