The challenge of managing pulmonary arterial hypertension in adults with congenital heart disease

Expert Review of Cardiovascular Therapy

Volumn 11, Issue 7, 2013, Pages 919-931

  Radke, Robert M ^a   Diller, Gerhard Paul ^a   Baumgartner, Helmut ^a  

^a UNIVERSITY HOSPITAL MÜNSTER   (Germany)

Author keywords

bosentan; congenital heart disease; disease targeting therapies; Eisenmenger syndrome; endothelin receptor antagonists; phosphodiesterase inhibitors; pulmonary arterial hypertension; shunt lesions; sildenafil

Indexed keywords

AMBRISENTAN; BOSENTAN; DIGOXIN; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; IMATINIB; PHOSPHODIESTERASE INHIBITOR; PLACEBO; PROSTACYCLIN; PROSTACYCLIN DERIVATIVE; PROSTAGLANDIN E1; SILDENAFIL; SITAXSENTAN; TADALAFIL; TREPROSTINIL;

ADD ON THERAPY; ARTERIAL OXYGEN SATURATION; ATRIOVENTRICULAR SEPTAL DEFECT; CARDIOVASCULAR MORTALITY; CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION; CONGENITAL HEART DISEASE; CONNECTIVE TISSUE DISEASE; DRUG WITHDRAWAL; EISENMENGER COMPLEX; EXERCISE; FUNCTIONAL STATUS; HEART ATRIUM SEPTUM DEFECT; HEART DISEASE; HEART FAILURE; HEART LEFT RIGHT SHUNT; HEART SURGERY; HEART VENTRICLE SEPTUM DEFECT; HUMAN; INJECTION SITE PAIN; LUNG ARTERY PRESSURE; LUNG CIRCULATION; LUNG DISEASE; LUNG HEMODYNAMICS; LUNG VASCULAR RESISTANCE; MONOTHERAPY; MORBIDITY; MULTICENTER STUDY (TOPIC); NEW YORK HEART ASSOCIATION CLASS; OUTCOME ASSESSMENT; OXYGEN SATURATION; OXYGEN THERAPY; PATENT DUCTUS ARTERIOSUS; PATHOPHYSIOLOGY; PREVALENCE; PULMONARY ARTERY OCCLUSION PRESSURE; PULMONARY HYPERTENSION; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SEPSIS; SURVIVAL; SYSTEMIC PULMONARY SHUNT; UNSPECIFIED SIDE EFFECT;

ADULT; AGE FACTORS; HEART DEFECTS, CONGENITAL; HUMANS; HYPERTENSION, PULMONARY; PATIENT SELECTION; SURVIVAL;

EID: 84881118649     PISSN: 14779072     EISSN: 17448344     Source Type: Journal    
DOI: 10.1586/14779072.2013.811966     Document Type: Review

References (93)

