The role of the right ventricle in pulmonary arterial hypertension

European Respiratory Review

Volumn 20, Issue 122, 2011, Pages 243-253

  Vonk Noordegraaf, A ^a   Galie N ^b  

^a VU UNIVERSITY MEDICAL CENTER   (Netherlands)

^b UNIVERSITY OF BOLOGNA   (Italy)

Author keywords

Prognostic factors; Pulmonary hypertension; Right ventricle

Indexed keywords

AMBRISENTAN; BOSENTAN; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; PLACEBO; PROSTACYCLIN; SILDENAFIL; SIMVASTATIN;

ADD ON THERAPY; CARDIOPULMONARY HEMODYNAMICS; CARDIOVASCULAR MAGNETIC RESONANCE; CARDIOVASCULAR PARAMETERS; DISEASE ASSOCIATION; DISEASE COURSE; DISEASE SEVERITY; ECHOCARDIOGRAPHY; HEART AFTERLOAD; HEART CATHETERIZATION; HEART INDEX; HEART PERFORMANCE; HEART RIGHT ATRIUM PRESSURE; HEART RIGHT VENTRICLE; HEART RIGHT VENTRICLE FAILURE; HEART RIGHT VENTRICLE FUNCTION; HEART VENTRICLE SIZE; HUMAN; LUNG BLOOD VESSEL; MORBIDITY; MORTALITY; MULTICENTER STUDY (TOPIC); NON INVASIVE MEASUREMENT; PERICARDIAL EFFUSION; PROGNOSIS; PULMONARY HYPERTENSION; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; RISK FACTOR; SURVIVAL; TRICUSPID ANNULAR PLANE SYSTOLIC EXCURSION;

ECHOCARDIOGRAPHY; HUMANS; HYPERTENSION, PULMONARY; KAPLAN-MEIER ESTIMATE; LUNG; MAGNETIC RESONANCE IMAGING; PROGNOSIS; SEVERITY OF ILLNESS INDEX; VENTRICULAR DYSFUNCTION, RIGHT;

EID: 82555183025     PISSN: 09059180     EISSN: 16000617     Source Type: Journal    
DOI: 10.1183/09059180.00006511     Document Type: Review

References (78)

