Diagnosis and treatment of secondary (non-category 1) pulmonary hypertension

Circulation

Volumn 118, Issue 21, 2008, Pages 2190-2199

  Rich, Stuart ^a,c,d   Rabinovitch, Marlene ^b,c  

^a UNIVERSITY OF CHICAGO   (United States)

^b STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^c Pulmonary Vascular Research Institute   (United States)

^d UNIVERSITY OF CHICAGO   (United States)

Author keywords

Heart failure; Hypertension; Pulmonary; Pulmonary heart disease

Indexed keywords

ADENOSINE; ANTIINFLAMMATORY AGENT; BOSENTAN; DIURETIC AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; IMMUNOSUPPRESSIVE AGENT; NITRIC OXIDE; PHOSPHODIESTERASE V INHIBITOR; PROSTACYCLIN; SILDENAFIL; VASODILATOR AGENT;

ARTERY INTIMA PROLIFERATION; ARTICLE; CHRONIC OBSTRUCTIVE LUNG DISEASE; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; DIAGNOSTIC PROCEDURE; DISEASE CLASSIFICATION; DOPPLER ECHOCARDIOGRAPHY; ELECTROCARDIOGRAM; EVALUATION; HEART LEFT VENTRICLE CONTRACTION; HEART LEFT VENTRICLE EJECTION FRACTION; HEART LEFT VENTRICLE FUNCTION; HEART LEFT VENTRICLE HYPERTROPHY; HEMODYNAMICS; HUMAN; HYPERTROPHY; IMMUNOSUPPRESSIVE TREATMENT; INSULIN RESISTANCE; INTERSTITIAL LUNG DISEASE; INTIMA; LUNG ARTERY PRESSURE; LUNG EDEMA; LUNG FUNCTION TEST; LUNG PRESSURE; MITRAL VALVE REPLACEMENT; MITRAL VALVE STENOSIS; NONHUMAN; PRIORITY JOURNAL; PULMONARY ARTERY OCCLUSION PRESSURE; PULMONARY HYPERTENSION; PULMONARY VEIN; THROMBOEMBOLISM; TREATMENT PLANNING; VENOUS PRESSURE;

DIAGNOSIS, DIFFERENTIAL; HUMANS; HYPERTENSION, PULMONARY; PRACTICE GUIDELINES AS TOPIC;

EID: 57449105233     PISSN: 00097322     EISSN: 15244539     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.107.723007     Document Type: Article

References (80)

1. Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126:7S-10S.
2. Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917-1928.
3. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425-1436.
4. Macchia A, Marchioli R, Marfisi R, Scarano M, Levantesi G, Tavazzi L, Tognoni G. A meta-analysis of trials of pulmonary hypertension: a clinical condition looking for drugs and research methodology. Am Heart J. 2007;153:1037-1047.
5. Mahoney P, Loh E, Blitz L, Herrmann H. Hemodynamic effects of inhaled nitric oxide in women wim mitral stenosis and pulmonary hypertension. Am J Cardiol. 2001;87:188-192.
6. Redfield MM. Understanding "diastolic" heart failure. N Engl J Med. 2004;350:1930-1931.
7. Willens HJ, Kessler KM. Severe pulmonary hypertension associated with diastolic left ventricular dysfunction. Chest. 1993;103:1877-1883.
8. Wood P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br Heart J. 1958;20:557-568.
9. Wagenvoort C, Wagenvoort N. Pathology of Pulmonary Hypertension. 2nd ed. New York, NY: John Wiley & Sons; 1977.
10. Hansmann G, Wagner RA, Schellong S, Perez VA, Urashima T, Wang L, Sheikh AY, Suen RS, Stewart DJ, Rabinovitch M. Pulmonary arterial hypertension is linked to insulin resistance and reversed by peroxisome proliferator-activated receptor-gamma activation. Circulation. 2007;115:1275-1284.
11. Runo J, Vnencak-Jones C, Prince M, Loyd J, Wheeler L, Robbins I, Lane K, Newman J, Johnson J, Nichols WC, Phillips JA III. Pulmonary venoocclusive disease caused by an inherited mutation in bone morphogenetic protein receptor II. Am J Respir Crit Care Med. 2003;167:889-894.
12. Angeja BG, Grossman W. Evaluation and management of diastolic heart failure. Circulation. 2003;107:659-663.
13. Rich S, Dantzker R, Ayres S, Bergofsky E, Brundage B, Detre K, Fishman A, Goldring R, Groves B, Koerner S, Levy P, Reid L, Vreim C, Williams G. Primary pulmonary hypertension: a national prospective study. Ann Intern Med. 1987;107:216-223.
14. Zile MR, Brutsaert DL. New concepts in diastolic dysfunction and diastolic heart failure, part I: diagnosis, prognosis, and measurements of diastolic function. Circulation. 2002;105:1387-1393.
15. Ghio S, Gavazzi A. Campana C, Inserra C, Klersy C, Sebastiani R, Arbuslini E, Recusani F, Tavazzi L. Independent and additive prognostic-value of right ventricular function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol. 2001;37:183-188.
16. Alexopoulos D, Lazzam C, Borrica S, Fiedler L, Ambrose JA. Isolated chronic mitral regurgitation with preserved systolic left ventricular function and severe pulmonary hypertension. J Am Coll Cardiol. 1989;14:319-322.
17. Malouf J, Enriguez-Sarano M, Pellikka P, Oh J, Bailey K, Chandrasekaran K, Mullany C, Tajik A. Severe pulmonary hypertension in patients with severe aortic valve stenosis: clinical profile and prognostic implications. J Am Coll Cardiol. 2002;40:789-795.
18. Appleton CP, Firstenberg MS, Garcia MJ, Thomas JD. The echo-Doppler evaluation of left ventricular diastolic function: a current perspective. Cardiol Clin. 2000;18:513-546.
19. Louie E, Rich S, Brundage B. Doppler echocardiographic assessment of impaired left ventricular filling with right ventricular pressure overload due to primary pulmonary hypertension. J Am Coll Cardiol. 1986;8:1298-1235.
20. Eduardo D, Flores R. Relation of mean pulmonary arterial wedge pressure and left ventricular end-diastolic pressure. Am J Cardiol. 1990;66:1532-1533.
21. Morris AH, Chapman RH. Wedge pressure confirmation by aspiration of pulmonary capillary blood. Crit Care Med. 1985;13:756-759.
22. Al-Kharrat T, Zarich S, Amoateng-Adiepong Y, Manthous CA. Analysis of observer variability in measurement of pulmonary artery occlusion pressures. Am J Respir Crit Care Med. 1999;160:415-420.
23. Du L, Sullivan C, Chu D, Cho A, Kido M, Wolf P, Yuan J, Deutsch R, Jamieson S, Thistlethwaite P. Signaling molecules in nonfamilial pulmonary hypertension. N Engl J Med. 2003;348:500-509.
24. Senni M, Redfield MM. Heart failure with preserved systolic function: a different natural history? J Am Coll Cardiol. 2001;38:1277-1282.
25. Nootens M, Wolfkiel CJ, Chomka EV, Rich S. Understanding right and left ventricular systolic function and interactions at rest and with exercise in primary pulmonary hypertension. Am J Cardiol. 1995;75:374-377.
26. Costard-Jackle A, Fowler MB. Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol. 1992;19:48-54.
27. Givertz MM, Hare JM, Loh E, Gauthier DF, Colucci WS. Effect of bolus milrinone on hemodynamic variables and pulmonary vascular resistance in patients with severe left ventricular dysfunction: a rapid test for reversibility of pulmonary hypertension. J Am Coll Cardiol. 1996;28:1775-1780.