1. Simonneau G, Robbins IM, Beghetti M et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 54(Suppl. 1), S43-S54 (2009
2. Hoeper MM. Definition, classification, and epidemiology of pulmonary arterial hypertension. Semin. Respir. Crit. Care Med. 30(4), 369-375 (2009
3. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br. Med. J. 2(5099), 755-762 (1958
4. Engelfriet PM, Duffels MG, Möller T et al. Pulmonary arterial hypertension in adults born with a heart septal defect: The Euro Heart Survey on adult congenital heart disease. Heart 93(6), 682-687 (2007
5. 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. 58(5), 538-546 (2011
6. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation 70(4), 580-587 (1984
7. Beghetti M, Galiè N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 53(9), 733-740 (2009
8. Duffels MG, Engelfriet PM, Berger RM et al. Pulmonary arterial hypertension in congenital heart disease: An epidemiologic perspective from a Dutch registry. Int. J. Cardiol. 120(2), 198-204 (2007
9. McLaughlin VV, Presberg KW, Doyle RL et al.; American College of Chest Physicians. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 126(Suppl. 1), 78S-92S (2004
10. Humenberger M, Rosenhek R, Gabriel H et al. Benefit of atrial septal defect closure in adults: Impact of age. Eur. Heart J. 32(5), 553-560 (2011
11. Huang ZW, Fan ZX, Sun JT et al. The short-And medium-Term results of transcatheter closure of atrial septal defect with severe pulmonary arterial hypertension. Heart Vessels 27(6), 603-609 (2012
12. Therrien J, Rambihar S, Newman B et al. Eisenmenger syndrome and atrial septal defect: Nature or nurture? Can. J. Cardiol. 22(13), 1133-1136 (2006
13. Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur. Respir. Rev. 18(113), 154-161 (2009
14. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation 115(2), 163-172 (2007
15. Schuuring MJ, Bouma BJ, Cordina R et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int. J. Cardiol. 164(1), 106-110 (2013
16. 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 18(4 Pt 1), 533-547 (1958
17. Rabinovitch M, Haworth SG, Castaneda AR, Nadas AS, Reid LM. Lung biopsy in congenital heart disease: A morphometric approach to pulmonary vascular disease. Circulation 58(6), 1107-1122 (1978
18. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N. Engl. J. Med. 351(16), 1655-1665 (2004
19. Haworth SG. Role of the endothelium in pulmonary arterial hypertension. Vascul. Pharmacol. 45(5), 317-325 (2006
20. Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J. Clin. Invest. 122(12), 4306-4313 (2012
21. Förstermann U, Münzel T. Endothelial nitric oxide synthase in vascular disease: From marvel to menace. Circulation 113(13), 1708-1714 (2006
22. Galiè N, Ghofrani HA, Torbicki A et al.; Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N. Engl. J. Med. 353(20), 2148-2157 (2005
23. Schiffrin EL. Vascular endothelin in hypertension. Vascul. Pharmacol. 43(1), 19-29 (2005
24. Dupuis J, Hoeper MM. Endothelin receptor antagonists in pulmonary arterial hypertension. Eur. Respir. J. 31(2), 407-415 (2008
25. Falcetti E, Hall SM, Phillips PG et al. Smooth muscle proliferation and role of the prostacyclin (IP) receptor in idiopathic pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 182(9), 1161-1170 (2010
26. Diller GP, Dimopoulos K, Broberg CS et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: A combined retrospective and case-control study. Eur. Heart J. 27(14), 1737-1742 (2006
27. McLaughlin VV, Davis M, Cornwell W. Pulmonary arterial hypertension. Curr. Probl. Cardiol. 36(12), 461-517 (2011
28. 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 122(2), 164-172 (2010
29. Mulder BJ. Changing demographics of pulmonary arterial hypertension in congenital heart disease. Eur. Respir. Rev. 19(118), 308-313 (2010
30. Daliento L, Somerville J, Presbitero P et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur. Heart J. 19(12), 1845-1855 (1998
31. Cantor WJ, Harrison DA, Moussadji JS et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am. J. Cardiol. 84(6), 677-681 (1999
32. Saha A, Balakrishnan KG, Jaiswal PK et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int. J. Cardiol. 45(3), 199-207 (1994
33. Oya H, Nagaya N, Satoh T et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. Heart 84(1), 53-58 (2000
34. Clarkson PM, Frye RL, DuShane JW, Burchell HB, Wood EH, Weidman WH. Prognosis for patients with ventricular septal defect and severe pulmonary vascular obstructive disease. Circulation 38(1), 129-135 (1968
35. Moceri P, Dimopoulos K, Liodakis E et al. Echocardiographic predictors of outcome in Eisenmenger syndrome. Circulation 126(12), 1461-1468 (2012
