Pulmonary Arterial Hypertension Article Recommendations

Classification

Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004. PubMed Abstract

Epidemiology

Newman JH, Wheeler L, Lane KB, Loyd E, Gaddipati R, Phillips JA, 3rd, Loyd JE. Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred. N Engl J Med 2001. PubMed Abstract

Elliott G, Alexander G, Leppert M, Yeates S, Kerber R. Coancestry in apparently sporadic primary pulmonary hypertension. Chest 1995. PubMed Abstract

Pathology

Yi ES, Kim H, Ahn H, Strother J, Morris T, Masliah E, Hansen LA, Park K, Friedman PJ. Distribution of obstructive intimal lesions and their cellular phenotypes in chronic pulmonary hypertension. A morphometric and immunohistochemical study. Am J Respir Crit Care Med 2000. PubMed Abstract

Tuder RM, Groves B, Badesch DB, Voelkel NF. Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension. Am J Pathol 1994. PubMed Abstract

Lee SD, Shroyer KR, Markham NE, Cool CD, Voelkel NF, Tuder RM. Monoclonal endothelial cell proliferation is present in primary but not secondary pulmonary hypertension. J Clin Invest 1998. PubMed Abstract

Loyd JE, Atkinson JB, Pietra GG, Virmani R, Newman JH. Heterogeneity of pathologic lesions in familial primary pulmonary hypertension. Am Rev Respir Dis 1988. PubMed Abstract

Pathogenesis

Loscalzo J. Endothelial dysfunction in pulmonary hypertension. N Engl J Med 1992. PubMed Abstract

Voelkel NF, Tuder RM. Cellular and molecular mechanisms in the pathogenesis of severe pulmonary hypertension. Eur Respir J 1995. PubMed Abstract

Archer S, Rich S. Primary pulmonary hypertension: a vascular biology and translational research "Work in progress". Circulation 2000. PubMed Abstract

Budhiraja R, Tuder RM, Hassoun PM. Endothelial dysfunction in pulmonary hypertension. Circulation 2004. PubMed Abstract

Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med 2004. PubMed Abstract

Genetics

Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000. PubMed Abstract

Lane KB, Machado RD, Pauciulo MW, Thomson JR, Phillips JA, 3rd, Loyd JE, Nichols WC, Trembath RC. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet 2000. PubMed Abstract

Thomson JR, Machado RD, Pauciulo MW, Morgan NV, Humbert M, Elliott GC, Ward K, Yacoub M, Mikhail G, Rogers P, Newman J, Wheeler L, Higenbottam T, Gibbs JS, Egan J, Crozier A, Peacock A, Allcock R, Corris P, Loyd JE, Trembath RC, Nichols WC. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-beta family. J Med Genet 2000. PubMed Abstract

Coagulation

Egermayer P, Town GI, Peacock AJ. Role of serotonin in the pathogenesis of acute and chronic pulmonary hypertension. Thorax 1999. PubMed Abstract

Herve P, Launay JM, Scrobohaci ML, Brenot F, Simonneau G, Petitpretz P, Poubeau P, Cerrina J, Duroux P, Drouet L. Increased plasma serotonin in primary pulmonary hypertension. Am J Med 1995. PubMed Abstract

Eddahibi S, Humbert M, Fadel E, Raffestin B, Darmon M, Capron F, Simonneau G, Dartevelle P, Hamon M, Adnot S. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001. PubMed Abstract

Marcos E, Adnot S, Pham MH, Nosjean A, Raffestin B, Hamon M, Eddahibi S. Serotonin transporter inhibitors protect against hypoxic pulmonary hypertension. Am J Respir Crit Care Med 2003. PubMed Abstract

Endothelium

Giaid A, Yanagisawa M, Langleben D, Michel RP, Levy R, Shennib H, Kimura S, Masaki T, Duguid WP, Stewart DJ. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993. PubMed Abstract

Christman BW, McPherson CD, Newman JH, King GA, Bernard GR, Groves BM, Loyd JE. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med 1992. PubMed Abstract

Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995. PubMed Abstract

Tuder RM, Cool CD, Geraci MW, Wang J, Abman SH, Wright L, Badesch D, Voelkel NF. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999. PubMed Abstract

