2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

European Heart Journal

Volumn 37, Issue 1, 2016, Pages 67-119

  Galiè, Nazzareno ^a,b   Humbert, Marc ^a,c   Vachiery, Jean Luc ^a   Gibbs, Simon ^a   Lang, Irene ^a   Torbicki, Adam ^a   Simonneau, Gérald ^a   Peacock, Andrew ^a   Vonk Noordegraaf, Anton ^a   Beghetti, Maurice ^a   Ghofrani, Ardeschir ^a   Gomez Sanchez, Miguel Angel ^a   Hansmann, Georg ^a   Klepetko, Walter ^a   Lancellotti, Patrizio ^a   Matucci, Marco ^a   McDonagh, Theresa ^a   Pierard, Luc A ^a   Trindade, Pedro T ^a   Zompatori, Maurizio ^a   Hoeper, Marius ^a   Aboyans, Victor ^a   Vaz Carneiro, Antonio ^a   Achenbach, Stephan ^a   Agewall, Stefan ^a   Allanore, Yannick ^a   Asteggiano, Riccardo ^a   Paolo Badano, Luigi ^a   Albert Barberà, Joan ^a   Bouvaist, Hélène ^a   Bueno, Héctor ^a   Byrne, Robert A ^a   Carerj, Scipione ^a   Castro, Graça ^a   Erol, Çetin ^a   Falk, Volkmar ^a   Funck Brentano, Christian ^a   Gorenflo, Matthias ^a   Granton, John ^a   Iung, Bernard ^a   Kiely, David G ^a   Kirchhof, Paulus ^a   Kjellstrom, Barbro ^a   Landmesser, Ulf ^a   Lekakis, John ^a   Lionis, Christos ^a   Lip, Gregory Y H ^a   Orfanos, Stylianos E ^a   Park, Myung H ^a   Piepoli, Massimo F ^a   Ponikowski, Piotr ^a   Revel, Marie Pierre ^a   Rigau, David ^a   Rosenkranz, Stephan ^a   Völler, Heinz ^a   Luis Zamorano, Jose ^a   Myftiu, Sokol ^d   Bonderman, Diana ^e   Firdovsi, Ibrahimov ^f   Lazareva, Irina ^g   De Pauw, Michel ^h   Sokolović, Šekib ⁱ   Velchev, Vasil ^j   Čikeš, Maja ^k   Moutiris, Josef Antoniou ^l   Jansa, Pavel ^m   Nielsen Kudsk, Jens Erik ⁿ   Anton, Ly ^o   Jääskeläinen, Pertti ^p   Bauer, Fabrice ^q   Chukhrukidze, Archil ^r   Opitz, Christian ^s   Giannakoulas, George ^t   Karlócai, Kristóf ^u   Oddsson, Hjörtur ^v   Gaine, Sean ^w   Menachemi, Doron ^x   Emdin, Michele ^y   Sooronbaev, Talant ^z   Rudzītis, Ainars ^aa   Gumbiene, Lina ^ab   Lebrun, Frederic ^ac   Micallef, Josef ^ad   Botnaru, Victor ^ae   Oukerraj, Latifa ^af   Andreassen, Arne K ^ag   Kurzyna, Marcin ^ah   Leite Baptista, Maria João Ribeiro ^ai   Coman, Ioan Mircea ^aj   Moiseeva, Olga ^ak   Stefanović, Branislav S ^al   Šimková, Iveta ^am   Wikström, Gerhard ^an   Schwerzmann, Markus ^ao   Srbinovska Kostovska, Elizabeta ^ap   van Dijk, Arie P J ^aq   Mahdhaoui, Abdallah ^ar   Kaymaz, Cihangir ^as   Coghlan, Gerry ^at   Sirenko, Yuriy ^au   more..

^a University of Bologna ^*  (United States)

^b UNIVERSITY OF BOLOGNA   (Italy)

^c BICÊTRE HOSPITAL   (France)

^d Albanian Society of Cardiology   (Albania)

^e Austrian Society of Cardiology   (Austria)

^f Azerbaijan Society of Cardiology   (Azerbaijan)

^g Belorussian Scientific Society of Cardiologists   (Belarus)

^h Belgian Society of Cardiology   (Belgium)

ⁱ Association of Cardiologists of Bosnia Herzegovina   (Bosnia and Herzegovina)

^j Bulgarian Society of Cardiology   (Bulgaria)

^k Croatian Cardiac Society   (Croatia)

^l Cyprus Society of Cardiology   (Cyprus)

^m Czech Society of Cardiology   (Czech Republic)

ⁿ Danish Society of Cardiology   (Denmark)

^o Estonian Society of Cardiology   (Estonia)

^p Finnish Cardiac Society   (Finland)

^q French Society of Cardiology   (France)

^r Georgian Society of Cardiology   (Georgia)

^s German Cardiac Society   (Germany)

^t Hellenic Cardiological Society   (Greece)

^u Hungarian Society of Cardiology   (Hungary)

^v Icelandic Society of Cardiology   (Iceland)

^w IRISH HEART FOUNDATION   (Ireland)

^x Israel Heart Society   (Israel)

^y Italian Federation of Cardiology   (Italy)

^z Kyrgyz Society of Cardiology   (Kyrgyzstan)

^aa Latvian Society of Cardiology   (Latvia)

^ab Lithuanian Society of Cardiology   (Lithuania)

^ac Luxembourg Society of Cardiology   (Luxembourg)

^ad Maltese Cardiac Society   (Malta)

^ae Moldavian Society of Cardiology   (Moldova)

^af Moroccan Society of Cardiology   (Morocco)

^ag Norwegian Society of Cardiology   (Norway)

^ah Polskie Towarzystwo Kardiologiczne   (Poland)

^ai Portuguese Society of Cardiology   (Portugal)

^aj Romanian Society of Cardiology   (Romania)

^ak Russian Journal of Cardiology   (Russian Federation)

^al Cardiology Society of Serbia   (Serbia)

^am Slovak Society of Cardiology   (Slovakia)

^an Swedish Society of Cardiology   (Sweden)

^ao Swiss Society of Cardiology   (Switzerland)

^ap Macedonian FYR Society of Cardiology   (Macedonia)

^aq Netherlands Society of Cardiology (NVVC)   (Netherlands)

^ar Tunisian Society of Cardiology and Cardio Vascular Surgery   (Tunisia)

^as Turkish Society of Cardiology   (Turkey)

^at British Cardiovascular Society   (United Kingdom)

^au Ukrainian Association of Cardiology   (Ukraine)

more..

Author keywords

Chronic thromboembolic pulmonary hypertension; Congenital heart disease; Connective tissue disease; Endothelin receptor antagonists; Guidelines; Heart failure; Left heart disease; Lung disease; Phosphodiesterase type 5 inhibitors; Prostacyclin analogues; Pulmonary arterial hypertension; Pulmonary hypertension; Respiratory failure

Indexed keywords

ANTICOAGULANT AGENT; BIOCHEMICAL MARKER; CALCIUM CHANNEL BLOCKING AGENT; CARDIOVASCULAR AGENT; DIGOXIN; DIURETIC AGENT; ENDOTHELIN RECEPTOR ANTAGONIST; GUANYLATE CYCLASE ACTIVATOR; OXYGEN; PHOSPHODIESTERASE V INHIBITOR; PROSTACYCLIN DERIVATIVE; ANTIHYPERTENSIVE AGENT; BIOLOGICAL MARKER;

ANEMIA; ARTERIAL GAS; ARTICLE; BALLOON ATRIAL SEPTOSTOMY; BLOOD ANALYSIS; CARDIOVASCULAR MAGNETIC RESONANCE; CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; CONGENITAL HEART DISEASE; CONNECTIVE TISSUE DISEASE; DISEASE SEVERITY; ECHOCARDIOGRAPHY; ELECTIVE SURGERY; ELECTROCARDIOGRAM; EXERCISE; GENETIC COUNSELING; GENETIC SCREENING; GENETICS; HEART ARRHYTHMIA; HEART CATHETERIZATION; HEART RIGHT VENTRICLE FAILURE; HEMODYNAMICS; HEMOPTYSIS; HORMONAL THERAPY; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; HYPOXIA; INFECTION PREVENTION; INTENSIVE CARE; LUNG DISEASE; LUNG FUNCTION TEST; PATIENT COMPLIANCE; PHYSICAL ACTIVITY; PORTAL HYPERTENSION; PREGNANCY; PRIORITY JOURNAL; PSYCHOSOCIAL CARE; PULMONARY CAPILLARY HEMANGIOMATOSIS; PULMONARY HYPERTENSION; PULMONARY VENO-OCCLUSIVE DISEASE; REHABILITATION CARE; RISK ASSESSMENT; RISK FACTOR; TERMINAL CARE; THORAX RADIOGRAPHY; TRANSPLANTATION; TRAVEL; ADULT; ALGORITHM; ARRHYTHMIAS, CARDIAC; BALLOON EMBOLECTOMY; CHILD; COMPLICATION; CONNECTIVE TISSUE DISEASES; CROSS INFECTION; DRUG INTERACTION; ELECTROCARDIOGRAPHY; EMPORIATRICS; EXERCISE TEST; EXPERIMENTAL THERAPY; FEMALE; HEALTH STATUS; HEART DEFECTS, CONGENITAL; HEART RIGHT VENTRICLE FUNCTION; HEMANGIOMA; HIV INFECTIONS; HYPERTENSION, PORTAL; HYPERTENSION, PULMONARY; KINESIOTHERAPY; LUNG TRANSPLANTATION; MAGNETIC RESONANCE ANGIOGRAPHY; METABOLISM; MULTIMODAL IMAGING; MULTIMODALITY CANCER THERAPY; PATIENT REFERRAL; PRACTICE GUIDELINE; PREGNANCY COMPLICATIONS, CARDIOVASCULAR; PROCEDURES; SOCIAL SUPPORT; THROMBOEMBOLISM; TREATMENT OUTCOME; X-RAY COMPUTED TOMOGRAPHY;

