Saudi guidelines on diagnosis and treatment of pulmonary arterial hypertension

Annals of Thoracic Medicine

Volumn 3, Issue SUPPL. 5, 2008, Pages

  Idrees, Majdy ^a   Al Hajjaj, Mohammed ^a   Khan, Javeed ^a   Al Hazmi, Manal ^a   Alanezi, Mohammed ^a   Saleemi, Sarfraz ^a   Sanai, Faisal ^a   Kashour, Tarek ^a   Wali, Siraj ^a   Barst, Robyn ^a  

^a Riyadh Military Hospital ^*  (Saudi Arabia)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE; AMBRISENTAN; ANTICOAGULANT AGENT; ARGININE; BERAPROST; BONE MORPHOGENETIC PROTEIN 2; BOSENTAN; CALCIUM CHANNEL BLOCKING AGENT; COCAINE; DIGITALIS; DIGOXIN; DIURETIC AGENT; DOBUTAMINE; ENDOTHELIN 1; ENDOTHELIN RECEPTOR ANTAGONIST; GUANYLATE CYCLASE; ILOPROST; NITRIC OXIDE; OXYGEN; PHOSPHODIESTERASE INHIBITOR; PLACEBO; PROSTACYCLIN; SEROTONIN TRANSPORTER; SILDENAFIL; SITAXSENTAN; TADALAFIL; THROMBOXANE A2; UNIPROST; VARDENAFIL; VASODILATOR AGENT;

ABDOMINAL CRAMP; ALTITUDE; ARTERIAL GAS; ASCITES; BACKACHE; BLALOCK HANLON OPERATION; BLOOD EXAMINATION; BLOOD TRANSFUSION; CLINICAL ASSESSMENT; CLINICAL FEATURE; CLINICAL TRIAL; COMPUTER ASSISTED TOMOGRAPHY; CONNECTIVE TISSUE DISEASE; COUGHING; DEMOGRAPHY; DIAGNOSTIC PROCEDURE; DIARRHEA; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISEASE SEVERITY; DIZZINESS; DRUG EFFICACY; DRUG HALF LIFE; DRUG SAFETY; DYSPEPSIA; EISENMENGER COMPLEX; ELECTIVE SURGERY; ELECTROCARDIOGRAM; ENDARTERECTOMY; ENDOTHELIAL DYSFUNCTION; EPISTAXIS; EVIDENCE BASED MEDICINE; EXERCISE TOLERANCE; FLUSHING; FOOT PAIN; FUNCTIONAL STATUS; HEADACHE; HEART LUNG TRANSPLANTATION; HEART RIGHT VENTRICLE FAILURE; HEMANGIOMATOSIS; HEMODYNAMICS; HEMOGLOBIN BLOOD LEVEL; HEMOGLOBINOPATHY; HISTOPATHOLOGY; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; HYPOTENSION; INCIDENCE; INFECTION; JAW; LEG PAIN; LEG SWELLING; LIVER TOXICITY; LUNG ANGIOGRAPHY; LUNG BIOPSY; LUNG CAPACITY; LUNG FUNCTION TEST; LUNG TRANSPLANTATION; LUNG VENTILATION PERFUSION RATIO; MAGNETIC RESONANCE ANGIOGRAPHY; MUTATION; NAUSEA; NOSE CONGESTION; NUCLEAR MAGNETIC RESONANCE IMAGING; OXYGEN THERAPY; PAIN; PATHOPHYSIOLOGY; PERCUTANEOUS TRANSLUMINAL ANGIOPLASTY; PERIPHERAL EDEMA; PHLEBOTOMY; PHYSICAL ACTIVITY; PORTOPULMONARY HYPERTENSION; PRACTICE GUIDELINE; PRIMARY PREVENTION; PROGNOSIS; PSYCHOLOGICAL ASPECT; PULMONARY HYPERTENSION; REVIEW; RISK FACTOR; SAUDI ARABIA; SICKLE CELL ANEMIA; SIDE EFFECT; SINUSITIS; SLEEP APNEA SYNDROME; SURGICAL TECHNIQUE; SURVIVAL; SYNCOPE; THORAX RADIOGRAPHY; THROMBOEMBOLISM; TISSUE REPAIR; TRANSTHORACIC ECHOCARDIOGRAPHY; TRAVEL; TREATMENT INDICATION; TREATMENT RESPONSE; TRICUSPID VALVE REGURGITATION; VASOCONSTRICTION; VISUAL DISORDER;

EID: 41949105559     PISSN: 18171737     EISSN: 19983557     Source Type: Journal    
DOI: None     Document Type: Review

References (446)

1. Simonneau G, Galie N, Rubin L. Clinical classification of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:S5-12.
2. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension : Results from a national prospective registry. Ann Intern Med 1991;115:343-9.
3. Sandoval J, Bauerele O, Palomar A, Gómez A, Martínez-Guerra ML, Beltrán M, et al. Survival in primary pulmonary hypertension: Validation of a prognostic equation. Circulation 1994;89:1733-44.
4. Okada O, Tanabe N, Yasuda J, Yoshida Y, Katoh K, Yamamoto T, et al. Prediction of life expectancy in patients with primary pulmonary hypertension: A retrospective nationwide survey from 1980-1990. Intern Med 1999;38:12-6.
5. Rajasekhar D, Balakrishnan KG, Venkitachalam CG, Tharakan JA, Titus T, Subramanian R, et al. Primary pulmonary hypertension: Natural history and prognostic factors. Indian Heart J 1994;46:165-70.
6. rd, Loyd JE, et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension: The International PPH Consortium. Nat Genet 2000;26:81-4.
7. Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000;67:737-44.
8. Teichert-Kuliszewska K, Kutryk MJ, Kuliszewski MA, Karoubi G, Courtman DW, Zucco L, et al. Bone morphogenetic protein receptor 2 signaling promotes pulmonary arterial endothelial cell survival: Implications for loss-of-function mutations in the pathogenesis of pulmonary hypertension. Circulation Res 2006;98:209-17.
9. Fishman AP. Primary pulmonary arterial hypertension: A look back. J Am Coll Cardiol 2004;43:S2-4.
10. Pietra GG, Edwards WD, Kay JM, Rich S, Kernis J, Schloo B, et al. Histopathology of primary pulmonary hypertension: A qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute, Primary Pulmonary Hypertension Registry. Circulation 1989;80:1198-206.
11. Yang X, Long L, Southwood M, Rudarakanchana N, Upton PD, Jeffery TK, et al. Dysfunctional smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension. Circulation Res 2005;96:1053-63.
12. Eddahibi S, Humbert M, Fadel E, Raffestin B, Darmon M, Capron F, et al. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001;108:1141-50.
13. Long L, MacLean MR, Jeffery TK, Morecroft I, Yang X, Rudarakanchana N, et al. Serotonin Increases Susceptibility to Pulmonary Hypertension in BMPR2 Deficient Mice. Circulation Res 2006;98:818-27.
14. Chambers CD, Hernandez-Diaz S, Van Marter LJ, Werler MM, Louik C, Jones KL, et al. Selective serotonin reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. N Engl J Med 2006;354:579-87.
15. Bonnet S, Rochefort G, Sutendra G, Archer SL, Haromy A, Webster L, et al. The nuclear factor of activated T cells in pulmonary arterial hypertension can be therapeutically targeted. Proc Natl Acad Sci USA 2007;104:11418-23.
16. Friedman R, Mears JG, Barst RJ. Continuous infusion of prostacyclin normalizes plasma markers of endothelial cell injury and platelet aggregation in primary pulmonary hypertension. Circulation 1997;96:2782-4.
17. Pietra GG, Capron F, Stewart S, Leone O, Humbert M, Robbins IM, et al. Pathologic assessment of vasculopathies in pulmonary hypertension. J Am Coll Cardiol 2004;43:25S-32S.
18. Eisenberg PR, Lucore C, Kaufman L, Sobel BE, Jaffe AS, Rich S. Fibrinopeptide A levels indicative of pulmonary vascular thrombosis in patients with primary pulmonary hypertension. Circulation 1990;82:841-7.
19. Wood P. Primary pulmonary hypertension, with special reference to the vasoconstrictive factor. Br Heart J 1958;20:557-70.
20. Budhiraja R, Tuder RM, Hassoun PM. Endothelial dysfunction in pulmonary hypertension. Circulation 2004;109:159-65.
21. Verhaar MC, Strachan FE, Newby DE, Cruden NL, Koomans HA, Rabelink TJ, et al. Endothelins and endothelin receptor antagonists: Therapeutic considerations for a novel class of cardiovascular drugs. Circulation 1998;97:752-6.
22. Chen SJ, Chen YF, Opgenorth TJ, Wessale JL, Meng QC, Durand J, et al. The orally active nonpeptide endothelin A-receptor antagonist A-127722 prevents and reverses hypoxia-induced pulmonary hypertension and pulmonary vascular remodeling in Sprague-Dawley rats. J Cardiovasc Pharmacol 1997;29:713-25.
23. Humbert M, Morrell NW, Archer SL, Stenmark KR, MacLean MR, Lang IM, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:13S-24S.
24. Eddahibi S, Humbert M, Fadel E, Raffestin B, Darmon M, Capron F, et al. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001;108:1141-50.
25. Jeffery TK, Wanstall JC. Pulmonary vascular remodeling: A target for therapeutic intervention in pulmonary hypertension. Pharmacol Ther 2001;92:1-20.
26. Murakoshi N, Miyauchi T, Kakinuma Y, Ohuchi T, Goto K, Yanagisawa M, et al. Vascular endothelin-B receptor system in vivo plays a favorable inhibitory role in vascular remodeling after injury revealed by endothelin-B receptor-Knockout mice. Circulation 2002;106:1991-8.
27. Chen SJ, Chen YF, Opgenorth TJ, Wessale JL, Meng QC, Durand J, et al. The orally active nonpeptide endothelin A-receptor antagonist A-127722 prevents and reverses hypoxia-induced pulmonary hypertension and pulmonary vascular remodeling in Sprague-Dawley rats. J Cardiovasc Pharmacol 1997;29:713-25.
28. Brookes C, Ravn H, White P, Moeldrup U, Oldershaw P, Redington A. Acute right ventricular dilatation in response to ischemia significantly impairs left ventricular systolic performance. Circulation 1999;100:761-7.
29. Gan CT, Lankhaar JW, Marcus JT, Westerhof N, Marques KM, Bronzwaer JG, 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: H1528-33.
30. Damiano RJ Jr, La Follette P Jr, Cox JL, Lowe JE, Santamore WP. Significant left ventricular contribution to right ventricular systolic function. Am J Physiol 1991;261:H1514-24.
31. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Primary pulmonary hypertension: A national prospective study. Ann Intern Med 1987;107:216-23.