Potassium channel regulation

Yuan JX, Aldinger AM, Juhaszova M, Wang J, Conte JV, Jr., Gaine SP, Orens JB, Rubin LJ. Dysfunctional voltage-gated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation 1998. PubMed Abstract

Weir EK, Archer SL. The mechanism of acute hypoxic pulmonary vasoconstriction: the tale of two channels. Faseb J 1995. PubMed Abstract  

Post JM, Hume JR, Archer SL, Weir EK. Direct role for potassium channel inhibition in hypoxic pulmonary vasoconstriction. Am J Physiol 1992. PubMed Abstract

Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004. PubMed Abstract

McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, Loyd JE. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004. PubMed Abstract

Barst RJ, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, Gaine S. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004. PubMed Abstract

Roeleveld RJ, Marcus JT, Boonstra A, Postmus PE, Marques KM, Bronzwaer JG, Vonk-Noordegraaf A. A comparison of noninvasive MRI-based methods of estimating pulmonary artery pressure in pulmonary hypertension. J Magn Reson Imaging 2005. PubMed Abstract

Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, Pochettino A, Kotloff RM. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003. PubMed Abstract

Sitbon O, Brenot F, Denjean A, Bergeron A, Parent F, Azarian R, Herve P, Raffestin B, Simonneau G. Inhaled nitric oxide as a screening vasodilator agent in primary pulmonary hypertension. A dose-response study and comparison with prostacyclin. Am J Respir Crit Care Med 1995. PubMed Abstract

Treatment—General

Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004. PubMed Abstract

Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005. PubMed Abstract

Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004. PubMed Abstract

Mereles D, Ehlken N, Kreuscher S, Ghofrani S, Hoeper MM, Halank M, Meyer FJ, Karger G, Buss J, Juenger J, Holzapfel N, Opitz C, Winkler J, Herth FF, Wilkens H, Katus HA, Olschewski H, Grünig E. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation. 2006 Oct 3 ; 114(14):1482-9 PubMed Abstract

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. PubMed Abstract

Treatment—Prostanoids—Epoprostenol

Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH. 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. PubMed Abstract

McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002. PubMed Abstract

Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, Rainisio M, Simonneau G. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002. PubMed Abstract

Treatment—Prostanoids—Treprostinil

Simonneau G, Barst RJ, Galie N, Naeije R, Rich S, Bourge RC, Keogh A, Oudiz R, Frost A, Blackburn SD, Crow JW, Rubin LJ. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002. PubMed Abstract

Gomberg-Maitland M, Tapson VF, Benza RL, McLaughlin VV, Krichman A, Widlitz AC, Barst RJ. Transition from intravenous epoprostenol to intravenous treprostinil in pulmonary hypertension. Am J Respir Crit Care Med 2005. PubMed Abstract

Tapson VF, Gomberg-Maitland M, McLaughlin VV, Benza RL, Widlitz AC, Krichman A, Barst RJ. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006. PubMed Abstract

Voswinckel R, Enke B, Reichenberger F, Kohstall M, Kreckel A, Krick S, Gall H, Gessler T, Schmehl T, Ghofrani HA, Schermuly RT, Grimminger F, Rubin LJ, Seeger W, Olschewski H. Favorable effects of inhaled treprostinil in severe pulmonary hypertension: results from randomized controlled pilot studies.  J Am Coll Cardiol. 2006 Oct 17 ; 48(8):1672-81 PubMed Abstract

Channick RN, Olschewski H, Seeger W, Staub T, Voswinckel R, Rubin LJ J Am Coll Cardiol. Safety and efficacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension. 2006 Oct 3 ; 48(7):1433-7 PubMed Abstract

Treatment—Prostanoids—Iloprost

Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, Nikkho S, Speich R, Hoeper MM, Behr J, Winkler J, Sitbon O, Popov W, Ghofrani HA, Manes A, Kiely DG, Ewert R, Meyer A, Corris PA, Delcroix M, Gomez-Sanchez M, Siedentop H, Seeger W. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 2002. PubMed Abstract