28. LaBourene JI CJ, Johnson DJ, Mehra A, Keeley FW, Rabinovitch M. Alterations in elastin and collagen related to the mechanism of progressive pulmonary venous obstruction in a piglet model: a hemodynamic, ultrastructural, and biochemical study. Circ Res. 1990;66:438-456.
29. Braunwald E, Braunwald N, Ross J, Morrow AG. Effects of mitral valve replacement on pulmonary vascular dynamics of patients with pulmonary hypertension. N Engl J Med. 1965;273:509-514.
30. Zener JC, Hancock EW, Shumway NE, Harrison DC. Regression of extreme pulmonary hypertension after mitral valve surgery. Am J Cardiol. 1972;30:820-826.
31. Levine M, Weinstein J, Diver D, Berman AD, Wyman RM, Cunningham MJ, Safian RD, Grossman W, McKay RG. Progressive improvement in pulmonary vascular resistance following percutaneous mitral valvuloplasty. Circulation. 1989;79:1061-1067.
32. Fawzy ME, Hassan W, Stefadouros M, Moursi M, El Shaer F, Chaudhary MA. Prevalence and fate of severe pulmonary hypertension in 559 consecutive patients with severe rheumatic mitral stenosis undergoing mitral balloon valvotomy. J Heart Valve Dis. 2004;13:942-947.
33. Packer M, McMurray J, Massie BM, Caspi A, Charlon V, Cohen-Solal A, Kiowski W, Kostuk W, Krum H, Levine B, Rizzon P, Soler J, Swedberg K, Anderson S, Demets DL. Clinical effects of endothelin receptor antagonism with bosentan in patients with severe chronic heart failure: results of a pilot study. J Card Fail. 2005;11:12-20.
34. Califf RM, Adams KF, McKenna WJ, Gheorghiade M, Uretsky BF, McNulty SE, Darius H, Schulman K, Zannad F, Handberg-Thurmond E, Harrell FE Jr, Wheeler W, Soler-Soler J, Swedberg K. A randomized controlled trial of epoprostenol therapy for severe congestive heart failure: the Flolan International Randomized Survival Trial (FIRST). Am Heart J. 1997;134:44-54.
35. Guazzi M, Tumminello G, Di Marco F, Fiorentini C, Guazzi MD. The effects of phosphodiesterase-5 inhibition with sildenafil on pulmonary hemodynamics and diffusion capacity, exercise ventilatory efficiency. and oxygen uptake kinetics in chronic heart failure. J Am Coll Cardiol. 2004;44:2339-2348.
36. Guazzi M, Samaja M, Arena R, Vicenzi M, Guazzi MD. Long-term use of sildenafil in the therapeutic management of heart failure. J Am Coll Cardiol. 2007;50:2136-2144.
37. Palmer S, Robinson L, Wang A, Gossage J, Bashore T, Tapson V. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest. 1998;113:237-240.
38. Humbert M, Maître S, Capron F, Rain B, Musset D, Simonneau G. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med. 1998;157:1681-1685.
39. Preston I, Klinger J, Houtchen J, Nelson D, Mehta S, Hill N. Pulmonary edema caused by inhaled nitric oxide therapy in two patients with pulmonary hypertension associated with CREST syndrome. CHEST. 2002;121:656-659.
40. Rabinovitch MGW, Nadas AS, Miettinen OS, Reid L. Rat pulmonary circulation after chronic hypoxia: hemodynamic and structural features. Am J Physiol. 1979;236:H818-H827.
41. Arias-Stella J. The terminal portion of the pulmonary arterial tree in people native to high altitudes. Circulation. 1963;28:915-925.
42. Weissmann N, Grimminger F, Seeger W. Hypoxia in lung vascular biology and disease. Cardiovasc Res. 2006;71:618-619.
43. Senior R, Anthonisen N. Chronic obstructive pulmonary disease (COPD). Am J Respir Crit Care Med. 1998;157:S139-S147.
44. Oswald-Mammosser M, Weitzenblum E, Quoix E, Moser G, Chaouat A, Charpentier C, Kessler R. Prognostic factors in COPD patients receiving long-term oxygen therapy. Chest. 1995;107:1193-1198.
45. Torbicki A, Kurzyna M, Kuca P, Fijalkowska A, Sikora J, Florczyk M, Pruszczyk P, Burakowski J, Wawrzynska L. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003;108:844-848.
46. Wright JL, Levy RD, Churg A. Pulmonary hypertension in chronic obstructive pulmonary disease: current theories of pathogenesis and their implications for treatment. Thorax. 2005;60:605-609.
47. Kawakami Y, Kishi F, Yamamoto H, Miyamoto K. Relation of oxygen delivery, mixed venous oxygenation, and pulmonary hemodynamics to prognosis in chronic obstructive pulmonary disease. N Engl J Med. 1983;308:1045-1049.
48. Dallari R, Barozzi G, Merighi GP, Grandi P, Manzotti M, Tartoni P. Predictors of survival in subjects with chronic obstructive pulmonary disease treated with long-term oxygen therapy. Respiration. 1994;61:8-13.
49. Archer SL, Mike D, Crow J, Long W, Weir EK. A placebo-controlled trial of prostacyclin in acute respiratory failure in COPD. Chest. 1996;109:750-755.
50. Nocturnal Oxygen Therapy Trial Group. Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease. Ann Intern Med. 1980;93:391-398.
51. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985;131:493-498.
52. Stevens D, Sharma K, Szidon P, Rich S, McLaughlin V, Kesten S. Severe pulmonary hypertension associated with COPD. Ann Transplant. 2000;5:8-12.