ADULT; ALGORITHMS; ANTIHYPERTENSIVE AGENTS; ARRHYTHMIAS, CARDIAC; BALLOON EMBOLECTOMY; BIOMARKERS; CARDIAC CATHETERIZATION; CHILD; COMBINED MODALITY THERAPY; CONNECTIVE TISSUE DISEASES; CROSS INFECTION; DRUG INTERACTIONS; ECHOCARDIOGRAPHY; ELECTIVE SURGICAL PROCEDURES; ELECTROCARDIOGRAPHY; EXERCISE TEST; EXERCISE THERAPY; FEMALE; GENETIC COUNSELING; GENETIC TESTING; HEALTH STATUS; HEART DEFECTS, CONGENITAL; HEMANGIOMA; HEMOPTYSIS; HIV INFECTIONS; HUMANS; HYPERTENSION, PORTAL; HYPERTENSION, PULMONARY; LUNG TRANSPLANTATION; MAGNETIC RESONANCE ANGIOGRAPHY; MULTIMODAL IMAGING; PATIENT COMPLIANCE; PREGNANCY; PREGNANCY COMPLICATIONS, CARDIOVASCULAR; REFERRAL AND CONSULTATION; RESPIRATORY FUNCTION TESTS; RISK ASSESSMENT; RISK FACTORS; SOCIAL SUPPORT; TERMINAL CARE; THERAPIES, INVESTIGATIONAL; THROMBOEMBOLISM; TOMOGRAPHY, X-RAY COMPUTED; TRAVEL MEDICINE; TREATMENT OUTCOME; VENTRICULAR DYSFUNCTION, RIGHT;

EID: 84958184473     PISSN: 0195668X     EISSN: 15229645     Source Type: Journal    
DOI: 10.1093/eurheartj/ehv317     Document Type: Article

References (456)

1. Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, KhannaD, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 2013;62(Suppl):D42-D50.
2. Kovacs G, Berghold A, Scheidl S, Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects A systematic review. Eur Respir J 2009;34: 888-894.
3. Herve P, Lau E, Sitbon O, Savale L, Montani D, Godinas L, Lador F, Jäs X, Parent F, Günther S, Humbert M, Simonneau G, Chemla D. Criteria for diagnosis of exercise pulmonary hypertension. Eur Respir J 2015;46:728-737.
4. Vachiery JL, Adir Y, Barbera JA, Champion HC, Coghlan JG, Cottin V, DeMarco T, Galiè N, Ghio S, Gibbs JS, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Seeger W. Pulmonary hypertension due to left heart disease. J Am Coll Cardiol 2013;62:D100-D108.
5. Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004;43(Suppl 1):S5-S12.
6. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, Gomez Sanchez MA, Krishna Kumar R, Landzberg M, Machado RF, Olschewski H, Robbins IM, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013;62:D34-D41.
7. Dhillon R. The management of neonatal pulmonary hypertension. Arch Dis Child Fetal Neonatal Ed 2012;97:F223-F228.
8. Porta NF, Steinhorn RH. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin Perinatol 2012; 39:149-164.
9. Ivy DD, Abman SH, Barst RJ, Berger RMF, Bonnet D, Fleming TR, Haworth SG, Raj JU, Rosenzweig EB, Schulze Neick I, Steinhorn RH, Beghetti M. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013;62:D117-D126.
10. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, Yaici A, Weitzenblum E, Cordier JF, Chabot F, Dromer C, Pison C, Reynaud-Gaubert M, Haloun A, Laurent M, Hachulla E, Simonneau G. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006;173:1023-1030.
11. Peacock AJ, Murphy NF, McMurray JJV, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007;30:104-109.
12. McGoon MD, Benza RL, Escribano-Subias P, Jiang X, Miller DP, Peacock AJ, Pepke-Zaba J, Pulido T, Rich S, Rosenkranz S, Suissa S, Humbert M. Pulmonary arterial hypertension: epidemiology and registries. J Am Coll Cardiol 2013; 62(Suppl):D51-D59.
13. Simonneau G, Robbins I, Beghetti M, Channick RN, Delcroix M, Denton CP, Elliott CG, Gaine S, Gladwin MT, Jing ZC, Krowka MJ, Langleben D, Nakanishi N, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54(Suppl):S43-S54.
14. Montani D, Bergot E, Günther S, Savale L, Bergeron A, Bourdin A, Bouvaist H, Canuet M, Pison C, Macro M, Poubeau P, Girerd B, Natali D, Guignabert C, Perros F, O'Callaghan DS, Jäs X, Tubert-Bitter P, Zalcman G, Sitbon O, Simonneau G, Humbert M. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation 2012;125:2128-2137.
15. Savale L, Chaumais MC, Cottin V, Bergot E, Frachon I, Prevot G, Pison C, Dromer C, Poubeau P, Lamblin N, Habib G, Reynaud-Gaubert M, Bourdin A, Sanchez O, Tubert-Bitter P, Jaïs X, Montani D, Sitbon O, Simonneau G, Humbert M. Pulmonary hypertension associated with benfluorex exposure. Eur Respir J 2012;40:1164-1172.
16. Savale L, Sattler C, Gunther S, Montani D, Chaumais MC, Perrin S, Jaïs X, Seferian A, Jovan R, Bulifon S, Parent F, Simonneau G, Humbert M, Sitbon O. Pulmonary arterial hypertension in patients treated with interferon. Eur Respir J 2014; 44:1627-1634.
17. Badesch BD, Champion HC, Gomez-Sanchez MA, Hoeper M, Loyd J, Manes A, McGoon M, Naeije R, Olschewski H, Oudiz R, Torbicki A. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(Suppl):S55-S56.
18. Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med 2007;28:233-241.
19. Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Baron-Esquivias G, Baumgartner H, Borger MA, Carrel TP, De Bonis M, Evangelista A, Falk V, Iung B, Lancellotti P, Pierard L, Price S, Schafers HJ, Schuler G, Stepinska J, Swedberg K, Takkenberg J, Von Oppell UO, Windecker S, Zamorano JL, Zembala M. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J 2012;33:2451-2496.
20. Seeger W, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, De Marco T, Galiè N, Ghio S, Gibbs S, Martinez FJ, Semigran MJ, Simonneau G, Wells AU, Vachiéry JL. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol 2013;62(Suppl):D109-D116.
21. Hurdman J, Condliffe R, Elliot CA, Swift A, Rajaram S, Davies C, Hill C, Hamilton N, Armstrong IJ, Billings C, Pollard L, Wild JM, Lawrie A, Lawson R, Sabroe I, Kiely DG. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur Respir J 2013;41:1292-1301.
22. Cottin V, Nunes H, Brillet PY, Delaval P, Devouassoux G, Tillie-Leblond I, Israel-Biet D, Court-Fortune, Valeyre D, Cordier JF. Combined pulmonary fibrosis and emphysema: A distinct underrecognised entity. Eur Respir J 2005;26: 586-593.
23. Escribano-Subias P, Blanco I, Lopez-Meseguer M, Lopez-Guarch CJ, Roman A, Morales P, Castillo-Palma MJ, Segovia J, Gomez-Sanchez MA, Barbera JA. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur Respir J 2012;40:596-603.
24. Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004;350: 2257-2264.
25. Pepke-Zaba J, Delcroix M, Lang I, Mayer E, Jansa P, Ambroz D, Treacy C, D'Armini AM, Morsolini M, Snijder R, Bresser P, Torbicki A, Kristensen B, Lewczuk J, Simkova I, Barberà JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jaïs X, Simonneau G. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011;124:1973-1981.
26. Soubrier F, Chung WK, Machado R, Grunig E, Aldred M, Geraci M, Loyd JE, Elliott CG, Trembath RC, Newman JH, Humbert M. Genetics and genomics of pulmonary arterial hypertension. J Am Coll Cardiol 2013;62(Suppl):D13-D21.
27. Ma L, Roman-Campos D, Austin ED, Eyries M, Sampson KS, Soubrier F, Germain M, Tregouet DA, Borczuk A, Rosenzweig EB, Girerd B, Montani D, Humbert M, Loyd JE, Kass RS, Chung WK. A novel channelopathy in pulmonary arterial hypertension. N Engl J Med 2013;369:351-361.
28. Eyries M, Montani D, Girerd B, Perret C, Leroy A, Lonjou C, Chelghoum N, Coulet F, Bonnet D, Dorfmuller P, Fadel E, Sitbon O, Simonneau G, Tregouet DA, Humbert M, Soubrier F. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet 2014;46:65-69.
29. 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;108:1839-1844.
30. Bonderman D, Wexberg P, Martischnig AM, Heinzl H, Lang MB, Sadushi R, Skoro-Sajer N, Lang IM. A noninvasive algorithm to exclude pre-capillary pulmonary hypertension. Eur Respir J 2011;37:1096-1103.
31. Rich JD, Thenappan T, Freed B, Patel AR, Thisted RA, Childers R, Archer SL. QTc prolongation is associated with impaired right ventricular function and predicts mortality in pulmonary hypertension. Int J Cardiol 2013;167:669-676.
32. Sun PY, Jiang X, Gomberg-Maitland M, Zhao QH, He J, Yuan P, Zhang R, Jing ZC. Prolonged QRS duration: A new predictor of adverse outcome in idiopathic pulmonary arterial hypertension. Chest 2012;141:374-380.
33. Olsson KM, Nickel NP, Tongers J, Hoeper MM. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int J Cardiol 2013;167:2300-2305.
34. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, Fishman AP, Goldring RM, Groves BM, Koerner SK. Primary pulmonary hypertension: A national prospective study. Ann Intern Med 1987;107:216-223.
35. Milne EN. Forgotten gold in diagnosing pulmonary hypertension: The plain chest radiograph. Radiographics 2012;32:1085-1087.
36. Trip P, Nossent EJ, de Man FS, van den Berk IA, Boonstra A, Groepenhoff H, Leter EM, Westerhof N, Grunberg K, Bogaard HJ, Vonk-Noordegraaf A. Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses. Eur Respir J 2013;42:1575-1585.
37. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003;41:1028-1035.
38. Hoeper MM, Pletz MW, Golpon H, Welte T. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2007; 29:944-950.
39. Pellegrino R, Viegi G, Brusasco V, Crapo RO, Burgos F, Casaburi R, Coates A, van der Grinten CP, Gustafsson P, Hankinson J, Jensen R, Johnson DC, MacIntyre N, McKay R, Miller MR, Navajas D, Pedersen OF, Wanger J. Interpretative strategies for lung function tests. Eur Respir J 2005;26:948-968.
40. Holverda S, Bogaard HJ, Groepenhoff H, Postmus PE, Boonstra A, Vonk-Noordegraaf A. Cardiopulmonary exercise test characteristics in patients with chronic obstructive pulmonary disease and associated pulmonary hypertension. Respiration 2008;76:160-167.
41. Jilwan FN, Escourrou P, Garcia G, Jaïs X, Humbert M, Roisman G. High occurrence of hypoxemic sleep respiratory disorders in precapillary pulmonary hypertension and mechanisms. Chest 2013;143:47-55.
42. Rafanan AL, Golish JA, Dinner DS, Hague LK, Arroliga AC. Nocturnal hypoxemia is common in primary pulmonary hypertension. Chest 2001;120:894-899.
43. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB. Guidelines for the echocardiographic assessment of the right heart in adults: A report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 2010;23:685-713.
44. Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T, Lancellotti P, Muraru D, Picard MH, Rietzschel ER, Rudski L, Spencer KT, TsangW, Voigt JU. Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging 2015;16: 233-271.
45. Foale R, Nihoyannopoulos P, McKenna W, Kleinebenne A, Nadazdin A, Rowland E, Smith G. Echocardiographic measurement of the normal adult right ventricle. Br Heart J 1986;56:33-44.