Treatment—Endothelin Receptor Antagonists

Lee SH, Channick RN. Endothelin antagonism in pulmonary arterial hypertension. Semin Respir Crit Care Med 2005. PubMed Abstract

Rich S, McLaughlin VV. Endothelin receptor blockers in cardiovascular disease. Circulation 2003. PubMed Abstract

Treatment—Endothelin Receptor Antagonists—Bosentan

Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, Pulido T, Frost A, Roux S, Leconte I, Landzberg M, Simonneau G. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002. PubMed Abstract

Sitbon O, Badesch DB, Channick RN, Frost A, Robbins IM, Simonneau G, Tapson VF, Rubin LJ. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest 2003. PubMed Abstract

McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N, Rainisio M, Simonneau G, Rubin LJ. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005. PubMed Abstract

Provencher S, Sitbon O, Humbert M, Cabrol S, Jais X, Simonneau G. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J 2006. PubMed Abstract

Treatment—Endothelin Receptor Antagonists—Sitaxsentan

Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, Naeije R, Galie N. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol 2006. PubMed Abstracts

Langleben D, Brock T, Dixon R, Barst R. STRIDE 1: Effects of the Selective ETA Receptor Antagonist, Sitaxsentan Sodium, in a Patient Population with Pulmonary Arterial Hypertension that meets Traditional Inclusion Criteria of Previous Pulmonary Arterial Hypertension Trials. J Cardiovasc Pharmacol 2004. PubMed Abstracts

Langleben D, Hirsch AM, Shalit E, Lesenko L, Barst RJ. Sustained symptomatic, functional, and hemodynamic benefit with the selective endothelin-A receptor antagonist, sitaxsentan, in patients with pulmonary arterial hypertension: a 1-year follow-up study. Chest 2004. PubMed Abstracts

Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, McLaughlin V, Hill N, Tapson VF, Robbins IM, Zwicke D, Duncan B, Dixon RA, Frumkin LR. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004. PubMed Abstracts

Treatment—Endothelin Receptor Antagonists—Ambrisentan

Galie N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, Frost AE, Zwicke D, Naeije R, Shapiro S, Olschewski H, Rubin LJ. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005. PubMed Abstracts

Treatment—Phosphodiesterase-5 Inhibitors—Sildenafil

Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, Fleming T, Parpia T, Burgess G, Branzi A, Grimminger F, Kurzyna M, Simonneau G. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005. PubMed Abstracts

Ruiz MJ, Escribano P, Delgado JF, Jiménez C, Tello R, Gómez MA, de la Calzada CS J.. Efficacy of sildenafil as a rescue therapy for patients with severe pulmonary arterial hypertension and given long-term treatment with prostanoids: 2-year experience. Heart Lung Transplant. 2006 Nov ; 25(11):1353-7 PubMed Abstracts

Treatment—Combination Therapy

Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005. PubMed Abstracts

Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, Seeger W, Grimminger F. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002. PubMed Abstracts

Hoeper MM, Taha N, Bekjarova A, Gatzke R, Spiekerkoetter E. Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids. Eur Respir J 2003. PubMed Abstracts

Treatment—Atrial Septostomy

Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med 2001. PubMed Abstracts

Treatment—Lung Transplantation

Conte JV, Gaine SP, Orens JB, Harris T, Rubin LJ. The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation. J Heart Lung Transplant 1998. PubMed Abstracts

Pulmonary Hypertension in Children

Rosenzweig EB, Barst RJ. Idiopathic pulmonary arterial hypertension in children. Curr Opin Pediatr 2005. PubMed Abstracts

Yetman AT, Taylor AL, Doran A, Ivy DD. Utility of cardiopulmonary stress testing in assessing disease severity in children with pulmonary arterial hypertension. Am J Cardiol 2005. PubMed Abstracts

Rosenzweig EB, Widlitz AC, Barst RJ. Pulmonary arterial hypertension in children. Pediatr Pulmonol 2004. PubMed Abstracts

Lammers A, Hislop A, Flynn Y, Haworth SG Epoprostenol treatment in children with severe pulmonary hypertension.Heart. 2006 Oct 25. PubMed Abstracts