32. MacGregor AJ, Canavan R, Knight C, Denton CP, Davar J, Coghlan J, et al. Pulmonary hypertension in systemic sclerosis: Risk factors for progression and consequences for survival. Rheumatology (Oxford) 2001;40:453-9.
33. McGoon M, Gutterman D, Steen V, Barst R, McCrory DC, Fortin TA, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126;14S-34S.
34. Ahearn GS, Tapson VF, Rebeiz A, Greenfield JC Jr. Electrocardiography to define clinical status in primary pulmonary hypertension and pulmonary arterial hypertension secondary to collagen vascular disease. Chest 2002;122:524-7.
35. Bossone E, Paciocco G, Iarussi D, Agretto A, Iacono A, Gillespie BW, et al. The prognostic role of the ECG in primary pulmonary hypertension. Chest 2002;121:513-8.
36. rd, Hoeck BE, Chitwood WR Jr, Lyerly HK, Sabiston DC Jr, Chen JT. Radiographic findings in pulmonary hypertension from unresolved embolism. AJR Am J Roentgenol 1985;144:681-6.
37. Schmidt HC, Kauczor HU, Schild HH, Renner C, Kirchhoff E, Lang P, et al. Pulmonary hypertension in patients with chronic pulmonary thromboembolism: Chest radiograph and CT evaluation before and after surgery. Eur Radiol 1996;6: 817-25.
38. Murata I, Kihara H, Shinohara S, Ito K. Echocardiographic evaluation of pulmonary arterial hypertension in patients with progressive systemic sclerosis and related syndromes. Jpn Circ J 1992;56:983-91.
39. Hinderliter AL, Willis PW 4th, Barst RJ, Rich S, Rubin LJ, Badesch DB, et al. Effects of long-term infusion of prostacyclin (epoprostenol) on echocardiographic measures of right ventricular structure and function in primary pulmonary hypertension. Circulation 1997;95:1479-86.
40. Borgeson DD, Seward JB, Miller FA Jr, Oh JK, Tajik AJ. Frequency of doppler measurable pulmonary artery pressures. J Am Soc Echocardiogr 1996;9:832-7.
41. Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, et al. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 2003;167:735-40.
42. Bossone E, Duong-Wagner TH, Paciocco G, Oral H, Ricciardi M, Bach DS, et al. Echocardiographic features of primary pulmonary hypertension. J Am Soc Echocardiogr 1999;12:655-62.
43. Kim WR, Krowka MJ, Plevak DJ, Lee J, Rettke SR, Frantz RP, et al. Accuracy of Doppler echocardiography in the assessment of pulmonary hypertension in liver transplant candidates. Liver Transpl 2000;6:453-8.
44. Shapiro SM, Oudiz RJ, Cao T, Romano MA, Beckmann XJ, Georgiou D, et al. Primary pulmonary hypertension: Improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol 1997;30:343-9.
45. Shen JY, Chen SL, Wu YX, Tao RQ, Gu YY, Bao CD, et al. Pulmonary hypertension in systemic lupus erythematosus. Rheumatol Int 1999;18:147-51.
46. Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984;70:657-62.
47. Currie PJ, Seward JB, Chan KL, Fyfe DA, Hagler DJ, Mair DD, et al. Continuous wave Doppler determination of right ventricular pressure: A simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol 1985;6:750-6.
48. Chan KL, Currie PJ, Seward JB, Hagler DJ, Mair DD, Tajik AJ. Comparison of three Doppler ultrasound methods in the prediction of pulmonary artery pressure. J Am Coll Cardiol 1987;9:549-54.
49. Isobe M, Yazaki Y, Takaku F, Koizumi K, Hara K, Tsuneyoshi H, et al. Prediction of pulmonary arterial pressure in adults by pulsed Doppler echocardiography. Am J Cardiol 1986;57:316-21.
50. Nagaya N, Satoh T, Uematsu M, Okano Y, Kyotani S, Nakanishi N, et al. Shortening of Doppler-derived deceleration time of early diastolic transmitral flow in the presence of pulmonary hypertension through ventricular interaction. Am J Cardiol 1997;79:1502-6.
51. Kitabatake A, Inoue M, Asao M, Masuyama T, Tanouchi J, Morita T, et al. Noninvasive evaluation of pulmonary hypertension by a pulsed Doppler technique. Circulation 1983;68:302-9.
52. Tüller D, Steiner M, Wahl A, Kabok M, Seiler C. Systolic right ventricular function assessment by pulsed wave tissue Doppler imaging of the tricuspid annulus. Swiss Med Wkly 2005;135:461-8.
53. Borges AC, Knebel F, Eddicks S, Panda A, Schattke S, Witt C, et al. Right ventricular function assessed by two-dimensional strain and tissue doppler echocardiography in patients with pulmonary arterial hypertension and effect of vasodilator therapy. Am J Cardiol 2006;98:530-4.
54. Galie N, Hinderliter AL, Torbicki A, Fourme T, Simonneau G, Pulido T, 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;41:1380-6.
55. Viner SM, Bagg BR, Auger WR, Ford GT. The management of pulmonary hypertension secondary to chronic thromboembolic disease. Prog Cardiovasc Dis 1994;37:79-92.
56. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003;41:1028-35.
57. Morris TA, Auger WR, Ysrael MZ, Olson LK, Channick RN, Fedullo PF, et al. Parenchymal scarring is associated with restrictive spirometric defects in patients with chronic thromboembolic pulmonary hypertension. Chest 1996;110:399-403.
58. Romano AM, Tomaselli S, Gualtieri G, Zoia MC, Fanfulla F, Berrayah L, et al. Respiratory function in precapillary pulmonary hypertension. Monaldi Arch Chest Dis 1993;48:201-4.
59. Steenhuis LH, Groen HJ, Koeter GH, van der Mark TW. Diffusion capacity and haemodynamics in primary and chronic thromboembolic pulmonary hypertension. Eur Respir J 2000;16:276-81.
60. Rafanan AL, Golish JA, Dinner DS, Hague LK, Arroliga AC. Nocturnal hypoxemia is common in primary pulmonary hypertension. Chest 2001;120:894-9.
61. Pauwels RA, Buist AS, Calverley PM, Jenkins CR, Hurd SS; GOLD Scientific Committee. Global strategy for the diagnosis, management and prevention of chronic obstructive pulmonary disease. NHLBI/WHO Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop summary. Am J Respir Crit Care Med 2001;163:1256-76.
62. Kazerooni EA, Martinez FJ, Flint A, Jamadar DA, Gross BH, Spizarny DL, et al. Thin-section CT obtained at 10-mm increments versus limited three-level thin-section CT for idiopathic pulmonary fibrosis: Correlation with pathologic scoring. AJR Am J Roentgenol 1997;169:977-83.
63. Viner SM, Bagg BR, Auger WR, Ford GT. The management of pulmonary hypertension secondary to chronic thromboembolic disease. Prog Cardiovasc Dis 1994;37:79-92.
64. Bailey CL, Channick RN, Auger WR, Fedullo PF, Kerr KM, Yung GL, et al. High probability perfusion lung scans in pulmonary venoocclusive disease. Am J Respir Crit Care Med 2000;162:1974-8.
65. Worsley DF, Palevsky HI, Alavi A. Ventilation-perfusion lung scanning in the evaluation of pulmonary hypertension. J Nucl Med 1994;35:793-6.
66. Bergin CJ, Hauschildt J, Rios G, Belezzuoli EV, Huynh T, Channick RN. Accuracy of MR angiography compared with radionuclide scanning in identifying the cause of pulmonary arterial hypertension. AJR Am J Roentgenol 1997;168:1549-55.
67. Rich S, Pietra GG, Kieras K, Hart K, Brundage BH. Primary pulmonary hypertension: Radiographic and scintigraphic patterns of histologic subtypes. Ann Intern Med 1986;105:499-502.
68. Ryan KL, Fedullo PF, Davis GB, Vasquez TE, Moser KM. Perfusion scanfindings understate the severity of angiographic and hemodynamiccompromise in chronic thromboembolic pulmonary hypertension. Chest 1988;93:1180-5.
69. Resten A, Maitre S, Capron F, Simonneau G, Musset D. Pulmonary hypertension: CT findings in pulmonary veno-occlusive disease. J Radiol 2003;84:1739-45.
70. Ley S, Kreitner KF, Fink C, Heussel CP, Borst MM, Kauczor HU. Assessment of pulmonary hypertension by CT and MR imaging. Eur Radiol 2004;14:359-68.
71. Dartevelle P, Fadel E, Mussot S, Chapelier A, Hervé P, de Perrot M, et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2004;23:637-48.
72. Bergin CJ, Hauschildt JP, Brown MA, Channick RN, Fedullo PF. Identifying the cause of unilateral hypoperfusion in patients suspected to have chronic pulmonary thromboembolism: Diagnostic accuracy of helical CT and conventional angiography. Radiology 1999;213:743-9.
73. Bergin CJ, Sirlin C, Deutsch R, Fedullo P, Hauschildt J, Huynh T, et al. Predictors of patient response to pulmonary thromboendarterectomy. AJR Am J Roentgenol 2000;174:509-15.
74. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001;345:1465-72.
75. Boxt LM. MR imaging of pulmonary hypertension and right ventricular dysfunction. Magn Reson Imaging Clin North Am 1996;4:307-25.
76. Frank H, Globits S, Glogar D, Neuhold A, Kneussl M, Mlczoch J. Detection and quantification of pulmonary artery hypertension with MR imaging: Results in 23 patients. AJR Am J Roentgenol 1993;161:27-31.
77. Nicod P, Moser KM. Primary pulmonary hypertension: The risk and benefit of lung biopsy. Circulation 1989;80:1486-8.
78. Rich S, Kieras K, Hart K, Groves BM, Stobo JD, Brundage BH. Antinuclear antibodies in primary pulmonary hypertension. J Am Coll Cardiol 1986;8:1307-11.
79. Peacock A, Naeije R, Galie N, Reeves JT. End-points for clinical trials in pulmonary arterial hypertension. Eur Respir J 2004;23: 947-53.
80. Appelbaum L, Yigla M, Bendayan D, Reichart N, Fink G, Priel I, et al. Primary pulmonary hypertension in Israel: A national survey. Chest 2001;119:1801-6.
81. 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;167:580-6.
82. Sitbon O, Humbert M, Ioos V. Who benefits from long-term calcium-channel blocker therapy in primary pulmonary hypertension? Am J Respir Crit Care Med 2003;167.
83. Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Hervé P, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: Prognostic factors and survival. J Am Coll Cardiol 2002;40:780-8.
84. Higenbottam T, Butt AY, McMahon A, Westerbeck R, Sharples L. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart 1998;80:151-5.