46. Hachulla E, Gressin V, Guillevin L, Carpentier P, Diot E, Sibilia J, Kahan A, Cabane J, Frances C, Launay D, Mouthon L, Allanore Y, Kiet PT, 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;52: 3792-3800.
47. Tunariu N, Gibbs SJR, Win Z, Gin-Sing W, Graham A, Gishen P, AL-Nahhas A. Ventilation-perfusion scintigraphy Is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med 2007;48:680-684.
48. Meng JJ, Zhang LJ, Wang Q, Fang W, Dai HJ, Yan J, Wang T, Yao ZM, He J, Li M, Mi HZ, Jiao J, Zheng YM. [A comparison of ventilation/perfusion single photon emission CT and CT pulmonary angiography for diagnosis of pulmonary embolism]. Zhonghua Jie He He Hu Xi Za Zhi 2013;36:177-181.
49. Rajaram S, Swift AJ, Telfer A, Hurdman J, Marshall H, Lorenz E, Capener D, Davies C, Hill C, Elliot C, Condliffe R, Wild JM, Kiely DG. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax 2013;68: 677-678.
50. Rajaram S, Swift AJ, Condliffe R, Johns C, Elliot CA, Hill C, Davies C, Hurdman J, Sabroe I, Wild JM, Kiely DG. CT features of pulmonary arterial hypertension and its major subtypes: A systematic CT evaluation of 292 patients from the ASPIRE Registry. Thorax 2015;70:382-387.
51. Shen Y, Wan C, Tian P, WuY, Li X, Yang T, An J, Wang T, Chen L, Wen F. CT-base pulmonary artery measurement in the detection of pulmonary hypertension: A meta-analysis and systematic review. Medicine (Baltimore) 2014;93:e256.
52. 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;113:1250-1256.
53. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, Simonneau G, Musset D. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am J Roentgenol 2004;183:65-70.
54. Dartevelle P, Fadel E, Mussot S, Chapelier A, Herve P, de Perrot M, Cerrina J, Ladurie FL, Lehouerou D, Humbert M, Sitbon O, Simonneau G. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2004;23:637-648.
55. Reichelt A, Hoeper MM, Galanski M, Keberle M. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur J Radiol 2008;71:49-54.
56. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med 2001;345:1465-1472.
57. Fukui S, Ogo T, Morita Y, Tsuji A, Tateishi E, Ozaki K, Sanda Y, Fukuda T, Yasuda S, Ogawa H, Nakanishi N. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur Respir J 2014;43:1394-1402.
58. Castaner E, Alguersuari A, Andreu M, Gallardo X, Spinu C, Mata JM. Imaging findings in pulmonary vasculitis. Semin Ultrasound CT MR 2012;33:567-579.
59. Nawaz A, Litt HI, Stavropoulos SW, Charagundla SR, Shlansky-Goldberg RD, Freiman DB, Chittams J, Pyeritz RE, Trerotola SO. Digital subtraction pulmonary arteriography versus multidetector CT in the detection of pulmonary arteriovenous malformations. J Vasc Interv Radiol 2008;19:1582-1588.
60. Peacock AJ, Vonk Noordegraaf A. Cardiac magnetic resonance imaging in pulmonary arterial hypertension. Eur Respir Rev 2013;22:526-534.
61. Swift AJ, Rajaram S, Condliffe R, Capener D, Hurdman J, Elliot CA, Wild JM, Kiely DG. Diagnostic accuracy of cardiovascular magnetic resonance imaging of right ventricular morphology and function in the assessment of suspected pulmonary hypertension results from the ASPIRE registry. J Cardiovasc Magn Reson 2012;14:40-50.
62. Swift AJ, Rajaram S, Hurdman J, Hill C, Davies C, Sproson TW, Morton AC, Capener D, Elliot C, Condliffe R, Wild JM, Kiely DG. Noninvasive estimation of PA pressure, flow, and resistance with CMR imaging: derivation and prospective validation study from the ASPIRE registry. JACC Cardiovasc Imaging 2013;6: 1036-1047.
63. Ley S, Kauczor HU, Heussel CP, Kramm T, Mayer E, Thelen M, Kreitner KF. Value of contrast-enhanced MR angiography and helical CT angiography in chronic thromboembolic pulmonary hypertension. Eur Radiol 2003;13:2365-2371.
64. van Wolferen SA, Marcus JT, Boonstra A, Marques KMJ, Bronzwaer JGF, Spreeuwenberg MD, Postmus PE, Vonk-Noordegraaf A. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J 2007;28:1250-1257.
65. Peacock AJ, Crawley S, McLure L, Blyth K, Vizza CD, Poscia R, Francone M, Iacucci I, Olschewski H, Kovacs G, VonkNoordegraaf A, Marcus JT, van de Veerdonk MC, Oosterveer FPT. Changes in right ventricular function measured by cardiac magnetic resonance imaging in patients receiving pulmonary arterial hypertension-targeted therapy: The EURO-MR Study. Circ Cardiovasc Imaging 2014;7:107-114.
66. van de Veerdonk MC, Kind T, Marcus JT, Mauritz GJ, Heymans MW, Bogaard HJ, Boonstra A, Marques KM, Westerhof N, Vonk-Noordegraaf A. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol 2011;58:2511-2519.
67. Albrecht T, Blomley MJ, Cosgrove DO, Taylor-Robinson SD, Jayaram V, Eckersley R, Urbank A, Butler-Barnes J, Patel N. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet 1999;353: 1579-1583.
68. Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. Swiss Med Wkly 2003;133:163-169.
69. Hoeper MM, Lee SH, Voswinckel R, Palazzini M, Jaïs X, Marinelli A, Barst RJ, Ghofrani HA, Jing ZC, Opitz C, Seyfarth HJ, Halank M, McLaughlin V, Oudiz RJ, Ewert R, Wilkens H, Kluge S, Bremer HC, Baroke E, Rubin LJ. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 2006;48:2546-2552.
70. Kovacs G, Avian A, Pienn M, Naeije R, Olschewski H. Reading pulmonary vascular pressure tracings. How to handle the problems of zero leveling and respiratory swings. Am J Respir Crit Care Med 2014;190:252-257.
71. Hoeper MM, Maier R, Tongers J, Niedermeyer J, Hohlfeld JM, Hamm M, Fabel H. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am J Respir Crit Care Med 1999;160: 535-541.
72. Frost AE, FarberHW, Barst RJ, Miller DP, Elliott CG, McGoon MD. Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16 to 18mm Hg: insights from the REVEAL Registry. Chest 2013;143:185-195.
73. AbrahamWT, Adamson PB, Bourge RC, Aaron MF, Costanzo MR, Stevenson LW, Strickland W, Neelagaru S, Raval N, Krueger S, Weiner S, Shavelle D, Jeffries B, Yadav JS. Wireless pulmonary artery haemodynamic monitoring in chronic heart failure: A randomised controlled trial. Lancet 2011;377:658-666.
74. Prasad A, Hastings JL, Shibata S, Popovic ZB, Arbab-Zadeh A, Bhella PS, Okazaki K, Fu Q, Berk M, Palmer D, Greenberg NL, Garcia MJ, Thomas JD, Levine BD. Characterization of static and dynamic left ventricular diastolic function in patients with heart failure with a preserved ejection fraction. Circ Heart Fail 2010;3:617-626.
75. Fujimoto N, Borlaug BA, Lewis GD, Hastings JL, Shafer KM, Bhella PS, Carrick-Ranson G, Levine BD. Hemodynamic responses to rapid saline loading: The impact of age, sex, and heart failure. Circulation 2013;127:55-62.
76. Fox BD, Shimony A, Langleben D, Hirsch A, Rudski L, Schlesinger R, Eisenberg MJ, Joyal D, Hudson M, Boutet K, Serban A, Masetto A, Baron M. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur Respir J 2013;42:1083-1091.
77. Robbins IM, Hemnes AR, Pugh ME, Brittain EL, Zhao DX, Piana RN, Fong PP, Newman JH. High prevalence of occult pulmonary venous hypertension revealed by fluid challenge in pulmonary hypertension. Circ Heart Fail 2014;7:116-122.
78. Borlaug BA, Nishimura RA, Sorajja P, Lam CS, Redfield MM. Exercise hemodynamics enhance diagnosis of early heart failure with preserved ejection fraction. Circ Heart Fail 2010;3:588-595.
79. Hager WD, Collins I, Tate JP, Azrin M, Foley R, Lakshminarayanan S, Rothfield NF. Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients. Clin Respir J 2013;7: 227-236.
80. Halpern SD, Taichman DB. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular enddiastolic pressure. Chest 2009;136:37-43.
81. Naeije R, Vachiery JL, Yerly P, Vanderpool R. The transpulmonary pressure gradient for the diagnosis of pulmonary vascular disease. Eur Respir J 2013;41:217-223.
82. Provencher S, Herve P, Sitbon O, Humbert M, Simonneau G, Chemla D. Changes in exercise haemodynamics during treatment in pulmonary arterial hypertension. Eur Respir J 2008;32:393-398.
83. Tedford RJ, Beaty CA, Mathai SC, Kolb TM, Damico R, Hassoun PM, Leary PJ, Kass DA, Shah AS. Prognostic value of the pre-transplant diastolic pulmonary artery pressure-to-pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension. J Heart Lung Transplant 2014;33: 289-297.
84. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension [see comments]. N Engl J Med 1992;327:76-81.
85. Sitbon O, Humbert M, Jaïs X, Ioos V, Hamid AM, Provencher S, Garcia G, Parent F, Herve P, Simonneau G. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005;111:3105-3111.
86. Barst R, McGoon M, Torbicki A, Sitbon O, Krowka MJ, Olschewski A, Gaine S. Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 2004;43(Suppl 1):S40-S47.
87. Morgan JM, McCormack DG, Griffiths MJ, Morgan CJ, Barnes PJ, Evans TW. Adenosine as a vasodilator in primary pulmonary hypertension [see comments]. Circulation 1991;84:1145-1149.
88. Nootens M, Schrader B, Kaufmann E, Vestal R, LongW, Rich S. Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension. Chest 1995;107:54-57.
89. Hoeper MM, Olschewski H, Ghofrani HA, Wilkens H, Winkler J, Borst MM, Niedermeyer J, Fabel H, SeegerW. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. J Am Coll Cardiol 2000;35:176-182.
90. Opitz CF, Wensel R, Bettmann M, Schaffarczyk R, Linscheid M, Hetzer R, Ewert R. Assessment of the vasodilator response in primary pulmonary hypertension. Comparing prostacyclin and iloprost administered by either infusion or inhalation. Eur Heart J 2003;24:356-365.