1. Galiè N, Manes A, Negro L, et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30: 394-403.
2. Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122: 156-163.
3. Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Artery Dis 2005; 16: 13-18.
4. 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.
5. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343-349.
6. Badano LP, Ginghina C, Easaw J, 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: 27-37.
7. Bogaard HJ, Abe K, Vonk Noordegraaf A, et al. The right ventricle under pressure: cellular and molecular mechanisms of right-heart failure in pulmonary hypertension. Chest 2009; 135: 794-804.
8. Haddad F, Ashley E, Michelakis ED. New insights for the diagnosis and management of right ventricular failure, from molecular imaging to targeted right ventricular therapy. Curr Opin Cardiol 2010; 25: 131-140.
9. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: Suppl. 1, S43-S54.
10. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425-1436.
11. Humbert M. Pulmonary arterial hypertension and chronic thromboembolic hypertension: pathophysiology. Eur Respir Rev
12. Handoko ML, de Man FS, Allaart CP, et al. Perspectives on novel therapeutic strategies for right heart failure in pulmonary arterial hypertension: lessons from the left heart. Eur Respir Rev 2010; 19: 72-82.
13. Sano M, Minamino T, Toko H, et al. p53-induced inhibition of Hif-1 causes cardiac dysfunction during pressure overload. Nature 2007; 446: 444-448.
14. Velez-Roa S, Ciarka A, Najem B, et al. Increased sympathetic nerve activity in pulmonary artery hypertension. Circulation 2004; 110: 1308-1312.
15. Ciarka A, Vachièry JL, Houssière A, et al. Atrial septostomy decreases sympathetic overactivity in pulmonary arterial hypertension. Chest 2007; 131: 1831-1837.
16. Galiè N, Manes A, Palazzini, et al. Pharmacological impact on right ventricular remodelling in pulmonary arterial hypertension. Eur Heart J 2007; 9: H68-H74.
17. Vonk-Noordegraaf A, Marcus JT, Gan CT, et al. Interventricular mechanical asynchrony due to right ventricular pressure overload in pulmonary hypertension plays an important role in impaired left ventricular filling. Chest 2005; 128: Suppl. 6, 628S-630S.
18. Gan CT, Lankhaar JW, Marcus JT, et al. Impaired left ventricular filling due to right-to-left ventricular interaction in patients with pulmonary arterial hypertension. Am J Physiol Heart Circ Physiol 2006; 290: H1528-H1533.
19. Marcus JT, Gan CT, Zwanenburg JJ, et al. Interventricular mechanical asynchrony in pulmonary arterial hypertension: leftto- right delay in peak shortening is related to right ventricular overload and left ventricular underfilling. J Am Coll Cardiol 2008; 51: 750-757.
20. Mauritz GJ, Marcus JT, Westerhof N, et al. Prolonged right ventricular post-systolic isovolumic period in pulmonary arterial hypertension is not a reflection of diastolic dysfunction. Heart 2011; 97: 473-478.
21. Hoeper MM, Lee SH, Voswinckel R, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006; 48: 2546-2552.
22. Hachulla E, Gressin V, Guillevin L, et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005; 52: 3792-3800.
23. Jurcut R, Giusca S, La Gerche A, et al. The echocardiographic assessment of the right ventricle: what to do in 2010? Eur J Echocardiogr 2010; 11: 81-96.
24. 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.
25. Raymond RJ, Hinderliter AL, Willis PW, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002; 39: 1214-1219.
26. Hinderliter AL, Willis PW 4th, Barst RJ, et al. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Primary Pulmonary Hypertension Study Group. Circulation 1997; 95: 1479-1486.
27. Bustamante-Labarta M, Perrone S, De Le Fuente RL, et al. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J Am Soc Echocardiogr 2002; 15: 1160-1164.
28. Ghio S, Pazzano AS, Klersy C, et al. Clinical and prognostic relevance of echocardiographic evaluation of right ventricular geometry in patients with idiopathic pulmonary arterial hypertension. Am J Cardiol 2011; 107: 628-632.
29. Ghio S, Klersy C, Magrini G, et al. Prognostic relevance of the echocardiographic assessment of right ventricular function in patients with idiopathic pulmonary arterial hypertension. Int J Cardiol 2010; 140: 272-278.
30. Forfia PR, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006; 174: 1034-1041.