85. Miyamoto S, Nagaya N, Satoh T, Kyotani S, Sakamaki F, Fujita M, et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension: Comparison with cardiopulmonary exercise testing. Am J Respir Crit Care Med 2000;161:487-92.
86. Wensel R, Opitz CF, Anker SD, Winkler J, Höffken G, Kleber FX, et al. Assessment of survival in patients with primary pulmonary hypertension: Importance of cardiopulmonary exercise testing. Circulation 2002;106:319-24.
87. Galie N, Manes A, Branzi A. The new clinical trials on pharmacological treatment in pulmonary arterial hypertension. Eur Respir J 2002;20:1037-49.
88. Guyatt GH, Sullivan MJ, Thompson PJ, Fallen EL, Pugsley SO, Taylor DW, et al. The 6-minute walk: A new measure of exercise capacity in patients with chronic failure. Can Med Assoc J 1985;132:919-23.
89. Paciocco G, Martinez FJ, Bossone E, Pielsticker E, Gillespie B, Rubenfire M. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur Respir J 2001;17:647-52.
90. Gitt AK, Wasserman K, Kilkowski C, Kleemann T, Kilkowski A, Bangert M, et al. Exercise anaerobic threshold and ventilatory efficiency identify heart failure patients for high risk of early death. Circulation 2002;106:3079-84.
91. Hinderliter AL, Willis PW 4th, Long W, Clarke WR, Ralph D, Caldwell EJ, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. Am J Cardiol 1999;84:481-4,A10.
92. Yeo TC, Dujardin KS, Tei C, Mahoney DW, McGoon MD, Seward JB. Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension. Am J Cardiol 1998;81:1157-61.
93. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002;39:1214-9.
94. Rich S, D'Alonzo GE, Dantzker DR, Levy PS. Magnitude and implications of spontaneous hemodynamic variability in primary pulmonary hypertension. Am J Cardiol 1985;55:159-63
95. Hoeper MH, Lee SH, Voswinckel R, Palazzini M, Jais X, Marinelli A, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006;48:2546-52.
96. Weir EK, Rubin LJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. The acute administration of vasodilators in primary pulmonary hypertension: Experience from the National Institutes of Health Registry on Primary Pulmonary Hypertension. Am Rev Respir Dis 1989;140:1623-30.
97. Barst R, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski H, et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:40S-7S.
98. Sitbon O, Humbert M, Ioos V. Who benefits from long-term calcium-channel blocker therapy in primary pulmonary hypertension? Am J Respir Crit Care Med 2003;167.
99. Galie N, Seeger W, Naeije R, Simonneau G, Rubin LJ. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43: 81S-8S.
100. Galie N, Ussia G, Passarelli P, Parlangeli R, Branzi A, Magnani B. Role of pharmacologic tests in the treatment of primary pulmonary hypertension. Am J Cardiol 1995;75:55A-62A.
101. Stibon O, Humber M, Jais X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary hypertension. Circulation 2005;111:3105-11.
102. Raffy O, Azarian R, Brenot F, Parent F, Sitbon O, Petitpretz P, et al. Clinical significance of the pulmonary vasodilator response during short-term infusion of prostacyclin in primary pulmonary hypertension. Circulation 1996;93:484-8.
103. Nagaya N, Nishikimi T, Uematsu M, Satoh T, Kyotani S, Sakamaki F, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000;102:865-70.
104. Rubens C, Ewert R, Halank M, Wensel R, Orzechowski HD, Schultheiss HP, et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001;120:1562-9.
105. Torbicki A, Kurzyna M, Kuca P, Fijałkowska A, Sikora J, Florczyk M, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 2003;108:844-8.
106. Fox AC, Reed GE, Meilman H, Silk BB. Release of nucleosides from canine and human hearts as an index of prior schemia. Am J Cardiol 1979;43:52-8.
107. Mentzer RM, Rubio R, Berne RM. Release of adenosine by hypoxic canine lung tissue and its possible role in pulmonary circulation. Am J Physiol 1975;229:1625-31.
108. Nagaya N, Uematsu M, Satoh T, Kyotani S, Sakamaki F, Nakanishi N, et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999;160:487-92.
109. Committee for Practice Guidelines (CPG) of the European Society of Cardiology. Recommendations for Task Force Creation and Report Production A document for Task Force members and expert panels responsible for the creation and production of Guidelines and Expert Consensus Documents Committee for Practice Guidelines (CPG) of the European Society of Cardiology; 2003.
110. McCrory DC, Lewis SZ. Methodology and grading for pulmonary hypertension evidence review and guideline development. Chest 2004;126:11S-3S.
111. Manes A, Galiè N, Branzi A. Women and pulmonary arterial hypertension. Eur Respir J 2003;8:219-27.
112. Nelson DM, Main E, Crafford W, Ahumada GG. Peripartum heart failure due to primary pulmonary hypertension. Obstet Gynecol 1983;62:58s-63s.
113. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: A systematic overview from 1978 through 1996. J Am Coll Cardiol 1998;31:1650-7.
114. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003;24:761-81.
115. Nootens M, Rich S. Successful management of labor and delivery in primary pulmonary hypertension. Am J Cardiol 1993;71:1124-5.
116. Abenhaim L, Moride Y, Brenot F, Rich S, Benichou J, Kurz X, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. N Engl J Med 1996;335:609-16.
117. Deanfield J, Thaulow E, Warnes C, Webb G, Kolbel F, Hoffman A, et al. Management of grown up congenital heart disease. Eur Heart J 2003;24:1035-84.
118. Girgis A, Sanson-Fisher RW. Breaking bad news: Consensus guidelines for medical practitioners. J Clin Oncol 1995;13: 2449-56.
119. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: Natural history and the importance of thrombosis. Circulation 1984;70:580-7.
120. Kanemoto N. Natural history of pulmonary hemodynamics in primary pulmonary hypertension. Am Heart J 1987;114:407-13
121. Sandoval J, Aguirre JS, Pulido T, Martinez-Guerra ML, Santos E, Alvarado P, et al. Nocturnal oxygen therapy in patients with the eisenmenger syndrome. Am J Respir Crit Care Med 2001;164: 1682-7.
122. Frank H, Mlczoch J, Huber K, Schuster E, Gurtner HP, Kneussl M. The effect of anticoagulant therapy in primary and anorectic drug-induced pulmonary hypertension. Chest 1997;112:714-21.
123. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, et al. Eisenmenger syndrome: Factors relating to deterioration and death. Eur Heart J 1998;19:1845-55.
124. Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: A randomized, controlled trial. Ann Intern Med 2000;132:425-34.
125. Rich S, Seidlitz M, Dodin E, Osimani D, Judd D, Genthner D, et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998;114:787-92.
126. Coates AL, Desmond K, Asher MI, Hortop J, Beaudry PH. The effect of digoxin on exercise capacity and exercising cardiac function in cystic fibrosis. Chest 1982;82:543-7.
127. 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;327:76-81.
128. Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: Evidence for long-term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation 1987;76:135-41.
129. Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;111:3105-11.
130. Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999;99:1197-208.
131. Yung D, Widlitz AC, Rosenzweig EB, et al. Outcomes in children with idiopathic pulmonary arterial hypertension. Circulation 2004;110:660-5.
132. Rich S, Kaufmann E. High dose titration of calcium channel blocking agents for primary pulmonary hypertension: Guidelines for short-term drug testing. J Am Coll Cardiol 1991;18:1323-7.
133. Jones DA, Benjamin CW, Linseman DA. Activation of thromboxane and prostacyclin receptors elicits opposing effects on vascular smooth muscle cell growth and mitogen-activated protein kinase signaling cascades. Mol Pharmacol 1995;48:890-6.
134. Galie N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med 2003;2:123-37.
135. Langleben D, Barst RJ, Badesch D, Groves BM, Tapson VF, Murali S, et al. Continuous infusion of epoprostenol improves the net balance between pulmonary endothelin 1 clearance and release in primary pulmonary hypertension. Circulation 1999;99:3266-71.
136. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. N Engl J Med 1996;334:296-302.
137. McLaughlin VV, Genthner DE, Panella MM, Rich S. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998;338:273-7.
138. Clapp LH, Finney P, Turcato S, Tran S, Rubin LJ, Tinker A. Differential effects of stable prostacyclin analogues on smooth muscle proliferation and cyclic AMP generation in human pulmonary artery. Am J Respir Cell Mol Biol 2002;26:194-201.
139. Rubin LJ, Mendoza J, Hood M, McGoon M, Barst R, Williams WB, et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol): Results of a randomized trial. Ann Intern Med 1990;112:485-91.
140. Robbins IM, Gaine SP, Schilz R, Tapson VF, Rubin LJ, Loyd JE. Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus. Chest 2000;117:14-8.
141. McLaughlin VV, Genthner DE, Panella MM, Rich S. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: A case series. Ann Intern Med 1999;130:740-3.
142. Kuo PC, Johnson LB, Plotkin JS, Howell CD, Bartlett ST, Rubin LJ. Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension. Transplantation 1997;63:604-6.
143. Aguilar RV, Farber HW. Epoprostenol (prostacyclin) therapy in HIV-associated pulmonary hypertension. Am J Respir Crit Care Med 2000;162:1846-50.
144. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99:1858-65.
145. Bresser P, Fedullo PF, Auger WR, Channick RN, Robbins IM, Kerr KM, et al. Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension. Eur Respir J 2004;23:595-600.
146. Geohas C, McLaughlin VV. Successful management of pregnancy in a patient with eisenmenger syndrome with epoprostenol. Chest 2003;124:1170-3.
147. Hoeper MM, Olschewski H, Ghofrani HA, Wilkens H, Winkler J, Borst MM, et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. J Am Coll Cardiol 2000;35:176-82.
148. Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, et al. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002;347:322-9.
149. Hoeper MM, Schwarze M, Ehlerding S, Adler-Schuermeyer A, Spiekerkoetter E, Niedermeyer J, et al. Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. N Engl J Med 2000;342:1866-70.
150. Opitz CF, Wensel R, Winkler J, Halank M, Bruch L, Kleber FX, et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J 2005;26:1895-902.
151. Elliot CA, Stewart P, Webster VJ, Mills GH, Hutchinson SP, Howarth ES, et al. The use of Iloprost in early pregnancy in patients with pulmonary arterial hypertension. Eur Respir 2005;26:168-73.
152. Barst RJ, Rich S, Widlitz A, Horn EM, McLaughlin V, McFarlin J. Clinical efficacy of sitaxsentan, an endothelin: A receptor antagonist, in patients with pulmonary arterial hypertension: Open-label pilot study. Chest 2002;121:1860-8.
153. Lang I, Gomez-Sanchez M, Kneussl M, Naeije R, Escribano P, Skoro-Sajer N, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest 2006;129:1636-43.