Congenital Systemic to Pulmonary Shunts

Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999. PubMed Abstracts

Toxin/drug-related pulmonary hypertension

Rich S, Rubin L, Walker AM, Schneeweiss S, Abenhaim L. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest 2000. PubMed Abstracts

Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, Higenbottam T, Oakley C, Wouters E, Aubier M, Simonneau G, Begaud B. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996. PubMed Abstracts

Garcia-Dorado D, Miller DD, Garcia EJ, Delcan JL, Maroto E, Chaitman BR. An epidemic of pulmonary hypertension after toxic rapeseed oil ingestion in Spain. J Am Coll Cardiol 1983. PubMed Abstracts

Rothman RB, Ayestas MA, Dersch CM, Baumann MH. Aminorex, fenfluramine, and chlorphentermine are serotonin transporter substrates. Implications for primary pulmonary hypertension. Circulation 1999. PubMed Abstract

Collagen Vascular Disease and Pulmonary Hypertension—General

Coghlan JG, Handler C. Connective tissue associated pulmonary arterial hypertension. Lupus 2006. PubMed Abstracts

Collagen Vascular Disease and Pulmonary Hypertension—Prevalence/Screening

Chung SM, Lee CK, Lee EY, Yoo B, Lee SD, Moon HB. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol 2006. PubMed Abstracts

Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, Kahan A, Cabane J, Frances C, Launay D, Mouthon L, Allanore Y, Tiev KP, Clerson P, de Groote P, Humbert M. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005. PubMed Abstracts

Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, Black CM, Coghlan JG. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003. PubMed Abstracts

Pope JE, Lee P, Baron M, Dunne J, Smith D, Docherty PS, Bookman A, Abu-Hakima M. Prevalence of elevated pulmonary arterial pressures measured by echocardiography in a multicenter study of patients with systemic sclerosis. J Rheumatol 2005. PubMed Abstracts

Wigley FM, Lima JA, Mayes M, McLain D, Chapin JL, Ward-Able C. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary health care level of community-based rheumatologists (the UNCOVER study). Arthritis Rheum 2005. PubMed Abstracts

Johnson SR, Gladman DD, Urowitz MB et al. Pulmonary hypertension in systemic lupus. Lupus 2004; 13(7):506-509. PubMed Abstracts

Williams MH, Handler CE, Akram R et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 2006; 27(12):1485-1494. PubMed Abstracts

Alkotob ML, Soltani P, Sheatt MA et al. Reduced exercise capacity and stress-induced pulmonary hypertension in patients with scleroderma. Chest 2006; 130(1):176 PubMed Abstracts

Collagen Vascular Disease and Pulmonary Hypertension—Predictors and Risk Factors

Chang B, Schachna L, White B, Wigley FM, Wise RA. Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 2006. PubMed Abstracts

MacGregor AJ, Canavan R, Knight C, Denton CP, Davar J, Coghlan J, Black CM. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford) 2001. PubMed Abstracts

Steen V, Medsger TA, Jr. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003. PubMed Abstracts

Collagen Vascular Disease and Pulmonary Hypertension—Treatment

Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, Rich S, Barst RJ, Barrett PS, Kral KM, Jobsis MM, Loyd JE, Murali S, Frost A, Girgis R, Bourge RC, Ralph DD, Elliott CG, Hill NS, Langleben D, Schilz RJ, McLaughlin VV, Robbins IM, Groves BM, Shapiro S, Medsger TA, Jr. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000. PubMed Abstracts

Kawut SM, Taichman DB, Archer-Chicko CL, Palevsky HI, Kimmel SE. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003. PubMed Abstracts

Kuhn KP, Byrne DW, Arbogast PG, Doyle TP, Loyd JE, Robbins IM. Outcome in 91 consecutive patients with pulmonary arterial hypertension receiving epoprostenol. Am J Respir Crit Care Med 2003. PubMed Abstracts

Oudiz RJ, Schilz RJ, Barst RJ, Galie N, Rich S, Rubin LJ, Simonneau G. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004. PubMed Abstracts

Williams MH, Das C, Handler CE, Akram MR, Davar J, Denton CP, Smith CJ, Black CM, Coghlan JG. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006. PubMed Abstracts