31. Yeo TC, Dujardin KS, Tei C, et al. Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension. Am J Cardiol 1998; 81: 1157-1161.
32. Grapsa I, Pavlopoulos H, Dawson D, et al. Retrospective study of pulmonary hypertensive patients: is right ventricular myocardial performance index a vital prognostic factor? Hellenic J Cardiol 2007; 48: 152-160.
33. Sachdev A, Villarraga HR, Frantz RP, et al. Right ventricular strain for prediction of survival in pulmonary arterial hypertension. Chest 2011; 139: 1299-1309.
34. López-Candales A, Dohi K, Rajagopalan N, et al. Right ventricular dyssynchrony in patients with pulmonary hypertension is associated with disease severity and functional class. Cardiovasc Ultrasound 2005; 3: 23.
35. Alkon J, Humpl T, Manlhiot C, et al. Usefulness of the right ventricular systolic to diastolic duration ratio to predict functional capacity and survival in children with pulmonary arterial hypertension. Am J Cardiol 2010; 106: 430-436.
36. Hinderliter AL, Willis PW 4th, Long W, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Primary pulmonary hypertension. Am J Cardiol 1999; 84: 481-484.
37. Miller D, Farah MG, Liner A, et al. The relation between quantitative right ventricular ejection fraction and indices of tricuspid annular motion and myocardial performance. J Am Soc Echocardiogr 2004; 17: 443-447.
38. Hsiao SH, Lin SK, Wang WC, et al. Severe tricuspid regurgitation shows significant impact in the relationship among peak systolic tricuspid annular velocity, tricuspid annular plane systolic excursion, and right ventricular ejection fraction. J Am Soc Echocardiogr 2006; 19: 902-910.
39. Lopez-Candales A, Rajagopalan N, Saxena N, et al. Right ventricular systolic function is not the sole determinant of tricuspid annular motion. Am J Cardiol 2006; 98: 973-977.
40. Tei C, Dujardin KS, Hodge DO, et al. Doppler echocardiographic index for assessment of global right ventricular function. J Am Soc Echocardiogr 1996; 9: 838-847.
41. Pirat B, McCulloch ML, Zoghbi WA. Evaluation of global and regional right ventricular systolic function in patients with pulmonary hypertension using a novel speckle tracking method. Am J Cardiol 2006; 98: 699-704.
42. Amaki M, Nakatani S, Kanzaki H, et al. Usefulness of threedimensional echocardiography in assessing right ventricular function in patients with primary pulmonary hypertension. Hypertens Res 2009; 32: 419-422.
43. Benza R, Biederman R, Murali S, et al. Role of cardiac magnetic resonance imaging in the management of patients with pulmonary arterial hypertension. J Am Coll Cardiol 2008; 52: 1683-1692.
44. Bossone E, Bodini BD, Mazza A, et al. Pulmonary arterial hypertension: the key role of echocardiography. Chest 2005; 127: 1836-1843.
45. Coghlan JG, Davar J. How should we assess right ventricular function in 2008?. Eur Heart J 2007; 9: H22-H28.
46. Hagger D, Condliffe R, Woodhouse N, et al. Ventricular mass index correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis-associated pulmonary arterial hypertension. Rheumatology 2009; 48: 1137-1142.
47. Bradlow WM, Assomull RG, Williams F, et al. Cardiovascular magnetic resonance measured right ventricular mass influences prognosis in pulmonary arterial hypertension. Eur Heart J 2010; 31: 8.
48. van Wolferen SA, Marcus JT, Boonstra A, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007; 28: 1250-1257.
49. Soma S, Murali S, Benza R, et al. LGE in pulmonary hypertension predicts clinical events. J Cardiovasc Magn Resonance 2011; 13: Suppl. 1, P297.
50. McLure LE, Peacock AJ. Cardiac magnetic resonance imaging for the assessment of the heart and pulmonary circulation in pulmonary hypertension. Eur Respir J 2009; 33: 1454-1466.
51. Kovacs G, Reiter G, Reiter U, et al. The emerging role of magnetic resonance imaging in the diagnosis and management of pulmonary hypertension. Respiration 2008; 76: 458-470.
52. Ritchie M, Waggoner AD, Dávila-Román VG, et al. Echocardiographic characterization of the improvement in right ventricular function in patients with severe pulmonary hypertension after single-lung transplantation. J Am Coll Cardiol 1993; 22: 1170-1174.
53. Kasimir MT, Seebacher G, Jaksch P, et al. Reverse cardiac remodelling in patients with primary pulmonary hypertension after isolated lung transplantation. Eur J Cardiothorac Surg 2004; 26: 776-781.