91. Kim NH, Delcroix M, Jenkins DP, Channick R, Dartevelle P, Jansa P, Lang I, Madani MM, Ogino H, Pengo V, Mayer E. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol 2013;62:D92-D99.
92. Montani D, Price LC, Dorfmuller P, Achouh L, Jaïs X, Yaici A, Sitbon O, Musset D, Simonneau G, Humbert M. Pulmonary veno-occlusive disease. Eur Respir J 2009; 33:189-200.
93. He J, Fang W, Lv B, He JG, Xiong CM, Liu ZH, He ZX. Diagnosis of chronic thromboembolic pulmonary hypertension: comparison of ventilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography. Nucl Med Commun 2012;33:459-463.
94. Cottin V, Le Pavec J, Prevot G, Mal H, Humbert M, Simonneau G, Cordier JF. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur Respir J 2010;35:105-111.
95. Taichman DB, McGoon MD, Harhay MO, Archer-Chicko C, Sager JS, Murugappan M, Chakinali MM, Palevsky HI, Gallop R. Wide variation in clinicians' assessment of New York Heart Association/World Health Organization functional class in patients with pulmonary arterial hypertension. Mayo Clin Proc 2009;84:586-592.
96. 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;40: 780-788.
97. Nickel N, Golpon H, Greer M, Knudsen L, Olsson K, Westerkamp V, Welte T, Hoeper MM. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2012;39:589-596.
98. Barst RJ, Chung L, Zamanian RT, Turner M, McGoon MD. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest 2013;144:160-168.
99. Benza RL, Miller DP, Gomberg-Maitland M, Frantz RP, Foreman AJ, Coffey CS, Frost A, Barst RJ, Badesch DB, Elliott CG, Liou TG, McGoon MD. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010;122:164-172.
100. McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galiè N, Rainisio M, Simonneau G, Rubin LJ. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005;25:244-249.
101. Sachdev A, Villarraga HR, Frantz RP, McGoon MD, Hsiao JF, Maalouf JF, Ammash NM, McCully RB, Miller FA, Pellikka PA, Oh JK, Kane GC. Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension. Chest 2011;139:1299-1309.
102. Raymond RJ, Hinderliter AL, Willis PW, Ralph D, Caldwell EJ, Williams W, Ettinger NA, Hill NS, Summer WR, de Boisblanc B, Schwartz T, Koch G, Clayton LM, Jobsis MM, Crow JW, LongW. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J Am Coll Cardiol 2002;39: 1214-1219.
103. Bustamante-Labarta M, Perrone S, De La Fuente RL, Stutzbach P, De La Hoz RP, Torino A, Favaloro R. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J Am Soc Echocardiogr 2002;15:1160-1164.
104. Forfia PR, Fisher MR, Mathai SC, Housten-Harris T, Hemnes AR, Borlaug BA, Chamera E, Corretti MC, Champion HC, Abraham TP, Girgis RE, Hassoun PM. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med 2006;174:1034-1041.
105. Thenappan T, Shah SJ, Rich S, Tian L, Archer SL, Gomberg-Maitland M. Survival in pulmonary arterial hypertension: A reappraisal of the NIH risk stratification equation. Eur Respir J 2010;35:1079-1087.
106. Wensel R, Opitz CF, Anker SD, Winkler J, Hoffken G, Kleber FX, Sharma R, Hummel M, Hetzer R, Ewert R. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation 2002;106:319-324.
107. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: The impact of epoprostenol therapy. Circulation 2002;106:1477-1482.
108. Fine NM, Chen L, Bastiansen PM, Frantz RP, Pellikka PA, Oh JK, Kane GC. Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension. Circ Cardiovasc Imaging 2013;6: 711-721.
109. Shimada YJ, Shiota M, Siegel RJ, Shiota T. Accuracy of right ventricular volumes and function determined by three-dimensional echocardiography in comparison with magnetic resonance imaging: A meta-analysis study. J Am Soc Echocardiogr 2010;23: 943-953.
110. Smith BC, Dobson G, Dawson D, Charalampopoulos A, Grapsa J, Nihoyannopoulos P. Three-dimensional speckle tracking of the right ventricle: Toward optimal quantification of right ventricular dysfunction in pulmonary hypertension. J Am Coll Cardiol 2014;64:41-51.
111. Grunig E, Tiede H, Enyimayew EO, Ehlken N, Seyfarth HJ, Bossone E, D'Andrea A, Naeije R, Olschewski H, Ulrich S, Nagel C, Halank M, Fischer C. Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension. Circulation 2013;128:2005-2015.
112. Swift AJ, Rajaram S, Marshall H, Condliffe R, Capener D, Hill C, Davies C, Hurdman J, Elliot CA, Wild JM, Kiely DG. Black blood MRI has diagnostic and prognostic value in the assessment of patients with pulmonary hypertension. Eur Radiol 2012;22:695-702.
113. Swift AJ, Rajaram S, Campbell MJ, Hurdman J, Thomas S, Capener D, Elliot C, Condliffe R, Wild JM, Kiely DG. Prognostic value of cardiovascular magnetic resonance imaging measurements corrected for age and sex in idiopathic pulmonary arterial hypertension. Circ Cardiovasc Imaging 2014;7:100-106.
114. Sitbon O, McLaughlin VV, Badesch DB, Barst RJ, Black C, Galiè N, Humbert M, Rainisio M, Rubin LJ, Simonneau G. 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; 60:1025-1030.
115. Rich JD, Archer SL, Rich S. Noninvasive cardiac output measurements in patients with pulmonary hypertension. Eur Respir J 2013;42:125-133.
116. Savarese G, Paolillo S, Costanzo P, D'Amore C, Cecere M, Losco T, Musella F, Gargiulo P, Marciano C, Perrone-Filardi P. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension: A meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012;60:1192-1201.
117. Gabler NB, French B, Strom BL, Palevsky HI, Taichman DB, Kawut SM, Halpern SD. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 2012;126:349-356.
118. Fritz JS, Blair C, Oudiz RJ, Dufton C, Olschewski H, Despain D, Gillies H, Kawut SM. Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest 2013;143: 315-323.
119. Paciocco G, Martinez F, 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;17:647-652.
120. Provencher S, Chemla D, Herve P, Sitbon O, Humbert M, Simonneau G. Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur Respir J 2006;27:114-120.
121. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation 2001;104:429-435.
122. Wensel R, Francis DP, Meyer FJ, Opitz CF, Bruch L, Halank M, Winkler J, Seyfarth HJ, Glaser S, Blumberg F, Obst A, Dandel M, Hetzer R, Ewert R. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int J Cardiol 2013;167:1193-1198.
123. Blumberg FC, Arzt M, Lange T, Schroll S, Pfeifer M, Wensel R. Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension. Eur J Heart Fail 2013;15:771-775.
124. Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, Johansson B, Bouzas B, Mullen MJ, Poole-Wilson PA, Francis DP, Gatzoulis MA. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005;112:828-835.
125. Arena R, Lavie CJ, Milani RV, Myers J, Guazzi M. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: An evidence-based review. J Heart Lung Transplant 2010;29:159-173.
126. Pullamsetti S, Kiss L, Ghofrani HA, Voswinckel R, Haredza P, Klepetko W, Aigner C, Fink L, Muyal JP, Weissmann N, Grimminger F, Seeger W, Schermuly RT. Increased levels and reduced catabolism of asymmetric and symmetric dimethylarginines in pulmonary hypertension. FASEB J 2005;19: 1175-1177.
127. Kielstein JT, Bode-Boger SM, Hesse G, Martens-Lobenhoffer J, Takacs A, Fliser D, Hoeper MM. Asymmetrical dimethylarginine in idiopathic pulmonary arterial hypertension. Arterioscler Thromb Vasc Biol 2005;25:1414-1418.
128. Kielstein JT, Impraim B, Simmel S, Bode-Boger SM, Tsikas D, Frolich JC, Hoeper MM, Haller H, Fliser D. Cardiovascular effects of systemic nitric oxide synthase inhibition with asymmetrical dimethylarginine in humans. Circulation 2004;109:172-177.
129. Kawut SM, Horn EM, Berekashvili KK, Widlitz AC, Rosenzweig EB, Barst RJ. von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension. Chest 2005;128:2355-2362.
130. Kumpers P, Nickel N, Lukasz A, Golpon H, Westerkamp V, Olsson KM, Jonigk D, Maegel L, Bockmeyer CL, David S, Hoeper MM. Circulating angiopoietins in idiopathic pulmonary arterial hypertension. Eur Heart J 2010;31:2291-2300.
131. Rubens C, Ewert R, Halank M, Wensel R, Orzechowski HD, Schultheiss HP, Hoeffken G. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 2001;120: 1562-1569.
132. Quarck R, Nawrot T, Meyns B, Delcroix M. C-reactive protein: A new predictor of adverse outcome in pulmonary arterial hypertension. J Am Coll Cardiol 2009;53: 1211-1218.
133. Balabanian K, Foussat A, Dorfmuller P, Durand-Gasselin I, Capel F, Bouchet-Delbos L, Portier A, Marfaing-Koka A, Krzysiek R, Rimaniol AC, Simonneau G, Emilie D, Humbert M. CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am J Respir Crit Care Med 2002;165:1419-1425.
134. Dorfmuller P, Zarka V, Durand-Gasselin I, Monti G, Balabanian K, Garcia G, Capron F, Coulomb-Lhermine A, Marfaing-Koka A, Simonneau G, Emilie D, Humbert M. Chemokine RANTES in severe pulmonary arterial hypertension. Am J Respir Crit Care Med 2002;165:534-539.
135. Humbert M, Monti G, Brenot F, Sitbon O, Portier A, Grangeot-Keros L, Duroux P, Galanaud P, Simonneau G, Emilie D. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med 1995;151:1628-1631.
136. Nagaya N, Nishikimi T, Uematsu M, Satoh T, Kyotani S, Sakamaki F, Kakishita M, Fukushima K, Okano Y, Nakanishi N, Miyatake K, Kangawa K. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 2000;102:865-870.