154. Tapson VF, Gomberg-Maitland M, McLaughlin VV, Benza RL, Widlitz AC, Krichman A, et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: A prospective, multicenter, open-label, 12-week trial. Chest 2006;129:683-8.
155. Galie N, Humbert M, Vachiery JL, Vizza CD, Kneussl M, Manes A, et al. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: A randomised, double-blind placebo-controlled trial. J Am Coll Cardiol 2002;39:1496-502.
156. Barst RJ, McGoon M, McLaughlin VV, Tapson V, Rich S, Rubin L, et al. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003;41:2115-25.
157. Stewart DJ, Levy RD, Cernacek P, Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: Marker or mediator of disease? Ann Intern Med 1991;114:464-9.
158. Giaid A, Yanagisawa M, Langleben D, Michel RP, Levy R, Shennib H, et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993;328:1732-9.
159. Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 2004;61:227-37.
160. Benigni A, Remuzzi G. Endothelin antagonists. Lancet 1999;353:133-8.
161. Clozel M, Breu V, Gray GA, Löffler BM. In vivo pharmacology of Ro 46-2005, the first synthetic nonpeptide endothelin receptor antagonist: Implications for endothelin physiology. J Cardiovasc Pharmacol 1993;22:S377-9.
162. Channick R, Badesch DB, Tapson VF, Simonneau G, Robbins I, Frost A, et al. Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary hypertension: A placebo-controlled study. J Heart Lung Transplant 2001;20:262-3.
163. Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002;346:896-903.
164. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: A randomised placebo-controlled study. Lancet 2001;358:1119-23.
165. McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galiè N, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005;25:244-9.
166. Sitbon O, McLaughlin VV, Badesch DB, Barst RJ, Black C, Galiè N, et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax 2005;60:1025-30.
167. McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galiè N, et al. Survival with first line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005;25:244-9.
168. Gatzoulis, MA, Rogers P, Li W, Harries C, Cramer D, Ward S, et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol 2005;98:147-51.
169. Galie N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006;114:48-54.
170. Barst RJ, Ivy D, Dingemanse J, Widlitz A, Schmitt K, Doran A, et al. Pharmacokinetics, safety and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 2003;73:372-82.
171. Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:697-704.
172. Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:697-704.
173. Fattinger K, Funk C, Pantze M, Weber C, Reichen J, Stieger B, et al. The endothelin antagonist bosentan inhibits the canalicular bile salt export pump: A potential mechanism for hepatic adverse reactions. Clin Pharmacol Ther 2001;69:223-31.
174. Barst RJ, Langleben D, Frost A, Horn EM, Oudiz R, Shapiro S, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169:441-7.
175. Barst RJ, Rich S, Widlitz A, et al. Clinical efficacy of sitaxsentan, an endothelin-A receptor antagonist 149.
176. Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, et al. Treatment of pulmonary arterial hypertension with the selective endothelin: A receptor antagonist sitaxsentan. J Am Coll Cardiol 2006;47:2049-56.
177. Benza RL, Mehta S, Keogh A, Lawrence EC, Oudiz RJ, Barst RJ. Sitaxsentan treatment for patients with pulmonary arterial hypertension discontinuing bosentan. J Heart Lung Transplant 2007;26:63-9.
178. Galie N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:529-35.
179. Oudiz R, Torres F, Frost A. ARIES-1: A placebo-controlled efficacy and safety study of ambrisentan in patients with pulmonary arterial hypertension. Chest 2006;130:121S
180. Olschewski H, Galie N, Ghofrani HA. Ambrisentan improves exercise capacity and time to clinical worsening in patients with pulmonary arterial hypertension: Results of the ARIES- Cohort Study. Proc Am Thorac Soc 2006;3:A728.
181. Dufton C, Gerber MJ, Yin O. No clinical relevant pharmacokinetic interaction between ambrisentan an sildenafil. Chest 2006; 130:254S.
182. Gerber MJ, Dufton C, Pentikis H. Ambrisentan has no clinically relevant effect on the pharmacokinetics or pharmacodynamics of warfarin. Chest 2006;130:256S.
183. Beavo JA, Reifsnyder DH. Primary sequence of cyclic nucleotide phosphodiesterase isozymes and the design of selective inhibitors. Trends Pharmacol Sci 1990;11:150-5.
184. Ahn HS, Foster M, Cable M, Pitts BJ, Sybertz EJ. Ca/CaM-stimulated and cGMP-specific phosphodiesterases in vascular and nonvascular tissues. Adv Exp Med Biol 1991;308:191-7.
185. Hanson KA, Burns F, Rybalkin SD, Miller JW, Beavo J, Clarke WR. Developmental changes in lung cGMP phosphodiesterase-5 activity, protein and message. Am J Respir Crit Care Med 1998;158:279-88.
186. Hanson KA, Ziegler JW, Rybalkin SD, Miller JW, Abman SH, Clarke WR. Chronic pulmonary hypertension increases fetal lung cGMP phosphodiesterase activity. Am J Physiol 1998;275: L931-41.
187. Braner DA, Fineman JR, Chang R, Soifer SJ. M and B 22948, a cGMP phosphodiesterase inhibitor, is a pulmonary vasodilator in lambs. Am J Physiol 1993;264:H252-8.
188. McMahon TJ, Ignarro LJ, Kadowitz PJ. Influence of Zaprinast on vascular tone and vasodilator responses in the cat pulmonary vascular bed. J Appl Physiol 1993;74:1704-11.
189. Clarke WR, Uezono S, Chambers A, Doepfner P. The type III phosphodiesterase inhibitor milrinone and type V PDE inhibitor dipyridamole individually and synergistically reduce elevated pulmonary vascular resistance. Pulm Pharmacol 1994;7:81-9.
190. Cohen AH, Hanson K, Morris K, Fouty B, McMurty IF, Clarke W, et al. Inhibition of cyclic 3′-5′-guanosine monophosphate-specific phosphodiesterase selectively vasodilates the pulmonary circulation in chronically hypoxic rats. J Clin Invest 1996;97:172-9.
191. Tantini B, Manes A, Fiumana E, Pignatti C, Guarnieri C, Zannoli R, et al. Antiproliferative effect of Sidenafil on human pulmonary artery smooth muscle cells. Basic Res Cardiol 2005;100:131-8.
192. rd, Russell JA, Steinhorn RH. The cGMP phosphodiesterase inhibitor zaprinast enhances the effect of nitric oxide. Am J Respir Crit Care Med 1995;152:1605-10.
193. Ziegler JW, Ivy DD, Fox JJ, Kinsella JP, Clarke WR, Abman SH. Dipyridamole potentiates pulmonary vasodilation induced by acetylcholine and nitric oxide in the ovine fetus. Am J Respir Crit Care Med 1998;157:1104-10.
194. Ghofrani HA, Osterloh IH, Grimminger F. Sildenafil: From angina to erectile dysfunction to pulmonary hypertension and beyond. Nat Rev Drug Discov 2006;5:689-702.
195. Prasad S, Wilkinson J, Gatzoulis MA. Sildenafil in primary pulmonary hypertension. N Engl J Med 2000;343:1342.
196. Bhatia S, Frantz RP, Severson CJ, Durst LA, McGoon MD. Immediate and long-term hemodynamic and clinical effects of sildenafil in patients with pulmonary arterial hypertension receiving vasodilator therapy. Mayo Clin Proc 2003;78:1207-13.
197. Michelakis ED, Tymchak W, Noga M, Webster L, Wu XC, Lien D, et al. Long-term treatment with oral sildenafil is safe and improves functional capacity and hemodynamics in patients with pulmonary arterial hypertension. Circulation 2003;108:2066-9.
198. Ghofrani HA, Schermuly RT, Rose F, Wiedemann R, Kohstall MG, Kreckel A, et al. Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 2003;167:1139-41.
199. Ghofrani HA, Wiedemann R, Rose F, Schermuly RT, Olschewski H, Weissmann N, et al. Sildenafil for treatment of lung fibrosis and pulmonary hypertension: A randomised controlled trial. Lancet 2002;360:895-900.
200. Sastry BK, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension: A randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol 2004;43:1149-53.
201. Galie N, Ghofrani A, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148-57.
202. Oudiz RJ, Roveran G, Hansen JE, Sun XG, Wasserman K. Effect of sildenafil on ventilatory efficiency and exercise tolerance in pulmonary hypertension. Eur J Heart Fail 2007;9:917-21.
203. Michelakis E, Tymchak W, Lien D, Webster L, Hashimoto K, Archer S. Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension comparison with inhaled nitric oxide. Circulation 2002;105: 2398-403.
204. Wilkins MR, Paul GA, Strange JW, Tunariu N, Gin-Sing W, Banya WA, et al. Sildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) Study. Am J Respir Crit Care Med 2005;171:1292-7.
205. Richalet JP, Gratadour P, Robach P, Pham I, Déchaux M, Joncquier-Latarjet A, et al. Sildenafil inhibits altitude-induced hypoxemia and pulmonary hypertension. Am J Respir Crit Care Med 2005;171:275-81.
206. Fraunfelder FW, Pomeranz HD, Egan RA. Nonarteritic anterior ischemic optic neuropathy and sildenafil. Arch Ophthalmol 2006;124:733-4.
207. Vosatka RJ, Kashyap S, Trifiletti RR. Arginine deficiency accompanies persistent pulmonary hypertension of the newborn. Biol Neonate 1994;66:65-70.
208. McCaffrey MJ, Bose CL, Reiter PD, Stiles AD. Effect of L-arginine infusion on infants with persistent pulmonary hypertension of the newborn. Biol Neonate 1995;67:240-3.
209. Mitani Y, Maruyama K, Sakurai M. Prolonged administration of L-arginine ameliorates chronic pulmonary hypertension and pulmonary vascular remodeling in rats. Circulation 1997;96: 689-97.
210. Mehta S, Stewart DJ, Langleben D, Levy RD. Short-term pulmonary vasodilation with L-arginine in pulmonary hypertension. Circulation 1995;92:1539-45.
211. Nagaya N, Uematsu M, Oya H, Sato N, Sakamaki F, Kyotani S, et al. Short-term oral administration of L-arginine improves hemodynamics and exercise capacity in patients with precapillary pulmonary hypertension. Am J Respir Crit Care Med 2001;163: 887-91.
212. Surdacki A, Zmudka K, Bieron K, Kostka-Trabka E, Dubiel JS, Gryglewski RJ. Lack of beneficial effects of L-arginine infusion in primary pulmonary hypertension. Wien Klin Wochenschr 1994;106:521-6.