54. Reesink HJ, Marcus JT, Tulevski II, et al. Reverse right ventricular remodeling after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: utility of magnetic resonance imaging to demonstrate restoration of the right ventricle. J Thorac Cardiovasc Surg 2007; 133: 58-64.
55. Galiè N, Hinderliter AL, Torbicki A, et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and Doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 1380-1386.
56. Wilkins MR, Paul GA, Strange JW, et al. Sildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) study. Am J Respir Crit Care Med 2005; 171: 1292-1297.
57. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896-903.
58. Barst RJ, Rubin LJ, McGoon MD, et al. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Ann Intern Med 1994; 121: 409-415.
59. Barst RJ, Rubin LJ, Long WA, 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: 296-302.
60. Keogh A, Strange G, Kotlyar E, et al. Survival after the initiation of combination therapy in patients with pulmonary arterial hypertension: an Australian collaborative report. Intern Med J 2011; 41: 235-244.
61. Sohn DW, Kim HK, Kim MA, et al. Beneficial and adverse effects of bosentan treatment in Korean patients with pulmonary artery hypertension. Korean Circ J 2009; 39: 105-110.
62. Sitbon O, Gressin V, Speich R, et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004; 170: 1212-1217.
63. Duffels MG, Hardziyenka M, Surie S, et al. Duration of right ventricular contraction predicts the efficacy of bosentan treatment in patients with pulmonary hypertension. Eur J Echocardiogr 2009; 10: 433-438.
64. Lammers AE, Hislop AA, Flynn Y, et al. Epoprostenol treatment in children with severe pulmonary hypertension. Heart 2007; 93: 739-743.
65. Ruiz MJ, Escribano P, Delgado JF, et al. Efficacy of sildenafil as a rescue therapy for patients with severe pulmonary arterial hypertension and given long-term treatment with prostanoids: 2- year experience. J Heart Lung Transplant 2006; 25: 1353-1357.
66. Dyer KL, Pauliks LB, Das B, et al. Use of myocardial performance index in pediatric patients with idiopathic pulmonary arterial hypertension. J Am Soc Echocardiogr 2006; 19: 21-27.
67. Allanore Y, Meune C, Vignaux O, et al. Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and tissue-Doppler echography study. J Rheumatol 2006; 33: 2464-2469.
68. Seyfarth HJ, Pankau H, Hammerschmidt S, et al. Bosentan improves exercise tolerance and Tei index in patients with pulmonary hypertension and prostanoid therapy. Chest 2005; 128: 709-713.
69. Sebbag I, Rudski LG, Therrien J, et al. Effect of chronic infusion of epoprostenol on echocardiographic right ventricular myocardial performance index and its relation to clinical outcome in patients with primary pulmonary hypertension. Am J Cardiol 2001; 88: 1060-1063.
70. Chin KM, Kingman M, de Lemos JA, et al. Changes in right ventricular structure and function assessed using cardiac magnetic resonance imaging in bosentan-treated patients with pulmonary arterial hypertension. Am J Cardiol 2008; 101: 1669-1672.
71. van Wolferen SA, Boonstra A, Marcus JT, et al. Right ventricular reverse remodelling after sildenafil in pulmonary arterial hypertension. Heart 2006; 92: 1860-1861.
72. Roeleveld RJ, Vonk-Noordegraaf A, Marcus JT, et al. Effects of epoprostenol on right ventricular hypertrophy and dilatation in pulmonary hypertension. Chest 2004; 125: 572-579.
73. Basil A, Liu T, Arkles J, et al. Serial changes in TAPSE correlate with functional assessment and clinical events in patients with PAH. Am J Respir Crit Care Med 2009; 179: A4882.
74. Nath J, Demarco T, Hourigan L, et al. Correlation between right ventricular indices and clinical improvement in epoprostenol treated pulmonary hypertension patients. Echocardiography 2005; 22: 374-379.
75. Blalock SE, Matulevicius S, Mitchell LC, et al. Long-term outcomes with ambrisentan monotherapy in pulmonary arterial hypertension. J Card Fail 2010; 16: 121-127.
76. Michelakis ED, Tymchak W, Noga M, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003; 108: 2066-2069.
77. 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 2008; 371: 2093-2100.
78. Banerjee D, Haddad F, Zamanian RT, et al. Right ventricular failure: a novel era of targeted therapy. Curr Heart Fail Rep 2010; 7: 202-211.