137. Leuchte HH, El NM, Tuerpe JC, Hartmann B, Baumgartner RA, Vogeser M, Muehling O, Behr J. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest 2007;131: 402-409.
138. Fijalkowska A, Kurzyna M, Torbicki A, Szewczyk G, Florczyk M, Pruszczyk P, Szturmowicz M. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest 2006;129:1313-1321.
139. Torbicki A, Kurzyna M, Kuca P, Fijalkowska A, Sikora J, Florczyk M, Pruszczyk P, Burakowski J, Wawrzynska L. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation 2003;108:844-848.
140. Nickel N, Kempf T, Tapken H, Tongers J, Laenger F, Lehmann U, Golpon H, Olsson K, Wilkins MR, Gibbs JS, Hoeper MM, Wollert KC. Growth differentiation factor-15 in idiopathic pulmonary arterial hypertension. Am J Respir Crit Care Med 2008;178:534-541.
141. Nagaya N, Uematsu M, Satoh T, Kyotani S, Sakamaki F, Nakanishi N, Yamagishi M, Kunieda T, Miyatake K. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 1999;160: 487-492.
142. Lorenzen JM, Nickel N, Kramer R, Golpon H, Westerkamp V, Olsson KM, Haller H, Hoeper MM. Osteopontin in patients with idiopathic pulmonary hypertension. Chest 2011;139:1010-1017.
143. Warwick G, Thomas PS, Yates DH. Biomarkers in pulmonary hypertension. Eur Respir J 2008;32:503-512.
144. Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005;26:858-863.
145. McLaughlin VV, Gaine SP, Howard LS, Leuchte HH, Mathier MA, Mehta S, Palazzini M, Park MH, Tapson VF, Sitbon O. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013;62(Suppl):D73-D81.
146. Galiè N, Manes A, Negro L, Palazzini M, Bacchi Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009;30:394-403.
147. Bai Y, Sun L, Hu S, Wei Y. Combination therapy in pulmonary arterial hypertension: A meta-analysis. Cardiology 2011;120:157-165.
148. Galiè N, Simonneau G. The Fifth World Symposium on Pulmonary Arterial Hypertension. J Am Coll Cardiol 2013;62(Suppl):D1-D3.
149. Galiè N, Corris P, Frost A, Girgis R, Granton J, Jing ZC, KlepetkoW, McGoon M, McLaughlin VV, Preston RJ, Rubin LJ, Sandoval J, Seeger W, Keogh AM. Updated treatment algorithm of pulmonary hypertension. J Am Coll Cardiol 2013; 62(Suppl):D60-D72.
150. Löwe B, Gräfe K, Ufer C, Kroenke K, Grünig E, HerzogW, BorstMM. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med 2004;66: 831-836.
151. Galiè N, Hoeper M, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Klepetko W, Joendeau G, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G. Guidelines on diagnosis and treatment of pulmonary hypertension: The Task Force on Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology and of the European Respiratory Society. Eur Heart J 2009;30:2493-2537.
152. 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, Grunig E. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 2006;114:1482-1489.
153. de Man FS, Handoko ML, Groepenhoff H, van 't Hul AJ, Abbink J, Koppers RJH, Grotjohan HP, Twisk JWR, Bogaard HJ, Boonstra A, Postmus PE, Westerhof N, Van Der Laarse WJ, Vonk-Noordegraaf A. Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2009;34:669-675.
154. Grunig E, Ehlken N, Ghofrani A, Staehler G, Meyer FJ, Juenger J, Opitz CF, Klose H, Wilkens H, Rosenkranz S, Olschewski H, Halank M. Effect of exercise and respiratory training on clinical progression and survival in patients with severe chronic pulmonary hypertension. Respiration 2011;81:394-401.
155. Grunig E, Maier F, Ehlken N, Fischer C, Lichtblau M, Blank N, Fiehn C, Stockl F, Prange F, Staehler G, Reichenberger F, Tiede H, Halank M, Seyfarth HJ, Wagner S, Nagel C. Exercise training in pulmonary arterial hypertension associated with connective tissue diseases. Arthritis Res Ther 2012;14:R148.
156. Grunig E, Lichtblau M, Ehlken N, Ghofrani HA, Reichenberger F, Staehler G, Halank M, Fischer C, Seyfarth HJ, Klose H, Meyer A, Sorichter S, Wilkens H, Rosenkranz S, Opitz C, Leuchte H, Karger G, Speich R, Nagel C. Safety and efficacy of exercise training in various forms of pulmonary hypertension. Eur Respir J 2012;40:84-92.
157. Becker-Grunig T, Klose H, Ehlken N, Lichtblau M, Nagel C, Fischer C, Gorenflo M, Tiede H, Schranz D, Hager A, Kaemmerer H, Miera O, Ulrich S, Speich R, Uiker S, Grunig E. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol 2013;168:375-381.
158. Weinstein AA, Chin LMK, Keyser RE, Kennedy M, Nathan SD, Woolstenhulme JG, Connors G, Chan L. Effect of aerobic exercise training on fatigue and physical activity in patients with pulmonary arterial hypertension. Respir Med 2013;107:778-784.
159. Chan L, Chin LM, Kennedy M, Woolstenhulme JG, Nathan SD, Weinstein AA, Connors G, Weir NA, Drinkard B, Lamberti J, Keyser RE. Benefits of intensive treadmill exercise training on cardiorespiratory function and quality of life in patients with pulmonary hypertension. Chest 2013;143:333-343.
160. Jaïs X, Olsson KM, Barbera JA, Blanco I, Torbicki A, Peacock A, Vizza CD, Macdonald P, Humbert M, Hoeper MM. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 2012;40:881-885.
161. Duarte AG, Thomas S, Safdar Z, Torres F, Pacheco LD, Feldman J, deBoisblanc B. Management of pulmonary arterial hypertension during pregnancy: A retrospective, multicenter experience. Chest 2013;143:1330-1336.
162. Thorne S, Nelson-Piercy C, MacGregor AJ, Gibbs S, Crowhurst J, Panay N, Rosenthal E, Walker F, Williams D, de Swiet M, Guillebaud J. Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006;32:75-81.
163. Bendayan D, Hod M, Oron G, Sagie A, Eidelman L, Shitrit D, Kramer MR. Pregnancy outcome in patients with pulmonary arterial hypertension receiving prostacyclin therapy. Obstet Gynecol 2005;106:1206-1210.
164. Bonnin M, Mercier FJ, Sitbon O, Jaïs 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;102:1133-1137.
165. Meyer S, McLaughlin VV, Seyfarth HJ, Bull TM, Vizza CD, Gomberg-Maitland M, Preston IR, Barberà JA, Hassoun PM, Halank M, Jaïs X, Nickel N, Hoeper MM, Humbert M. Outcomes of noncardiac, nonobstetric surgery in patients with PAH: An international prospective survey. Eur Respir J 2013;41:1302-1307.
166. Olofsson C, Bremme K, Forssell G, Ohqvist G. Cesarean section under epidural ropivacaine 0. 75% in a parturient with severe pulmonary hypertension. Acta Anaesthesiol Scand 2001;45:258-260.
167. Raines DE, Liberthson RR, Murray JR. Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger's physiology. J Clin Anesth 1996;8:341-347.
168. Guillevin L, Armstrong I, Aldrighetti R, Howard LS, Ryftenius H, Fischer A, Lombardi S, Studer S, Ferrari P. Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives. Eur Respir Rev 2013;22:535-542.
169. Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis 1985;131: 493-498.
170. Frydman N, Steffann J, Girerd B, Frydman R, Munnich A, Simonneau G, Humbert M. Pre-implantation genetic diagnosis in pulmonary arterial hypertension due to BMPR2 mutation. Eur Respir J 2012;39:1534-1535.
171. Fuster V, Steele PM, Edwards WD, Gersh BJ, McGoon MD, Frye RL. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984;70:580-587.
172. Herve P, Humbert M, Sitbon O, Parent F, Nunes H, Legal C, Garcia G, Simonneau G. Pathobiology of pulmonary hypertension: The role of platelets and thrombosis. Clin Chest Med 2001;22:451-458.
173. Hoeper MM, Sosada M, Fabel H. Plasma coagulation profiles in patients with severe primary pulmonary hypertension. Eur Respir J 1998;12:1446-1449.
174. Huber K, Beckmann R, Frank H, Kneussl M, Mlczoch J, Binder BR. Fibrinogen, t-PA, and PAI-1 plasma levels in patients with pulmonary hypertension. Am J Respir Crit Care Med 1994;150:929-933.
175. Olsson KM, Delcroix M, Ghofrani HA, Tiede H, Huscher D, Speich R, Grünig E, Staehler G, Rosenkranz S, Halank M, Held M, Lange TJ, Behr J, Klose H, Claussen M, Ewert R, Opitz CF, Vizza CD, Scelsi L, Vonk-Noordegraaf A, Kaemmerer H, Gibbs JS, Coghlan G, Pepke-Zaba J, Schulz U, Gorenflo M, Pittrow D, Hoeper MM. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation 2014;129: 57-65.
176. Galiè N, Delcroix M, Ghofrani A, Jansa P, Minai OA, Perchenet L, Rubin LJ, Sastry BKS, Torbicki A, Simonneau G. Anticoagulant therapy does not influence long-term outcomes in patients with pulmonary arterial hypertension (PAH): insights from the randomised controlled SERAPHIN trial of macitentan. Eur Heart J 2014;35:10.
177. Preston RJ, Roberts KE, Miller DP, Hill NS, Farber HW. Effect of warfarin treatment on survival of patients with pulmonary arterial hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL). Am J Respir Crit Care Med 2014;189:A2464.
178. Cohn JN. Optimal diuretic therapy for heart failure. Am J Med 2001;111:577.
179. Sandoval J, Aguirre JS, Pulido T, Martinez-Guerra ML, Santos E, Alvarado P, Rosas M, Bautista E. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med 2001;164:1682-1687.
180. Rich S, Seidlitz M, Dodin E, Osimani D, Judd D, Genthner D, McLaughlin V, Francis G. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest 1998;114:787-792.
181. Ruiter G, Lankhorst S, Boonstra A, Postmus PE, Zweegman S, Westerhof N, Van Der Laarse WJ, Vonk-Noordegraaf A. Iron deficiency is common in idiopathic pulmonary arterial hypertension. Eur Respir J 2011;37:1386-1391.