53. Marcos E, Adnot S, Pham M, Nosjean A, Raffestin B, Hamon M, Eddahibi S. Serotonin transporter inhibitors protect against hypoxic pulmonary hypertension. Am J Respir Crit Care Med. 2003;168:487-493.
54. Harari S, Simonneau G, DeJuli E, Brenot F, Cerrina J, Colombo P, Gronda E, Micallef E, Parent F, Dartevelle P. Prognostic value of pulmonary hypertension in patients with chronic interstitial lung disease referred for lung or heart-lung transplantation. J Heart Lung Transplant. 1997;16:460-463.
55. Cool C, Kennedy D, Voelkel N, Tuder R. Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection. Hum Pathol. 1997;28:434-442.
56. Launay DML, Hachulla E, Pagnoux C, de Groote P, Remy-Jardin M, Matran R, Lambert M, Queyrel V, Morell-Dubois S, Guillevin L, Hatron PY. Prevalence and characteristics of moderate to severe pulmonary hypertension in systemic sclerosis with and without interstitial lung disease. J Rheumatol. 2007;34:1005-1511.
57. Alkotob ML, Soltani P, Sheatt MA, Katsetos MC, Rothfield N, Hager WD, Foley RJ, Silverman DI. Reduced exercise capacity and stress-induced pulmonary hypertension in patients with scleroderma. Chest. 2006;130:176-181.
58. Schachna L, Wigley FM, Chang B, White B, Wise RA, Gelber AC. Age and risk of pulmonary arterial hypertension in scleroderma. Chest. 2003;124:2098-2104.
59. Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, Krishnan JA, Wigley F, Hassoun PM. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;54:3043-3050.
60. Nathan SD, Noble PW, Tuder RM. Idiopathic pulmonary fibrosis and pulmonary hypertension: connecting the dots. Am J Respir Crit Care Med. 2007;175:875-880.
61. Fell CD, Martinez FJ. The impact of pulmonary arterial hypertension on idiopathic pulmonary fibrosis. Chest. 2007;131:641-643.
62. Lettieri CJ, Naman SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest. 2006;129:746-752.
63. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement: American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000;161:646-664.
64. Weissmann N, Özlem Kocer BG, Nollen M, Hackemack S, Ghofrani HA, Schermuly RT, Butrous G, Schulz A, Roth M. Hypoxia-induced pulmonary hypertension: different impact of iloprost, sildenafil, and nitric oxide. Respir Med. 2007;101:2125-2132.
65. Ghofrani H, Wiedemann R, Rose F, Schermuly R, Olschewski H, Weissmann N, Gunther A, Walmrath D, Seeger W, Grimminger F. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomized controlled trial. Lancet. 2002;360:895-900.
66. King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, Stahler G, Leconte I, Roux S, Raghu G. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopamic pulmonary fibrosis. Am J Respir Crit Care Med. 2008;177:75-81.
67. Noth I. Recent advances in idiopathic pulmonary fibrosis. Chest. 2007;132:637-650.
68. Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation. 2006;113:2011-2020.
69. Fedullo P, Auger W, Kerr K, Rubin L. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2001;345:1465-1472.
70. Moser KM. Pulmonary vascular lesions occurring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest. 1993;103:685-692.
71. Galie N, Kim NH. Pulmonary microvascular disease in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006;3:571-576.
72. Archibald C, Auger W, Fedullo P, Channick R, Kerr K, Jamieson S, Kapelanski D, Watt C, Moser K. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med. 1999;160:523-528.
73. Bresser P, Pepke-Zaba J, Jais X, Humbert M, Hoeper MM. Medical therapies for chronic thromboembolic pulmonary hypertension: an evolving treatment paradigm. Proc Am Thorac Soc. 2006;3:594-600.
74. Peacock A, Simonneau G, Rubin L. Controversies, uncertainties and future research on the treatment of chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc. 2006;3:608-614.
75. Ghofrani H, Schermuly R, Rose F, Wiedemann R, Kohstall M, Kreckel A, Olschewski H, Weissmann N, Enke B, Ghofrani S, Seeger W, Grimminger F. Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2003;167:1139-1141.
76. Hoeper MM, Kramm T, Wilkens H, Schulze C, Schafers HJ, Welte T, Mayer E. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest. 2005;128:2363-2367.
77. Fisher KA, Serlin DM, Wilson KC, Walter RE, Berman JS, Farber HW. Sarcoidosis-associated pulmonary hypertension: outcome with long-term epoprostenol treatment. Chest. 2006;130:1481-1488.
78. McLaughlin V, Genthner D, Panella M, Hess D, Rich S. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 1999;130:740-743.
79. Pietra GG CF, Stewart S, Leone O, Humbert M, Robbins IM, Reid LM, Tuder RM. Pathologic assessment of vasculopathies in pulmonary hypertensio. J Am Coll Cardiol. 2004;43:25S-32S.
80. Santos S, Peinado VI, Ramírez J, Melgosa T, Roca J, Rodriguez-Roisin R, Barberà JA. Characterization of pulmonary vascular remodelling in smokers and patients with mild COPD. Eur Respir J. 2002;19:632-638.