Denton CP, Humbert M, Rubin L et al. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 2006; 65(10):1336-1340. PubMed Abstracts

Sanchez O, Sitbon O, Jais X et al. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 2006; 130(1):182-189. PubMed Abstracts

 

Hepatic Disease and Pulmonary Hypertension

Budhiraja R, Hassoun PM. Portopulmonary hypertension: a tale of two circulations. Chest 2003. PubMed Abstracts

Hoeper MM, Halank M, Marx C, Hoeffken G, Seyfarth HJ, Schauer J, Niedermeyer J, Winkler J. Bosentan therapy for portopulmonary hypertension. Eur Respir J 2005. PubMed Abstracts

Krowka MJ. Portopulmonary hypertension: diagnostic advances and caveats. Liver Transpl 2003. PubMed Abstracts

Krowka MJ, Frantz RP, McGoon MD, Severson C, Plevak DJ, Wiesner RH. Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): A study of 15 patients with moderate to severe portopulmonary hypertension. Hepatology 1999. PubMed Abstracts

Kuo PC, Plotkin JS, Johnson LB, Howell CD, Laurin JM, Bartlett ST, Rubin LJ. Distinctive clinical features of portopulmonary hypertension. Chest 1997. PubMed Abstracts

Przybylowski T, Krenke R, Fangrat A, Nasilowski J, Grabczak EM, Styczynski G, Pruszczyk P, Krawczyk M, Chazan R.Gas exchange abnormalities in patients listed for liver transplantation. J Physiol Pharmacol. 2006 Sep;57 Suppl 4:313-PubMed Abstract

Moller S, Henriksen JH. Cardiopulmonary complications in chronic liver disease.World J Gastroenterol. 2006 Jan 28;12(4):526-38. Review. PubMed Abstract

Imamura M, Vitello AM, Limbird JN, Ivy DD, Fallon MB, Carter EP. Endothelin-B receptor overexpression prevents hypoxic pulmonary hypertension in cirrhotic rats. Chest. 2005 Dec;128(6 Suppl):580S-581S. PubMed Abstract

HIV and Pulmonary Hypertension

Aguilar RV, Farber HW. Epoprostenol (prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med 2000. PubMed Abstracts

Nunes H, Humbert M, Sitbon O, Morse JH, Deng Z, Knowles JA, Le Gall C, Parent F, Garcia G, Herve P, Barst RJ, Simonneau G. Prognostic factors for survival in human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2003. PubMed Abstracts

Opravil M, Pechere M, Speich R, Joller-Jemelka HI, Jenni R, Russi EW, Hirschel B, Luthy R. HIV-associated primary pulmonary hypertension. A case control study. Swiss HIV Cohort Study. Am J Respir Crit Care Med 1997. PubMed Abstracts

Sitbon O, Gressin V, Speich R, Macdonald PS, Opravil M, Cooper DA, Fourme T, Humbert M, Delfraissy JF, Simonneau G. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004. PubMed Abstracts

Sickle Cell Disease and Pulmonary Hypertension

Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood 2003. PubMed Abstracts

Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004. PubMed Abstracts

Morris CR, Morris SM, Jr., Hagar W, Van Warmerdam J, Claster S, Kepka-Lenhart D, Machado L, Kuypers FA, Vichinsky EP. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 2003. PubMed Abstracts

Machado RF, Martyr S, Kato GJ, Barst RJ, Anthi A, Robinson MR, Hunter L, Coles W, Nichols J, Hunter C, Sachdev V, Castro O, Gladwin MT. Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension. Br J Haematol 2005. PubMed Abstracts

Ataga KI, Sood N, De Gent G, Kelly E, Henderson AG, Jones S, Strayhorn D, Lail A, Lieff S, Orringer EP. Pulmonary hypertension in sickle cell disease. Am J Med 2004. PubMed Abstracts

COPD and Pulmonary Hypertension

Chaouat A, Bugnet AS, Kadaoui N, Schott R, Enache I, Ducolone A, Ehrhart M, Kessler R, Weitzenblum E. Severe pulmonary hypertension and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2005. PubMed Abstracts