36. Diller GP, Alonso-Gonzalez R, Kempny A et al. B-Type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: Predictive value and response to disease targeting therapy. Heart 98(9), 736-742 (2012
37. Budts W, Van Pelt N, Gillyns H, Gewillig M, Van De Werf F, Janssens S. Residual pulmonary vasoreactivity to inhaled nitric oxide in patients with severe obstructive pulmonary hypertension and Eisenmenger syndrome. Heart 86(5), 553-558 (2001
38. Post MC, Janssens S, Van de Werf F, Budts W. Responsiveness to inhaled nitric oxide is a predictor for mid-Term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur. Heart J. 25(18), 1651-1656 (2004
39. Baumgartner H, Bonhoeffer P, De Groot NM et al.; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur. Heart J. 31(23), 2915-2957 (2010
40. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 115(8), 1039-1050 (2007
41. Broberg CS, Bax BE, Okonko DO et al. Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J. Am. Coll. Cardiol. 48(2), 356-365 (2006
42. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J. Am. Coll. Cardiol. 28(3), 768-772 (1996
43. Perloff JK, Rosove MH, Child JS, Wright GB. Adults with cyanotic congenital heart disease: Hematologic management. Ann. Intern. Med. 109(5), 406-413 (1988
44. Broberg CS, Ujita M, Prasad S et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J. Am. Coll. Cardiol. 50(7), 634-642 (2007
45. Ammash NM, Connolly HM, Abel MD, Warnes CA. Noncardiac surgery in Eisenmenger syndrome. J. Am. Coll. Cardiol. 33(1), 222-227 (1999
46. Oechslin E. Eisenmenger's syndrome. In: Adult Congenital Heart Disease. Gatzoulis M, Webb G, Daubeney P (Eds). Elsevier, PA, USA, 363-378 (2003
47. Rubin LJ, Mendoza J, Hood M et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann. Intern. Med. 112(7), 485-491 (1990
48. Fernandes SM, Newburger JW, Lang P et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am. J. Cardiol. 91(5), 632-635 (2003
49. Rosenzweig EB, Kerstein D, Barst RJ. Long-Term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 99(14), 1858-1865 (1999
50. Simonneau G, Barst RJ, Galie N et al.; Treprostinil Study Group. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: A doubleblind, randomized, placebo-controlled trial. Am. J. Respir. Crit. Care Med. 165(6), 800-804 (2002
51. Olschewski H, Simonneau G, Galiè N et al.; Aerosolized Iloprost Randomized Study Group. Inhaled iloprost for severe pulmonary hypertension. N. Engl. J. Med. 347(5), 322-329 (2002
52. Channick RN, Olschewski H, Seeger W, Staub T, Voswinckel R, Rubin LJ. Safety and efficacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. J. Am. Coll. Cardiol. 48(7), 1433-1437 (2006
53. Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC), European Respiratory Society (ERS), International Society of Heart and Lung Transplantation (ISHLT) et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Respir. J. 34(6), 1219-1263 (2009
54. 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. 31(17), 2080-2086 (2010
55. Galiè N, Beghetti M, Gatzoulis MA et al.; Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study. Circulation 114(1), 48-54 (2006
56. Gatzoulis MA, Beghetti M, Galiè N et al.; BREATHE-5 Investigators. Longer-Term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int. J. Cardiol. 127(1), 27-32 (2008
57. Apostolopoulou SC, Manginas A, Cokkinos DV, Rammos S. Effect of the oral endothelin antagonist bosentan on the clinical, exercise, and haemodynamic status of patients with pulmonary arterial hypertension related to congenital heart disease. Heart 91(11), 1447-1452 (2005
58. van Loon RL, Hoendermis ES, Duffels MG et al Long-Term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-To-pulmonary shunt: Does the beneficial effect persist? Am. Heart J. 154(4), 776-782 (2007
59. Vis JC, Duffels MG, Mulder P et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int. J. Cardiol. 164(1), 64-69 (2013
60. Diller GP, Alonso-Gonzalez R, Dimopoulos K et al. Disease targeting therapies in patients with Eisenmenger syndrome: Response to treatment and long-Term efficiency. Int. J. Cardiol. doi:10.1016/j.ijcard.2012.02.007 (2012) (Epub ahead of print
61. Tay EL, Papaphylactou M, Diller GP et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int. J. Cardiol. 149(3), 372-376 (2011
62. Schulze-Neick I, Gilbert N, Ewert R et al. Adult patients with congenital heart disease and pulmonary arterial hypertension: First open prospective multicenter study of bosentan therapy. Am. Heart J. 150(4), 716 (2005
63. Zhang ZN, Jiang X, Zhang R et al. Oral sildenafil treatment for Eisenmenger syndrome: A prospective, open-label, multicentre study. Heart 97(22), 1876-1881 (2011
64. Mukhopadhyay S, Sharma M, Ramakrishnan S et al. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: A preliminary observational study. Circulation 114(17), 1807-1810 (2006
65. Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Søndergaard L. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: A randomized, placebo-controlled, double-blinded trial. Eur. Heart J. 31(9), 1124-1131 (2010
66. D'Alto M, Romeo E, Argiento P et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int. J. Cardiol. 155(3), 378-382 (2012
67. Galiè N, Rubin Lj, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): A double-blind, randomised controlled trial. Lancet 371(9630), 2093-2100 (2008
68. Dimopoulos K, Inuzuka R, Goletto S et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 121(1), 20-25 (2010
69. Patterson KC, Weissmann A, Ahmadi T, Farber HW. Imatinib mesylate in the treatment of refractory idiopathic pulmonary arterial hypertension. Ann. Intern. Med. 145(2), 152-153 (2006
70. 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 127(10), 1128-1138 (2013
71. Peacock A, Keogh A, Humbert M. Endpoints in pulmonary arterial hypertension: The role of clinical worsening. Curr. Opin. Pulm. Med. 16(Suppl. 1), S1-S9 (2010
72. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N. Engl. J. Med. 346(12), 896-903 (2002
73. Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am. J. Cardiol. 94(2), 261-263 (2004
74. Barst RJ, Langleben D, Frost A et al.; STRIDE-1 Study Group. Sitaxsentan therapy for pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 169(4), 441-447 (2004
75. Gatzoulis MA, Rogers P, Li W et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int. J. Cardiol. 98(1), 147-151 (2005
76. Kotlyar E, Sy R, Keogh AM et al. Bosentan for the treatment of pulmonary arterial hypertension associated with congenital cardiac disease. Cardiol. Young 16(3), 268-274 (2006
77. Benza RL, Rayburn BK, Tallaj JA et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest 129(4), 1009-1015 (2006
78. Sitbon O, Beghetti M, Petit J et al. Bosentan for the treatment of pulmonary arterial hypertension associated with congenital heart defects. Eur. J. Clin. Invest. 36(Suppl. 3), 25-31 (2006
79. Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebocontrolled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am. Heart J. 151(4), 851.e1-851.e5 (2006
80. Barst RJ, Langleben D, Badesch D et al.; STRIDE-2 Study Group. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J. Am. Coll. Cardiol. 47(10), 2049-2056 (2006
81. D'Alto M, Vizza CD, Romeo E et al. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): Safety, tolerability, clinical, and haemodynamic effect. Heart 93(5), 621-625 (2007
82. Apostolopoulou SC, Manginas A, Cokkinos DV, Rammos S. Long-Term oral bosentan treatment in patients with pulmonary arterial hypertension related to congenital heart disease: A 2-year study. Heart 93(3), 350-354 (2007
83. Diller GP, Dimopoulos K, Kaya MG et al. Long-Term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart 93(8), 974-976 (2007
84. Chau EM, Fan KY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int. J. Cardiol. 120(3), 301-305 (2007
85. Galiè N, Olschewski H, Oudiz RJ et al.; Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. 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 117(23), 3010-3019 (2008
86. Galiè N, Brundage BH, Ghofrani HA et al.; Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil therapy for pulmonary arterial hypertension. Circulation 119(22), 2894-2903 (2009
87. Duffels MG, Vis JC, van Loon RL et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome. Am. J. Cardiol. 103(9), 1309-1315 (2009
88. Kermeen FD, Franks C, O'Brien K et al. Endothelin receptor antagonists are an effective long term treatment option in pulmonary arterial hypertension associated with congenital heart disease with or without trisomy 21. Heart. Lung Circ. 19(10), 595-600 (2010
89. Lu XL, Xiong CM, Shan GL et al. Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease. Cardiovasc. Ther. 28(6), 350-355 (2010
90. D'Alto M, Romeo E, Argiento P et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. Int. J. Cardiol. 164(3), 323-326 (2013
91. Zeng WJ, Lu XL, Xiong CM et al.; Sildenafil Therapy on Pulmonary Arterial Hypertension Associated With Different Types of Congenital Heart Disease Study Group. The efficacy and safety of sildenafil in patients with pulmonary arterial hypertension associated with the different types of congenital heart disease. Clin. Cardiol. 34(8), 513-518 (2011
92. Monfredi O, Griffiths L, Clarke B, Mahadevan VS. Efficacy and safety of bosentan for pulmonary arterial hypertension in adults with congenital heart disease. Am. J. Cardiol. 108(10), 1483-1488 (2011
93. Dong MF, Ma ZS, Ma SJ et al. Effect of prostaglandin E1 on pulmonary arterial hypertension following corrective surgery for congenital heart disease. J. Cardiovasc. Pharmacol. Ther. 17(3), 303-307 (2012