213. Baudouin SV, Bath P, Martin JF, Du Bois R, Evans TW. L-arginine infusion has no effect on systemic hemodynamics in normal volunteers or systemic and pulmonary hemodynamics in patients with elevated pulmonary vascular resistance. Br J Clin Pharmacol 1993;36:45-9.
214. Humbert M, Barst RJ, Robbins IM, Channick RN, Galiè N, Boonstra A, et al. Combination of Epoprosterol and Bosentan in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004;24:353-9.
215. Hoeper MM, Dinh-Xuan AT. Combination therapy for pulmonary arterial hypertension: Still more questions than answers. Eur Respir J 2004;24:339-40.
216. Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg 1997;3:494-500.
217. Seyfarth HJ, Pankau H, Hammerschmidt S, Schauer J, Wirtz H, Winkler J. Bosentan improves exercise tolerance and Tei index in patients with pulmonary hypertension and prostanoid therapy. Chest 2005;128:709-13.
218. 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 2006;48:1433-7.
219. 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;101:927-34.
220. Ghofrani HA, Rose F, Schermuly RT, Olschewski H, Wiedemann R, Kreckel A, et al. Oral sildenafil as long-term adjunct therapy to inhaled iloprost in severe pulmonary arterial hypertension. J Am Coll Cardiol 2003;42:158-64.
221. Wilkens H, Guth A, Konig J, Forestier N, Cremers B, Hennen B, et al. Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension. Circulation 2001;104:1218-22.
222. Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002;136:515-22.
223. Battista S, Bar F, Mengozzi G, Zanon E, Grosso M, Molino G. Hyperdynamic circulation in patients with cirrhosis: Direct measurement of nitric oxide levels in hepatic and portal veins. J Hepatol 1997;26:75-80.
224. Mathai SC, Girgis RE, Fisher MR, Champion HC, Hous ten-Harris T, Zaiman A, et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 2007;29:469-75.
225. Paul GA, Gibbs JS, Boobis AR, Abbas A, Wilkins MR. Bosentan decreases the plasma concentration of sildenafil when co-prescribed in pulmonary hypertension. Br J Clin Pharmacol 2005;60:107-12.
226. Morales J, Santos S, de Jover L, Sala E, Paré C, Roca J, et al. Clinical value of vasodilator test with inhaled nitric oxide for predicting longterm response top oral vasodilators in pulmonary hypertension. Respir Med 2004;98:225-34.
227. Langleben D, Christman BW, Barst RJ, Dias VC, Galiè N, Higenbottam TW, et al. Effects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertension. Am Heart J 2002;143:E4.
228. Findlay JF, Harrison BA, Plevak DJ, Krowka MJ. Inhaled nitric oxide reduces pulmonary artery pressures in portopulmonary hypertension. Liver Transpl 1999;5:381-7.
229. Austen WG, Morrow AG, Berry WB. Experimental studies of the surgical treatment of primary pulmonary hypertension. J Thorac Cardiovasc Surg 1964;48:448-55.
230. Rozkovec A, Montanes P, Oakley CM. Factors that influence the outcome of primary pulmonary hypertension. Br Heart J 1986;55:449-58.
231. Sandoval J, Rothman A, Pulido T. Atrial septostomy for pulmonary hypertension. Clin Chest Med 2001;22:547-60.
232. Klepetko W, Mayer E, Sandoval J, Trulock EP, Vachiery JL, Dartevelle P, et al. Interventional and surgical modalities of treatment for pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:73S-80S.
233. 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;24:166-72.
234. Kothari SS, Yusuf A, Juneja R, Yadav R, Naik N. Graded balloon atrial septostomy in severe pulmonary hypertension. Indian Heart J 2002;54:164-9.
235. Kerstein D, Levy P, Hsu DT, Hordof AJ, Gersony WM, Barst RJ. Blade balloon atrial septostomy in patients with severe primary pulmonary hypertension. Circulation 1995;91:2028-35.
236. Rothman A, Sklansky MS, Lucas VW, Kashani IA, Shaughnessy RD, Channick RN, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999;84:682-6.
237. Law MA, Grifka RG, Mullins CE, Nihill MR. Atrial septostomy improves survival in select patients with pulmonary hypertension. Am Heart J 2007;153:779-84.
238. Moscucci M, Dairywala IT, Chetcuti S, Mathew B, Li P, Rubenfire M, et al. Balloon atrial septostomy in end-stage pulmonary hypertension guided a novel intracarduiac echocardiographic transducer. Catheter Cardiovasc Interv 2001;52:530-4.
239. Hertz MI, Taylor DO, Trulock EP, Boucek MM, Mohacsi PJ, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: Nineteenth Official Report 2002. J Heart Lung Transplant 2002;21:950-70.
240. Chapelier A, Vouhe P, Macchiarini P, Lenot B, Cerrina J, Le Roy Ladurie F, et al. Comparative outcome of heart-lung and lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1993;106:229-307.
241. Chapelier A, Vouhe P, Macchiarini P, Lenot B, Cerrina J, Le Roy Ladurie F, et al. Comparative outcome of heart-lung and lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg 1993;106:229-307.
242. Hertz MI, Mohacsi PJ, Taylor DO, Trulock EP, Boucek MM, Deng MC, et al. The Registry of the International Society of Heart and Lung Transplantation: Twentieth official report. J Heart Lung Transplant 2003;22:610-5.
243. Whyte RI, Robbins RC, Altinger J, Barlow CW, Doyle R, Theodore J, et al. Heart-lung transplantation for primary pulmonary hypertension. Ann Thorac Surg 1999;67:937-41.
244. Sharples LD, Tamm M, McNeil K, Higenbottam TW, Stewart S, Wallwork J. Development of bronchiolitis obliterans syndrome in recipients of heart-lung transplantation: Early risk factors. Transplantation 1996;61:560-6.
245. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998;128:745-55.
246. Kidd L, Driscoll DJ, Gersony WM, Hayes CJ, Keane JF, O'Fallon WM, et al. Second natural history study of congenital heart defects: Results of treatment of patients with ventricular septal defects. Circulation 1993;87:I38-51.
247. Besterman E. Atrial septal defects with pulmonary hypertension. Br Heart J 1961;23:587-98.
248. Hoffman JE, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol 1985;16:634-53.
249. Vogel M, Berger F, Kramer A, Alexi-Meshkishvili V, Lange PE. Incidence of secondary pulmonary hypertension in adults with atrial septal or sinus venosus defects. Heart 1999;82:30-3.
250. Neumayer U, Stone S, Somerville J. Small ventricular septal defects in adults. Eur Heart J 1988;19:1573-82.
251. Roberts KE, McElroy JJ, Wong WP, Yen E, Widlitz A, Barst RJ, et al. BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur Respir J 2004;24:371-4.
252. Dimopoulos K, Okonko DO, Diller GP, Broberg CS, Salukhe TV, Babu-Narayan SV, et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation 2006;113:2796-802.
253. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol 1996;28:768-72.
254. Perloff JK, Hart EM, Greaves SM, Miner PD, Child JS. Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 2003;92:182-7.
255. Silversides CK, Granton JT, Konen E, Hart MA, Webb GD, Therrien J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003;42:1982-7.
256. Broberg C, Ujita M, Bouzas B. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with ventricular dysfunction, neurohormonal activation and poorer exercise capacity. Circulation 2004;110:497.
257. Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, et al. Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol 2007;50:634-42.
258. Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant 1996;15:100-5.
259. Diller GP, Dimopoulos K, Broberg GS, Kaya MG, Naghotra US, Uebing A, et al. Presentation, survival prospects and predictors of death in Eisenmenger syndrome: A combined retrospective and case-control study. Eur Heart J 2006;27:1737-42.
260. 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 2004;25:1651-6.
261. Berman EB, Barst RJ. Eisenmenger's syndrome: Current management. Prog Cardiovasc Dis 2002;45:129-38.
262. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults: Second of two parts. N Engl J Med 2000;342:334-42.
263. Bakst AE, Gaine SP, Rubin LJ. Continuous intravenous epoprostenol therapy for pulmonary hypertension in Gaucher.s disease. Chest 1999;116:1127-9.
264. Vachiery JL, Hill N, Zwicke D, Barst R, Blackburn S, Naeije R. Transitioning from IV epoprostenol to subcutaneous treprostinil in pulmonary arterial hypertension. Chest 2002;121:1561-5.
265. Hadengue A, Benhayoun MK, Lebrec D, Benhamou JP. Pulmonary hypertension complicating portal hypertension: Prevalence and relation to splanchnic hemodynamics. Gastroenterology 1991;100:520-8.
266. Herve P, Lebrec D, Brenot F, Simonneau G, Humbert M, Sitbon O, et al. Pulmonary vascular disorders in portal hypertension. Eur Respir J 1998;11:1153-66.
267. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 2004;363: 1461-8.
268. McDonnell PJ, Toye PA, Hutchins GM. Primary pulmonary hypertension and cirrhosis: are they related. Am Rev Respir Dis 1983;127:437-41.
269. Mandell MS, Groves BM. Pulmonary hypertension in chronic liver diseases. Clin Chest Med 1996;17:17-33.
270. Taura P, Garcia-Valdecasas JC, Beltran J, Izquierdo E, Navasa M, Sala-Blanch J, et al. Moderate primary pulmonary hypertension in patients undergoing liver transplantation. Anesth Analg 1996;83:675-80.
271. Budhiraja R, Hassoun PM. Portopulmonary hypertension: A tale of two circulations. Chest 2003;123:562-76.
272. Castro M, Krowka MJ, Schroeder DR, Beck KC, Plevak DJ, Rettke SR, et al. Frequency and clinical implications of increased pulmonary artery pressures in liver transplantation. Mayo Clin Proc 1996;71:543-51.
273. Ramsay MA, Simpson BR, Nguyen AT, Ramsay KJ, East C, Klintmalm GB. Severe pulmonary hypertension in liver transplant candidates. Liver Transpl Surg 1997;3:494-500.
274. Plevak D, Krowka MJ, Rettke S, Dunn W, Southorn P. Successful liver transplantation in patients with mild to moderate pulmonary hypertension. Transplant Proc 1993;25:1840.
275. El-Hazmi MA, Warsy AS, Bahakim H. Blood proteins C and S in sickle cell disease. Acta Haematologica 1993;90:114-9.
276. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95.
277. Ataga K, Jones S, Olajide O, Strayhorn D, Lail A, Orringer EP. The relationship of pulmonary hypertension and survival in sickle cell disease. Blood 2004b;104:463a.
278. Haque AK, Gokhale S, Rampy BA, Adegboyega P, Duarte A, Saldana MJ. Pulmonary hypertension in sickle cell hemoglobinopathy: A clinicopathologic study of 20 cases. Hum Pathol 2002;33:1037-43.
279. Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF. Pulmonary hypertension in sickle cell disease. Am J Cardiol 1994;74:626-8.
280. Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med 2004;350:857-9.
281. Castro LM, Jonassiant JC, Graham FL, Ashley-Koch A, Tellen MJ. Pulmonary hypertension in SS, SC and Sβ thalassemia: Prevalence, associated clinical syndromes and mortality. Blood 2004;104:462a.
282. Machado RF, Gladwin MT. Chronic sickle cell lung disease: New insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension. Br J Haematol 2005;129:449-64.
283. Aslan M, Ryan TM, Adler B, Townes TM, Parks DA, Thompson JA, et al. Oxygen radical inhibition of nitric oxide-dependent vascular function in sickle cell disease. Proc Nat Acad Sci U S A 2001;98:15215-20.
284. Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Cannon RO 3rd, Schechter AN, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle cell disease. Nat Med 2002;8:1383-9.
285. Morris CR, Morris SM Jr, Hagar W, Van Warmerdam J, Claster S, Kepka-Lenhart D, et al. Arginine therapy: A new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 2003;168:63-9.
286. Graido-Gonzalez E, Doherty JC, Bergreen EW, Organ G, Telfer M, McMillen MA. Plasma endothelin-1, cytokine and prostaglandin E2 levels in sickle cell disease and acute vaso-occlusive sickle crisis. Blood 1998;92:2551-5.
287. Yung GL, Channick RN, Fedullo PF, Auger WR, Kerr KM, Jamieson SW, et al. Successful pulmonary thromboendarterectomy in two patients with sickle cell disease. Am J Respir Crit Care Med 1998;157:1690-3.
288. Manci EA, Culberson DE, Yang YM, Gardner TM, Powell R, Haynes J Jr, et al. Investigators of the cooperative study of sickle cell disease: Causes of death in sickle cell disease: An autopsy study. Br J Haematol 2003;123:359-65.
289. Oppenheimer EH, Esterly JR. Pulmonary changes in sickle cell disease. Am Rev Respir Dis 1971;103:858-9.
290. Adedeji MO, Cespedes J, Allen K, Subramony C, Hughson MD. Pulmonary thrombotic arteriopathy in patients with sickle cell disease. Arch Pathol Lab Med 2001;125:1436-41.
291. Marfaing-Koka A, Boyer-Neumann C, Wolf M, Leroy-Matheron C, Cynober T, Tchernia G. Decreased protein S activity in sickle cell disease. Nouvelle Revue Francaise de Hematologie 1993;35:425-30.
292. Kato GJ, Martyr S, Machado RF, Sachdev V, Ernst I, Hunter LA, et al. Acute on chronic pulmonary hypertension in patients with sickle cell disease. Blood 2004;104:464a.
293. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: Risks and benefits up to 9 years of treatment. J Am Med Assoc 2003;289:1645-51.
294. Charache S, Terrin ML, Moore RD, Dover GJ, Barton FB, Eckert SV, et al. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia: Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995;332:1317-22.
295. Pegelow CH, Adams RJ, McKie V, Abboud M, Berman B, Miller ST, et al. Risk of recurrent stroke in patients with sickle cell disease treated with erythrocyte transfusions. J Pediatr 1995;126:896-9.
296. Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med 1998;339:5-11.
297. Hargrave DR, Wade A, Evans JP, Hewes DK, Kirkham FJ. Nocturnal oxygen saturation and painful sickle cell crises in children. Blood 2003;101:846-8.
298. Setty BN, Stuart MJ, Dampier C, Brodecki D, Allen JL. Hypoxaemia in sickle cell disease: Biomarker modulation and relevance to pathophysiology. Lancet 2003;362:1450-5.
299. Jison ML, Gladwin MT. Hemolytic anemia-associated pulmonary hypertension of sickle cell disease and the nitric oxide/arginine pathway. Am J Respir Crit Care Med 2003;168:3-4.
300. Badesch DB, McLaughlin VV, Delcroix M, Vizza CD, Olschewski H, Sitbon O, et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:56S-61S.
301. Channick RN, Sitbon O, Barst RJ, Manes A, Rubin LJ. Endothelin receptor antagonists in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:62S-7S.
302. Ghofrani HA, Pepke-Zaba J, Barbera JA, Channick R, Keogh AM, Gomez-Sanchez MA, et al. Nitric oxide pathway and phosphodiesterase inhibitors in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:68S-72S.
303. Machado RF, Martyr SE, Anthi A, Kato GJ, Hunter LA, Coles WA, et al. Pulmonary hypertension in sickle cell disease: Cardiopulmonary evaluation and response to chronic phosphodiasterase 5 inhibitor therapy. Blood 2004;104:71.
304. Lattimore JD, Celermajer DS, Wilcox I. Obstructive sleep apnea and cardiovascular disease. J Am Coll Cardio I 2003;41:1429-37.
305. Hoeper MM, Seyfarth HJ, Hoeffken G, Wirtz H, Spiekerkoetter E, Pletz MW, et al. Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 2007;30:1096-102.
306. Netzer NC, Hoegel JJ, Loube D, Netzer CM, Hay B, Alvarez-Sala R, et al. Prevalence of symptoms and risk of sleep apnea in primary care. Chest 2003;124:1406-14.
307. Kessler R, Chaouat A, Weitzenblum E, Oswald M, Ehrhart M, Apprill M, et al. pulmonary hypertension in the obstructive sleep apnea syndrome: Prevalence, causes and therapeutic consequences. Eur Respir J 1996;9:787-94.
308. Alchanatis M, Tourkohoriti G, Kakouros S, Kosmas E, Podaras S, Jordanoglou JB. Daytime pulmonary hypertension in patients with obstructive apnea: The effect of continous positive airway pressure on pulmonary hemodynamics. Respiration 2001;68:666-72.
309. Bradley TD, Rutherford R, Grossman RF, Lue F, Zamel N, Moldofsky H, et al. Role of daytime hypoxemia in the pathogenesis of right heart failure in the obstructive sleep apnea syndrome. Am Rev Respir Dis 1985;131:835-9.
310. Sajkov D, Cowie RJ, Thornton AT, Espinoza HA, McEvoy RD. Pulmonary hypertension and hypoxemia in obstructive sleep apnea syndrome. Am J Respir Crit Care Med 1994;149:416-22.
311. Guidry UC, Mendes LA, Evans JC, Levy D, O'Connor GT, Larson MG, et al. Echocardiographic features of the right heart in sleep-disordered breathing: The Framingham Heart Study. Am J Respir Crit Care Med 2001;164:933-8.
312. Kessler R, Chaouat A, Schinkewitch P, Faller M, Casel S, Krieger J, et al. The obesity-hypoventilation syndrome revisited: A prospective study of 34 consecutive cases. Chest 2001;120:369-76.
313. Mehta NJ, Khan IA, Mehta RN, Gowda RM. Acute coronary syndrome in patients with human immunodeficiency virus disease. Angiology 2002;53:545-9.
314. Opravil M, Pechère M, Speich R, Joller-Jemelka HI, Jenni R, Russi EW, et al. HIV-associated primary pulmonary hypertension: A case control study. Am J Respir Crit Care Med 1997;155:990-5.
315. Humbert M, Monti G, Fartoukh M, Magnan A, Brenot F, Rain B, et al. Platelet-derived growth factor expression in primary pulmonary hypertension: Comparison of HIV seropositive and HIV seronegative patients. Eur Respir J 1998;11:554-9.
316. Ehrenreich H, Rieckmann P, Sinowatz F, Weih KA, Arthur LO, Goebel FD, et al. Potent stimulation of monocytic endothelin-1 production by HIV-1 glycoprotein 120. J Immunol 1993;150:4601-9.
317. Moser KM, Houk VN, Jones RC, Hufnagel CC. Chronic, massive thrombotic obstruction of the pulmonary arteries: Analysis of four operated cases. Circulation 1965;32:377-85.
318. 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;144:275-85.
319. Voelkel NF, Cool C, Lee SD, Wright L, Geraci MW, Tuder RM. Primary pulmonary hypertension between inflammation and cancer. Chest 1998;114:225S-30S.
320. Auger WR, Kerr KM, Kim NH, Ben-Yehuda O, Knowlton KU, Fedullo PF. Chronic thromboembolic pulmonary hypertension. Cardiol Clin 2004;22:453-66.
321. Cool CD, Rai PR, Yeager ME, Hernandez-Saavedra D, Serls AE, Bull TM, et al. Expression of human herpes virus 8 in primary pulmonary hypertension. N Engl J Med 2003;349:1113-22.
322. Hoeper MM, Mayer E, Simmoneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006;113:2011-20.
323. Fedullo PF, Auger WR, Channick RN, Moser KM, Jamieson SW. Chronic thromboembolic pulmonary hypertension. Clin Chest Med 1995;16:353-74.
324. Louis M, Thorens JB, Chevrolet JC. Calcium channel blockers, testing for primary pulmonary hypertension associated with HIV infection. Am Rev Respir Dis 1993;147:536A.
325. Ghofrani HA, Friese G, Discher T, Olschewski H, Schermuly RT, Weissmann N, et al. Inhaled iloprost is a potent acute pulmonary vasodilator in HIV-related severe pulmonary hypertension. Eur Respir J 2004;23:321-6.
326. Sitbon O, Gressin V, Speich R, et al. Bosentan in pulmonary arterial hypertension associated with HIV infection. Eur Respir J 2003;22:563s.
327. Sitbon O, Gressin V, Speich R, Macdonald PS, Opravil M, Cooper DA, et al. Bosentan for the treatment of human immunodeficiency virus-associated pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;170:1212-7.
328. Zuber JP, Calmy A, Evison JM, Hasse B, Schiffer V, Wagels T, et al. Pulmonary arterial hypertension related to HIV infection: Improved hemodynamics and survival associated with antiretroviral therapy. Clin Infect Dis 2004;38:1178-85.
329. Speich R, Jenni R, Opravil M, Jaccard R. Regression of HIV-associated pulmonary arterial hypertension and long-term survival during antiretroviral therapy. Swiss Med Wkly 2001;131:663-5.
330. Hoeper M, Galie N, Simonneau G, Rubin LJ. New treatments for pulmonary arterial hypertension. Am J Respir Crit Care Med 2002;165:1209-16.
331. Mukerjee D, St George D, Coleiro B, Knight C, Denton CP, Davar J, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: Application of a registry approach. Ann Rheum Dis 2003;62:1088-93.
332. Pan TL, Thumboo J, Boey ML. Primary and secondary pulmonary hypertension in systemic lupus erythematosus. Lupus 2000;9:338-42.
333. Jais X, Till SJ, Cynober T, Ioos V, Gaecia G, Tchernia G, et al. An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: Gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation. Hemoglobin 2003;27:139-47.
334. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. Prog Cardiovasc Dis 2002;45:225-34.