182. Ruiter G, Lanser IJ, de Man FS, Van Der Laarse WJ, Wharton J, Wilkins MR, Howard LS, Vonk-Noordegraaf A, Voskuyl AE. Iron deficiency in systemic sclerosis patients with and without pulmonary hypertension. Rheumatology (Oxford) 2014;53:285-292.
183. Broberg CS, Bax BE, Okonko DO, RamplingMW, Bayne S, Harries C, Davidson SJ, Uebing A, Khan AA, Thein S, Gibbs JS, Burman J, Gatzoulis MA. Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol 2006;48:356-365.
184. Rhodes CJ, Howard LS, Busbridge M, Ashby D, Kondili E, Gibbs JS, Wharton J, Wilkins MR. Iron deficiency and raised hepcidin in idiopathic pulmonary arterial hypertension clinical prevalence, outcomes, and mechanistic insights. J Am Coll Cardiol 2011;58:300-309.
185. Van De Bruaene A, Delcroix M, Pasquet A, De BJ, De PM, Naeije R, Vachiery JL, Paelinck B, Morissens M, BudtsW. Iron deficiency is associated with adverse outcome in Eisenmenger patients. Eur Heart J 2011;32:2790-2799.
186. Viethen T, Gerhardt F, Dumitrescu D, Knoop-Busch S, ten Freyhaus H, Rudolph TK, Baldus S, Rosenkranz S. Ferric carboxymaltose improves exercise capacity and quality of life in patients with pulmonary arterial hypertension and iron deficiency: A pilot study. Int J Cardiol 2014;175:233-239.
187. Galié 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.
188. 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;62: 1088-1093.
189. Montani D, Savale L, Natali D, Jaïs X, Herve P, Garcia G, Humbert M, Simonneau G, Sitbon O. Long-term response to calcium-channel blockers in nonidiopathic pulmonary arterial hypertension. Eur Heart J 2010;31:1898-1907.
190. 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;328:1732-1739.
191. Stewart DJ, Levy RD, Cernacek P, Langleben D. Increased plasma endothelin-1 in pulmonary hypertension: marker or mediator of disease? Ann Intern Med 1991; 114:464-469.
192. Galié N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res 2004;61:227-237.
193. Galié N, Badesch BD, Oudiz R, Simonneau G, McGoon M, Keogh A, Frost A, Zwicke D, Naeije R, Shapiro RS, Olschewski H, Rubin L. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:529-535.
194. Galié N, Olschewski H, Oudiz RJ, Torres F, Frost A, Ghofrani HA, Badesch DB, McGoon MD, McLaughlin VV, Roecker EB, Gerber MJ, Dufton C, Wiens BL, Rubin LJ. Ambrisentan for the treatment of pulmonary arterial hypertension. Results of the ambrisentan in pulmonary arterial hypertension, randomized, doubleblind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008;117:3010-3019.
195. McGoon M, Frost A, Oudiz R, Badesch BD, Galiè N, Olschewski H, McLaughlin VV, Gerber MJ, Dufton C, Despain DJ, Rubin LJ. Ambrisentan therapy in patients with pulmonary arterial hypertension who discontinued bosentan or sitaxsentan due to liver function test abnormalities. Chest 2009;135:122-129.
196. Channick RN, Simonneau G, Sitbon O, Robbins IM, Frost A, Tapson VF, Badesch DB, Roux S, Rainisio M, Bodin F, Rubin LJ. Effects of the dual endothelinreceptor antagonist bosentan in patients with pulmonary hypertension: A randomised placebo-controlled study. Lancet 2001;358:1119-1123.
197. Rubin LJ, Badesch DB, Barst RJ, Galiè 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;346:896-903.
198. Humbert M, Barst RJ, Robbins IM, Channick RN, Galiè N, Boonstra A, Rubin LJ, Horn EM, Manes A, Simonneau G. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004;24:353-359.
199. Galiè N, Rubin LJ, Hoeper M, Jansa P, Al-Hiti H, Meyer GMB, Chiossi E, Kusic-Pajic A, Simonneau G. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): A double-blind, randomised controlled trial. Lancet 2008;371:2093-2100.
200. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, Chiossi E, Landzberg M. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006;114: 48-54.
201. Pulido T, Adzerikho I, Channick RN, Delcroix M, Galiè N, Ghofrani HA, Jansa P, Jing ZC, Le Brun FO, Mehta S, Mittelholzer CM, Perchenet L, Sastry BKS, Sitbon O, Souza R, Torbicki A, Zeng X, Rubin LJ, Simonneau G. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369:809-818.
202. Wharton J, Strange JW, Moller GMO, Growcott EJ, Ren X, Franklyn AP, Phillips SC, Wilkins MR. Antiproliferative effects of phosphodiesterase type 5 inhibition in human pulmonary artery cells. Am J Respir Crit Care Med 2005;172: 105-113.
203. Tantini B, Manes A, Fiumana E, Pignatti C, Guarnieri G, Zannoli R, Branzi A, Galiè N. Antiproliferative effect of sildenafil on human pulmonary artery smooth muscle cells. Basic Res Cardiol 2005;100:131-138.
204. Ghofrani HA, Voswinckel R, Reichenberger F, Olschewski H, Haredza P, Karadas B, Schermuly RT, Weissmann N, Seeger W, Grimminger F. Differences in hemodynamic and oxygenation responses to three different phosphodiesterase-5 inhibitors in patients with pulmonary arterial hypertension: A randomized prospective study. J Am Coll Cardiol 2004;44:1488-1496.
205. Galiè 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, the Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353: 2148-2157.
206. Sastry BKS, 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;43:1149-1153.
207. Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Søndergaard L. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: A randomized, placebo-controlled, double-blinded trial. Eur Heart J 2010;31:1124-1131.
208. Singh T, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebocontrolled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J 2006;151:851. e1-851 e5.
209. Simonneau G, Rubin L, Galiè N, Barst RJ, Fleming T, Frost A, Engel PJ, Kramer MR, Burgess G, Collings L, Cossons N, Sitbon O, Badesch BD. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension. Ann Intern Med 2008;149:521-530.
210. Vachiery JL, Huez S, Gillies H, Layton G, Hayashi N, Gao X, Naeije R. Safety, tolerability and pharmacokinetics of an intravenous bolus of sildenafil in patients with pulmonary arterial hypertension. Br J Clin Pharmacol 2011;71:289-292.
211. Galiè N, Brundage BH, Ghofrani HA, Oudiz RJ, Simonneau G, Safdar Z, Shapiro S, White RJ, Chan M, Beardsworth A, Frumkin L, Barst RJ. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894-2903.
212. Jing ZC, Yu ZX, Shen JY, Wu BX, Xu KF, Zhu XY, Pan L, Zhang ZL, Liu XQ, Zhang YS, Jiang X, Galiè N. Vardenafil in pulmonary arterial hypertension: A randomized, double-blind, placebo-controlled study. Am J Respir Crit Care Med 2011; 183:1723-1729.
213. Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995;333:214-221.
214. Ghofrani HA, Galiè N, Grimminger F, Grunig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, Neuser D, Rubin LJ. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013;369:330-340.
215. Galiè N, Muller K, Scalise AV, Grunig E. PATENT PLUS: A blinded, randomised and extension study of riociguat plus sildenafil in PAH. Eur Respir J 2015;45: 1314-1322.
216. 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: 890-896.
217. Galiè N, Manes A, Branzi A. Prostanoids for pulmonary arterial hypertension. Am J Respir Med 2003;2:123-137.
218. Galiè N, Humbert M, Vachiery JL, Vizza CD, Kneussl M, Manes A, Sitbon O, Torbicki A, Delcroix M, Naeije R, Hoeper M, Chaouat A, Morand S, Besse B, Simonneau G. Effects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: A randomised, double-blind placebocontrolled trial. J Am Coll Cardiol 2002;39:1496-1502.
219. Barst RJ, McGoon M, McLaughlin VV, Tapson V, Rich S, Rubin L, Wasserman K, Oudiz R, Shapiro S, Robbins I, Channick R, Badesch BD, Rayburn BK, Flinchbaugh R, Sigman J, Arneson K, Jeffs R. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003;41:2119-2125.
220. Rubin LJ, Mendoza J, Hood M, McGoon M, Barst R, Williams WB, Diehl JH, Crow J, Long W. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990;112:485-491.
221. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, Groves BM, Tapson VF, Bourge RC, Brundage BH, Koerner SK, Langleben D, Keller C, Murali S, Uretsky BF, Clayton LM, Jöbsis MM, Blackburn SD, Shortino D, Crow JW. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension [see comments]. N Engl J Med 1996;334:296-302.
222. 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 [see comments]. Ann Intern Med 2000;132:425-434.
223. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99: 1858-1865.
224. 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;30:641-648.
225. 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;167:1433-1439.
226. Cabrol S, Souza R, Jaïs X, Fadel E, Ali RHS, Humbert M, Dartevelle P, Simonneau G, Sitbon O. Intravenous epoprostenol in inoperable chronic thromboembolic pulmonary hypertension. J Heart Lung Transplant 2007;26: 357-362.
227. Doran AK, Ivy DD, Barst RJ, Hill N, Murali S, Benza RL. Guidelines for the prevention of central venous catheter-related blood stream infections with prostanoid therapy for pulmonary arterial hypertension. Int J Clin Pract Suppl 2008;160:5-9.
228. Sitbon O, Delcroix M, Bergot E, Boonstra AB, Granton J, Langleben D, Subás PE, Galiè N, Pfister T, Lemarié JC, Simonneau G. EPITOME-2: An open-label study assessing the transition to a new formulation of intravenous epoprostenol in patients with pulmonary arterial hypertension. Am Heart J 2014;167:210-217.