Christensen CC, Ryg MS, Edvardsen A, Skjonsberg OH. Relationship between exercise desaturation and pulmonary haemodynamics in COPD patients. Eur Respir J 2004. PubMed Abstracts

Eddahibi S, Chaouat A, Morrell N, Fadel E, Fuhrman C, Bugnet AS, Dartevelle P, Housset B, Hamon M, Weitzenblum E, Adnot S. Polymorphism of the serotonin transporter gene and pulmonary hypertension in chronic obstructive pulmonary disease. Circulation 2003. PubMed Abstracts

Kessler R, Faller M, Weitzenblum E, Chaouat A, Aykut A, Ducolone A, Ehrhart M, Oswald-Mammosser M. "Natural history" of pulmonary hypertension in a series of 131 patients with chronic obstructive lung disease. Am J Respir Crit Care Med 2001. PubMed Abstracts

Scharf SM, Iqbal M, Keller C, Criner G, Lee S, Fessler HE. Hemodynamic characterization of patients with severe emphysema. Am J Respir Crit Care Med 2002. PubMed Abstracts

Thabut G, Dauriat G, Stern JB, Logeart D, Levy A, Marrash-Chahla R, Mal H. Pulmonary hemodynamics in advanced COPD candidates for lung volume reduction surgery or lung transplantation. Chest 2005. PubMed Abstracts

Vonbank K, Ziesche R, Higenbottam TW, Stiebellehner L, Petkov V, Schenk P, Germann P, Block LH. Controlled prospective randomised trial on the effects on pulmonary haemodynamics of the ambulatory long term use of nitric oxide and oxygen in patients with severe COPD. Thorax 2003. PubMed Abstracts

Wright JL, Levy RD, Churg A. Pulmonary hypertension in chronic obstructive pulmonary disease: current theories of pathogenesis and their implications for treatment. Thorax 2005. PubMed Abstracts

Naeje R. Pulmonary Hypertension and Right Heart Failure in Chronic Obstructive Pulmonary Disease Proc Am Thorac Vol 2.20-25 ,2005 ILD and Pulmonary Hypertension PubMed Abstracts

Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006. PubMed Abstracts

Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, Ryu JH. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest 2005. PubMed Abstracts

Gagermeier J, Dauber J, Yousem S, Gibson K, Kaminski N. Abnormal vascular phenotypes in patients with idiopathic pulmonary fibrosis and secondary pulmonary hypertension. Chest. 2005 Dec;128(6 Suppl):601S. PubMed Abstracts

Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, Ryu JH. The impact of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis. Chest. 2005 Dec;128(6 Suppl):616S-617S. PubMed Abstracts

 

Sleep Disordered Breathing and Pulmonary Hypertension

Atwood CW, Jr., McCrory D, Garcia JG, Abman SH, Ahearn GS. Pulmonary artery hypertension and sleep-disordered breathing: ACCP evidence-based clinical practice guidelines. Chest 2004. PubMed Abstracts

Kessler R, Chaouat A, Schinkewitch P, Faller M, Casel S, Krieger J, Weitzenblum E. The obesity-hypoventilation syndrome revisited: a prospective study of 34 consecutive cases. Chest 2001. PubMed Abstracts

Sarcoidosis and Pulmonary Hypertension

Shorr AF, Helman DL, Davies DB, Nathan SD. Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics. Eur Respir J 2005. PubMed Abstracts

Nunes H, Humbert M, Capron F, Brauner M, Sitbon O, Battesti JP, Simonneau G, Valeyre D. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 2006. PubMed Abstracts

Fisher KA, Serlin DM, Wilson KC, Walter RE, Berman JS, Farber W. Sarcoidosis-associated pulmonary hypertension: outcome with long-term epoprostenol treatment. Chest. 2006 Nov;130(5):1481-8. PubMed Abstracts

Handa T, Nagai S, Miki S, Fushimi Y, Ohta K, Mishima M, Izumi T. Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest. 2006 May;129(5):1246-52. PubMed Abstracts

Pregnancy and Pulmonary Hypertension

Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jais X, Humbert M, Audibert F, Frydman R, Simonneau G, Benhamou D. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology 2005. PubMed Abstracts