335. Yoshida S, Katayama M. Pulmonary hypertension in patients with connective tissue diseases. Nippon Rinsho 2001;59:1164-7.
336. Dorfmuller P, Perros F, Balabanian K, Humbert M. Inflammation in pulmonary arterial hypertension. Eur Respir J 2003;22: 358-63.
337. Humbert M, Chaouat A, Bertocchi M. ItinerAIR-HTAP: A French national prospective registry of pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169:A169.
338. Cool CD, Kennedy D, Voelkel NF, Tuder RM. Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection. Hum Pathol 1997;28:434-42.
339. Quismorio FP Jr, Sharma O, Koss M, Boylen T, Edmiston AW, Thornton PJ, et al. Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus. Semin Arthritis Rheum 1984;13:349-59.
340. Asherson RA, Oakley CM. Pulmonary hypertension and systemic lupus erythematosus. J Rheumatol 1986;13:1-5.
341. Esther JH, Sharp GC, Agia G. Pulmonary hypertension in patients with connective tissue disease and antibody to nuclear ribonucleoprotein. Arthritis Rheum 1981;24:S105.
342. Wiener-Kronish JP, Solinger AM, Warnock ML, Churg A, Ordonez N, Golden JA. Severe pulmonary involvement in mixed connective tissue disease. Am Rev Respir Dis 1981;124:499-503.
343. Hosoda Y, Suzuki Y, Takano M, Tojo T, Homma M. Mixed connective tissue disease with pulmonary hypertension: A clinical and pathological study. J Rheumatol 1987;14:826-30.
344. Oudiz RJ, Schilz RJ, Barst RJ, Galié N, Rich S, Rubin LJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 2004;126:420-7.
345. el-Hazmi MA, Warsy AS, Bahakim H. Blood proteins C and S in sickle cell disease. Acta Haematologica 1993;90:114-9.
346. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95.
347. Ataga K, Jones S, Olajide O, Strayhorn D, Lail A, Orringer EP. The relationship of pulmonary hypertension and survival in sickle cell disease. Blood 2004b;104:463a.
348. Haque AK, Gokhale S, Rampy BA, Adegboyega P, Duarte A, Saldana MJ. Pulmonary hypertension in sickle cell hemoglobinopathy: A clinicopathologic study of 20 cases. Hum Pathol 2002;33:1037-43.
349. Sutton LL, Castro O, Cross DJ, Spencer JE, Lewis JF. Pulmonary hypertension in sickle cell disease. Am J Cardiol 1994;74:626-8.
350. Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med 2004;350:857-9.
351. Castro LM, Jonassiant JC, Graham FL, Ashley-Koch A, Tellen MJ. Pulmonary hypertension in SS, SC and Sβ thalassemia: Prevalence, associated clinical syndromes and mortality. Blood 2004;104:462a.
352. Machado RF, Gladwin MT. Chronic sickle cell lung disease: New insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension. Br J Haematol 2005;129:449-64.
353. Aslan M, Ryan TM, Adler B, Townes TM, Parks DA, Thompson JA, et al. Oxygen radical inhibition of nitric oxide-dependent vascular function in sickle cell disease. Proc Nat Acad Sci U S A 2001;98:15215-20.
354. Hartz RS, Byrne JG, Levitsky S, Park J, Rich S. Predictors of mortality in pulmonary thromboendarterectomy. Ann Thorac Surg 1996;62:1255-9.
355. Archibald CJ, Auger WR, Fedullo PF, Channick RN, Kerr KM, Jamieson SW, et al. Long-term outcome after pulmonary thromboendarterectomy. Am J Respir Crit Care Med 1999;160:523-8.
356. Lewczuk J, Piszko P, Jagas J, Porada A, Wojciak S, Sobkowicz B, et al. Prognostic factors in medically treated patients with chronic pulmonary embolism. Chest 2001;119:818-23.
357. Bonderman D, Nowotny R, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Klepetko W, et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 2005;128:2599-603.
358. Hughes R, George P, Parameshwar J, Cafferty F, Dunning J, Morrell NW, et al. Bosentan in inoperable chronic thromboembolic pulmonary hypertension. Thorax 2005;60:707.
359. Hughes RJ, Jais X, Bonderman D, Suntharalingam J, Humbert M, Lang I, et al. Bosentan in inoperable chronic thromboembolic pulmonary hypertension: Efficacy at 1 year. Eur Respir J 2006;28:138-43.
360. Hoeper MM, Kramm T, Wilkens H, Schulze C, Schafers HJ, Welte T, et al. Bosentan therapy for inoperable chronic hromboembolic pulmonary hypertension. Chest 2005;128:2363-7.
361. Sheth A, Park JE, Ong YE, Ho TB, Madden BP. Early haemodynamic benefit of sildenafil in patients with coexisting chronic thromboembolic pulmonary hypertension and left ventricular dysfunction. Vasc Pharmacol 2005;42:41-5.
362. Ono F, Nagaya N, Okumura H, Shimizu Y, Kyotani S, Nakanishi N, et al. Effect of orally active prostacyclin analogue on survival in patients with chronic thromboembolic pulmonary hypertension without major vessel obstruction. Chest 2003;123:1583-8.
363. Scelsi L, Ghio S, Campana C, D'Armini AM, Serio A, Klersy C, et al. Epoprostenol in chronic thromboembolic pulmonary hypertension with distal lesions. Ital Heart J 2004;5:618-23.
364. Kramm T, Eberle B, Krummenauer F, Guth S, Oelert H, Mayer E. Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: Effects before and after pulmonary thromboendarterectomy. Ann Thorac Surg 2003;76:711-8.
365. Nagaya N, Sasaki N, Ando M, Ogino H, Sakamaki F, Kyotani S, et al. Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 2003;123:338-43.
366. Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002;136:515-22.
367. Stiebellehner L, Petkov V, Vonbank K, Funk G, Schenk P, Ziesche R, et al. Long-term treatment with oral sildenafil in addition to continuous IV epoprostenol in patients with pulmonary arterial hypertension. Chest 2003;123:1293-5.
368. Setty BN, Stuart MJ, Dampier C, Brodecki D, Allen JL. Hypoxaemia in sickle cell disease: Biomarker modulation and relevance to pathophysiology. Lancet 2003;362:1450-5.
369. Jison ML, Gladwin MT. Hemolytic anemia-associated pulmonary hypertension of sickle cell disease and the nitric oxide/arginine pathway. Am J Respir Crit Care Med 2003;168:3-4.
370. Badesch DB, McLaughlin VV, Delcroix M, Vizza CD, Olschewski H, Sitbon O, et al. Prostanoid therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:56S-61S.
371. Channick RN, Sitbon O, Barst RJ, Manes A, Rubin LJ. Endothelin receptor antagonists in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:62S-7S.
372. Ghofrani HA, Pepke-Zaba J, Barbera JA, Channick R, Keogh AM, Gomez-Sanchez MA, et al. Nitric oxide pathway and phosphodiesterase inhibitors in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43:68S-72S.
373. Machado RF, Martyr SE, Anthi A, Kato GJ, Hunter LA, Coles WA, et al. Pulmonary hypertension in sickle cell disease: Cardiopulmonary evaluation and response to chronic phosphodiasterase 5 inhibitor therapy. Blood 2004;104:71.
374. Lattimore JD, Celermajer DS, Wilcox I. Obstructive sleep apnea and cardiovascular disease. J Am Coll Cardio I 2003;41:1429-37.
375. Young T, Skatrud J, Peppard PE. Risk factors for obstructive sleep apnea in adults. JAMA 2004;291:2013.
376. Netzer NC, Hoegel JJ, Loube D, Netzer CM, Hay B, Alvarez-Sala R, et al. Prevalence of symptoms and risk of sleep apnea in primary care. Chest 2003;124:1406-14.
377. Kessler R, Chaouat A, Weitzenblum E, Oswald M, Ehrhart M, Apprill M, et al. pulmonary hypertension in the obstructive sleep apnea syndrome: Prevalence, causes and therapeutic consequences. Eur Respir J 1996;9:787-94.
378. Alchanatis M, Tourkohoriti G, Kakouros S, Kosmas E, Podaras S, Jordanoglou JB. Daytime pulmonary hypertension in patients with obstructive apnea: The effect of continous positive airway pressure on pulmonary hemodynamics. Respiration 2001;68:666-72.
379. Bradley TD, Rutherford R, Grossman RF, Lue F, Zamel N, Moldofsky H, et al. Role of daytime hypoxemia in the pathogenesis of right heart failure in the obstructive sleep apnea syndrome. Am Rev Respir Dis 1985;131:835-9.
380. Sajkov D, Cowie RJ, Thornton AT, Espinoza HA, McEvoy RD. Pulmonary hypertension and hypoxemia in obstructive sleep apnea syndrome. Am J Respir Crit Care Med 1994;149:416-22.
381. Guidry UC, Mendes LA, Evans JC, Levy D, O'Connor GT, Larson MG, et al. Echocardiographic features of the right heart in sleep-disordered breathing: The Framingham Heart Study. Am J Respir Crit Care Med 2001;164:933-8.
382. Kessler R, Chaouat A, Schinkewitch P, Faller M, Casel S, Krieger J, et al. The obesity-hypoventilation syndrome revisited: A prospective study of 34 consecutive cases. Chest 2001;120:369-76.
383. Arias MA, Garcia-Rio F, Alonso-Fernandez A, Martínez I, Villamor J. Pulmonary hypertension in obstructive sleep apnoea: Effects of continuous positive airway pressure: A randomized, controlled cross-over study. Eur Heart J 2006;27:1106-13.
384. Hirshkowitz M, Sharafkhaneh A. Positive airway pressure therapy of OSA. Semin Respir Crit Med 2005;26:68-79.
385. Moser KM, Houk VN, Jones RC, Hufnagel CC. Chronic, massive thrombotic obstruction of the pulmonary arteries: Analysis of four operated cases. Circulation 1965;32:377-85.
386. Moor GF, Sabiston DC Jr. Embolectomy for chronic PE and pulmonary hypertension. Rev Surg 1969;26:449-51.
387. Riedel M, Stanek V, Widimsky J, Prerovsky I. Long term follow-up of patients with pulmonary thromboembolism: Late prognosis and evolution of hemodynamic and respiratory data. Chest 1982;81:151-8.
388. Auger WR, Kerr KM, Kim NH, Ben-Yehuda O, Knowlton KU, Fedullo PF. Chronic thromboembolic pulmonary hypertension. Cardiol Clin 2004;22:453-66.
389. Galie N, Kim NH. Pulmonary microvascular disease in CTEPH. Proc Am Thorac Soc 2006;3:571-6.
390. Hoeper MM, Mayer E, Simmoneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006;113:2011-20.
391. Bonnet S, Rochefort G, Sutendra G, Archer SL, Haromy A, Webster L, et al. The nuclear factor of activated T cells in pulmonary arterial hypertension can be therapeutically targeted. Proc Natl Acad Sci USA 2007;104:11418-23.
392. Fedullo PF, Rubin LJ, Kerr KM, Auger WR, Channick RN. The natural history of acute and chronic thromboembolic disease: The search for the missing link. Eur Respir J 2000;15:435-7.
393. Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004;350:2257-64.
394. Lang IM. Chronic thromboembolic pulmonary hypertension: Not so rare after all. N Engl J Med 2004;350:2236-8.
395. Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 1990;81:1735-43.
396. Borges AC, Knebel F, Eddicks S, Panda A, Schattke S, Witt C, et al. Right ventricular function assessed by two-dimensional strain and tissue doppler echocardiography in patients with pulmonary arterial hypertension and effect of vasodilator therapy. Am J Cardiol 2006;98:530-4.
397. Egermayer P, Peacock AJ. Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J 2000;15:440-8.
398. Lang IM, Kerr K. Risk factors for CTEPH. Proc Am Thorac Soc 2006;3.
399. Hoeper MH, Lee SH, Voswinckel R, Palazzini M, Jais X, Marinelli A, et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006;48:2546-52.
400. Chou R, DeLoughery TG. Recurrent thromboembolic disease following splenectomy for pyruvate kinase deficiency. Am J Hematol 2001;67:197-9.
401. Jais X, Till SJ, Cynober T, Ioos V, Gaecia G, Tchernia G, et al. An extreme consequence of splenectomy in dehydrated hereditary stomatocytosis: Gradual thrombo-embolic pulmonary hypertension and lung-heart transplantation. Hemoglobin 2003;27:139-47.
402. Clapp LH, Finney P, Turcato S, Tran S, Rubin LJ, Tinker A. Differential effects of stable prostacyclin analogs on smooth muscle proliferation and cyclic AMP generation in human pulmonary artery. Am J Respir Cell Mol Biol 2002;26:194-201.
403. D'Alonzo GE, Bower JS, Dantzker DR. Differentiation of patients with primary and thromboembolic pulmonary hypertension. Chest 1984;85:457-61.
404. Bergin CJ, Rios G, King MA, Belezzuoli E, Luna J, Auger WR. Accuracy of highresolution CT in identifying chronic pulmonary thromboembolic disease. AJR Am J Roentgenol 1996;166:1371-7.
405. Bergin CJ, Sirlin CB, Hauschildt JP, Huynh TV, Auger WR, Fedullo PF, et al. Chronic thromboembolism: Diagnosis with helical CT and MR imaging with angiographic and surgical correlation. Radiology 1997;204:695-702.
406. Gatzoulis, MA, Rogers P, Li W, Harries C, Cramer D, Ward S, et al. Safety and tolerability of bosentan in adults with Eisenmenger physiology. Int J Cardiol 2005;98:147-51.
407. Schoepf UJ, Becker CR, Hofmann LK, Das M, Flohr T, Ohnesorge BM, et al. Multislice CT angiography. Eur Radiol 2003;13:1946-61.
408. Benza RL, Mehta S, Keogh A, Lawrence EC, Oudiz RJ, Barst RJ. Sitaxsentan treatment for patients with pulmonary arterial hypertension discontinuing bosentan. J Heart Lung Transplant 2007;26:63-9.
409. Ghofrani HA, Osterloh IH, Grimminger F. Sildenafil: From angina to erectile dysfunction to pulmonary hypertension and beyond. Nat Rev Drug Discov 2006;5:689-702.
410. Hatabu H, Gefter WB, Axel L, Palevsky HI, Cope C, Reichek N, et al. MR imaging with spatial modulation of magnetization in the evaluation of chronic central pulmonary thromboemboli. Radiology 1994;190:791-6.
411. Oudiz RJ, Roveran G, Hansen JE, Sun XG, Wasserman K. Effect of sildenafil on ventilatory efficiency and exercise tolerance in pulmonary hypertension. Eur J Heart Fail 2007;9:917-21.
412. Fraunfelder FW, Pomeranz HD, Egan RA. Nonarteritic anterior ischemic optic neuropathy and sildenafil. Arch Ophthalmol 2006;124:733-4.
413. Nakayama Y, Sugimachi M, Nakanishi N, Takaki H, Okano Y, Satoh T, et al. Noninvasive differential diagnosis between chronic pulmonary thromboembolism and primary pulmonary hypertension by means of Doppler ultrasound measurement. J Am Coll Cardiol 1998;31:1367-71.
414. Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002;136:515-22.
415. Langleben D, Christman BW, Barst RJ, Dias VC, Galiè N, Higenbottam TW, et al. Effects of the thromboxane synthetase inhibitor and receptor antagonist terbogrel in patients with primary pulmonary hypertension. Am Heart J 2002;143:E4.
416. Nicod P, Peterson K, Levine M, Dittrich H, Buchbinder M, Chappuis F, et al. Pulmonary angiography in severe chronic pulmonary hypertension. Ann Intern Med 1987;107:565-8.
417. Rothman A, Sklansky MS, Lucas VW, Kashani IA, Shaughnessy RD, Channick RN, et al. Atrial septostomy as a bridge to lung transplantation in patients with severe pulmonary hypertension. Am J Cardiol 1999;84:682-6.
418. Law MA, Grifka RG, Mullins CE, Nihill MR. Atrial septostomy improves survival in select patients with pulmonary hypertension. Am Heart J 2007;153:779-84.
419. Kim NH. Assessment of operability in chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc 2006;3:584-8.
420. Kim NH, Fesler P, Channick RN, Knowlton KU, Ben-Yehuda O, Lee SH, et al. Pre-operative partitioning of pulmonary vascular resistance correlates with early outcome after thromboendarterectomy for chronic thromboembolic pulmonary hypertension. Circulation 2004;109:18-22.
421. Diller GP, Dimopoulos K, Broberg GS, Kaya MG, Naghotra US, Uebing A, et al. Presentation, survival prospects and predictors of death in Eisenmenger syndrome: A combined retrospective and case-control study. Eur Heart J 2006;27:1737-42.
422. 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 2004;25:1651-6.
423. Lewczuk J, Piszko P, Jagas J, Porada A, Wojciak S, Sobkowicz B, et al. Prognostic factors in medically treated patients with chronic pulmonary embolism. Chest 2001;119:818-23.
424. Bonderman D, Nowotny R, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Klepetko W, et al. Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 2005;128:2599-603.
425. Hughes R, George P, Parameshwar J, Cafferty F, Dunning J, Morrell NW, et al. Bosentan in inoperable chronic thromboembolic pulmonary hypertension. Thorax 2005;60:707.
426. Colle IO, Moreau R, Godinho E, Belghiti J, Ettori F, Cohen-Solal A, et al. diagnosis of portopulmonary hypertension in candidates for liver transplantation: A prospective study. Hepatology 2003;37:401-9.
427. Hoeper MM, Kramm T, Wilkens H, Schulze C, Schafers HJ, Welte T, et al. Bosentan therapy for inoperable chronic hromboembolic pulmonary hypertension. Chest 2005;128:2363-7.
428. Sheth A, Park JE, Ong YE, Ho TB, Madden BP. Early haemodynamic benefit of sildenafil in patients with coexisting chronic thromboembolic pulmonary hypertension and left ventricular dysfunction. Vasc Pharmacol 2005;42:41-5.
429. Ono F, Nagaya N, Okumura H, Shimizu Y, Kyotani S, Nakanishi N, et al. Effect of orally active prostacyclin analogue on survival in patients with chronic thromboembolic pulmonary hypertension without major vessel obstruction. Chest 2003;123:1583-8.
430. Scelsi L, Ghio S, Campana C, D'Armini AM, Serio A, Klersy C, et al. Epoprostenol in chronic thromboembolic pulmonary hypertension with distal lesions. Ital Heart J 2004;5:618-23.
431. Kramm T, Eberle B, Krummenauer F, Guth S, Oelert H, Mayer E. Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: Effects before and after pulmonary thromboendarterectomy. Ann Thorac Surg 2003;76:711-8.
432. Nagaya N, Sasaki N, Ando M, Ogino H, Sakamaki F, Kyotani S, et al. Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 2003;123:338-43.
433. Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, et al. Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 2002;136:515-22.
434. Stiebellehner L, Petkov V, Vonbank K, Funk G, Schenk P, Ziesche R, et al. Long-term treatment with oral sildenafil in addition to continuous IV epoprostenol in patients with pulmonary arterial hypertension. Chest 2003;123:1293-5.
435. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation 2001;104:429-35.
436. Yuan JX, Aldinger AM, Juhaszova M, Wang J, Conte JV Jr, Gaine SP, et al. Dysfunctional voltage-gated K+ channels in pulmonary artery smooth muscle cells of patients with primary pulmonary hypertension. Circulation 1998;98:1400-6.
437. Perloff JK, Hart EM, Greaves SM, Miner PD, Child JS. Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 2003;92:182-7.
438. Galie N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension (The task force on diagnosis and treatment of pulmonary arterial hypertension of the European Society of Cardiology). Eur Heart J 2004;25:2243-78.
439. Naeije R, Vachiéry JL. Medical therapy of pulmonary hypertension. Conventional therapies. Clin Chest Med 2001;22:517-27.
440. Kuriyama K, Gamsu G, Stern RG, Cann CE, Herfkens RJ, Brundage BH. CT-determined pulmonary artery diameters in predicting pulmonary hypertension. Invest Radiol 1984;19:16-22.
441. Tan RT, Kuzo R, Goodman LR, Siegel R, Haasler GB, Presberg KW. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Chest 1998;113:1250-6.
442. Choe KO, Hong YK, Kim HJ, Joo SH, Cho BK, Chang BC, et al. The use of high resolution computed tomography in the evaluation of pulmonary hemodynamics in patients with congenital heart disease: In pulmonary vessels larger than 1 mm in diameter. Pediatr Cardiol 2000;21:202-10.
443. Ng CS, Wells AU, Padley SP. A CT sign of chronic pulmonary arterial hypertension: The ratio of main pulmonary artery to aortic diameter. J Thorac Imaging 1999;14:270-8.
444. Baque-Juston MC, Wells AU, Hansell DM. Pericardial thickening or effusion in patients with pulmonary artery hypertension: A CT study. AJR Am J Roentgenol 1999;172:361-4.
445. Rubin LJ, Galie N, Badesch BD. Ambrisentan improves exercise capacity and clinical measures in pulmonary arterial hypertension (PAH). Am J Crit Care Med 2004;169:A210.
446. Doyle RL, McCrory D, Channick RN, Simonneau G, Conte J; American College of Chest Physicians. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004;126:63S-71S.