229. Olschewski H, Simonneau G, Galiè N, Higenbottam T, Naeije R, Rubin LJ, Nikkho S, Sitbon O, Speich R, Hoeper M, Behr J, Winkler J, Seeger W. Inhaled iloprost in severe pulmonary hypertension. N Engl J Med 2002;347:322-329.
230. McLaughlin VV, Oudiz RJ, Frost A, Tapson VF, Murali S, Channick RN, Badesch DB, Barst RJ, Hsu HH, Rubin LJ. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006;174:1257-1263.
231. Hoeper M, Leuchte H, Halank M, Wilkens H, Meyer FJ, Seyfarth HJ, Wensel R, Ripken F, Bremer H, Kluge S, Hoeffken G, Behr J. Combining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2006;4:691-694.
232. 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;80:151-155.
233. Simonneau G, Barst RJ, Galiè 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;165:800-804.
234. Hiremath J, Thanikachalam S, Parikh K, Shanmugasundaram S, Bangera S, Shapiro L, Pott GB, Vnencak-Jones CL, Arneson C, Wade M, White RJ. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: A placebo-controlled trial. J Heart Lung Transplant 2010;29:137-149.
235. 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; 129:683-688.
236. Sitbon O, Manes A, Jaïs X, Pallazini M, Humbert M, Presotto L, Paillette L, Zaccardelli D, Davis G, Jeffs R, Simonneau G, Galiè N. Rapid switch from intravenous epoprostenol to intravenous treprostinil in patients with pulmonary arterial hypertension. J Cardiovasc Pharmacol 2007;49:1-5.
237. McLaughlin V, Rubin L, Benza RL, Channick R, Vosswinkel R, Tapson V, Robbins I, Olschewski H, SeegerW. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: A randomized controlled clinical trial. J Am Coll Cardiol 2010;55:1915-1922.
238. Tapson VF, Torres F, Kermeen F, Keogh AM, Allen RP, Frantz RP, Badesch DB, Frost AE, Shapiro SM, Laliberte K, Sigman J, Arneson C, Galiè N. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): A randomized controlled trial. Chest 2012;142: 1383-1390.
239. Tapson VF, Jing ZC, Xu KF, Pan L, Feldman J, Kiely DG, Kotlyar E, McSwain CS, Laliberte K, Arneson C. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): A randomized controlled trial. Chest 2013;144:952-958.
240. Jing ZC, Parikh K, Pulido T, Jerjes-Sanchez C, White RJ, Allen R, Torbicki A, Xu KF, Yehle D, Laliberte K, Arneson C, Rubin LJ. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: A randomized, controlled trial. Circulation 2013;127:624-633.
241. Simonneau G, Torbicki A, Hoeper MM, Delcroix M, Karlocai K, Galiè N, Degano B, Bonderman D, Kurzyna M, Efficace M, Giorgino R, Lang IM. Selexipag, an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012;40:874-880.
242. Chen SL, Zhang FF, Xu J, Xie DJ, Zhou L, Nguyen T, StoneGW. Pulmonary artery denervation to treat pulmonary arterial hypertension: The single-center, prospective, first-in-man PADN-1 study (first-in-man pulmonary artery denervation for treatment of pulmonary artery hypertension). J Am Coll Cardiol 2013;62: 1092-1100.
243. Galiè N, Manes A. New treatment strategies for pulmonary arterial hypertension: hopes or hypes? J Am Coll Cardiol 2013;62:1101-1102.
244. Galiè N, Palazzini M, Manes A. Pulmonary arterial hypertension: from the kingdom of the near-dead to multiple clinical trial meta-analyses. Eur Heart J 2010;31: 2080-2086.
245. Kemp K, Savale L, O'Callaghan DS, Jaïs X, Montani D, Humbert M, Simonneau G, Sitbon O. Usefulness of first-line combination therapy with epoprostenol and bosentan in pulmonary arterial hypertension: An observational study. J Heart Lung Transplant 2012;31:150-158.
246. Sitbon O, Jaïs X, Savale L, Cottin V, Bergot E, Macari EA, Bouvaist H, Dauphin C, Picard F, Bulifon S, Montani D, Humbert M, Simonneau G. Upfront triple combination therapy in pulmonary arterial hypertension: A pilot study. Eur Respir J 2014; 43:1691-1697.
247. Galiè N, Barbera JA, Frost A, Ghofrani A, Hoeper M, Mc Laughlin VV, Peacock A, Simonneau G, Vachiery JL, Grunig E, Oudiz RG, Vonk-Nordegraaf A, White J, Blair C, Gillies HC, Miller L, Harris JHN, Langley J, Rubin LJ. Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension. New Engl J Med 2015; 379(9):834-844.
248. Sitbon O, Channick R, Kelly C, Frey A, Gaine S, Galie N, Ghofrani A, Hoeper M, Lang I, Preiss R, Rubin L, Di Scala L, Tapson V, Adzerikho I, Liu J, Moiseeva O, Zeng X, Simonneau G, McLaughlin V. Selexipag for the Treatment of Pulmonary Arterial Hypertension. New Engl J Med 2015;373:2522-2533.
249. Badesch BD, Feldman J, Keogh A, Mathier MA, Oudiz R, Shapiro S, Farber HW, McGoon M, Frost A, Allard M, Despain D, Dufton C, Rubin LJ. ARIES-3: Ambrisentan therapy in a diverse population of patients with pulmonary hypertension. Cardiovasc Ther 2012;30:93-99.
250. Provencher S, Sitbon O, Humbert M, Cabrol S, Jaïs X, Simonneau G. Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J 2006;27:589-595.
251. McLaughlin V, Channick RN, Ghofrani HA, Lemarié JC, Naeije R, Packer M, Souza R, Tapson VF, Tolson J, Al Hit Hi, Meyer G, Hoeper M. M. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J 2015;46:405-413.
252. Dardi F, Manes A, Palazzini M, Bachetti C, Mazzanti G, Rinaldi A, Albini A, Gotti E, Monti E, Bacchi Reggiani ML, Galiè N. Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights. Eur Respir J 2015;46:414-421.
253. Sandoval J, Gaspar J, Pulido T, Bautista E, Martnez Guerra ML, Zeballos M, Palomar A, Gómez A. Graded balloon dilation atrial septostomy in severe primary pulmonary hypertension. A therapeutic alternative for patients nonresponsive to vasodilator treatment. J Am Coll Cardiol 1998;32:297-304.
254. Kurzyna M, Dabrowski M, Bielecki D, Fijalkowska A, Pruszczyk P, Opolski G, Burakowski J, Florczyk M, Tomkowski WZ, Wawrzynska L, Szturmowicz M, Torbicki A. Atrial septostomy in treatment of end-stage right heart failure in Patients with pulmonary hypertension. Chest 2007;131:977-983.
255. Althoff TF, Knebel F, Panda A, McArdle J, Gliech V, Franke I, Witt C, Baumann G, Borges AC. Long-term follow-up of a fenestrated Amplatzer atrial septal occluder in pulmonary arterial hypertension. Chest 2008;133:283-285.
256. Keogh A, Benza RL, Corris P, Dartevelle P, Frost A, Kim NH, Lang I, Pepke-Zaba J, Sandoval J, Mayer E. Interventional and surgical modalities of treatment in pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(Suppl):S67-S77.
257. Sztrymf B, Souza R, Bertoletti I, Jaïs X, Price LC, Simonneau G, Humbert M. Prognostic factors of acute heart failure in patients with pulmonary arterial hypertension. Eur Respir J 2010;35:1286-1293.
258. Zamanian RT, Haddad F, Doyle RL, Weinacker AB. Management strategies for patients with pulmonary hypertension in the intensive care unit. Crit Care Med 2007; 35:2037-2050.
259. Price LC, Wort SJ, Finney SJ, Marino PS, Brett SJ. Pulmonary vascular and right ventricular dysfunction in adult critical care: current and emerging options for management: A systematic literature review. Crit Care 2010;14:R169.
260. Hoeper MM, Granton J. Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med 2011; 184:1114-1124.
261. Rosenzweig EB, Brodie D, Abrams DC, Agerstrand CL, Bacchetta M. Extracorporeal membrane oxygenation as a novel bridging strategy for acute right heart failure in group 1 pulmonary arterial hypertension. ASAIO J 2014;60:129-133.
262. Olsson KM, Simon A, Strueber M, Hadem J, Wiesner O, Gottlieb J, Fuehner T, Fischer S, Warnecke G, Kuhn C, Haverich A, Welte T, Hoeper MM. Extracorporeal membrane oxygenation in nonintubated patients as bridge to lung transplantation. Am J Transplant 2010;10:2173-2178.
263. Fuehner T, Kuehn C, Hadem J, Wiesner O, Gottlieb J, Tudorache I, Olsson KM, Greer M, SommerW, Welte T, Haverich A, Hoeper MM, Warnecke G. Extracorporeal membrane oxygenation in awake patients as bridge to lung transplantation. Am J Respir Crit Care Med 2012;185:763-768.
264. de Perrot M, Granton JT, McRae K, Cypel M, Pierre A, Waddell TK, Yasufuku K, Hutcheon M, Chaparro C, Singer L, Keshavjee S. Impact of extracorporeal life support on outcome in patients with idiopathic pulmonary arterial hypertension awaiting lung transplantation. J Heart Lung Transplant 2011;30:997-1002.
265. Strueber M, Hoeper MM, Fischer S, Cypel M, Warnecke G, Gottlieb J, Pierre A, Welte T, Haverich A, Simon AR, Keshavjee S. Bridge to thoracic organ transplantation in patients with pulmonary arterial hypertension using a pumpless lung assist device. Am J Transplant 2009;9:853-857.
266. Trulock EP, Edwards LB, Taylor DO, Trulock EP, Waltz DA, Keck BM, Hertz MI. Registry of the International Society for Heart and Lung Transplantation: Twenty-third official adult lung and heart lung transplantation report-2006. J Heart Lung Transplant 2006;25:880-892.
267. Toyoda Y, Thacker J, Santos R, Nguyen D, Bhama J, Bermudez C, Kormos R, Johnson B, Crespo M, Pilewski J, Teuteberg J, Alvarez R, Mathier M, McNamara D, McCurry K, Zenati M, Hattler B. Long-term outcome of lung and heart-lung transplantation for idiopathic pulmonary arterial hypertension. Ann Thorac Surg 2008;86:1116-1122.
268. Fadel E, Mercier O, Mussot S, Leroy-Ladurie F, Cerrina J, Chapelier A, Simonneau G, Dartevelle P. Long-term outcome of double-lung and heart2lung transplantation for pulmonary hypertension: A comparative retrospective study of 219 patients. Eur J Cardiothorac Surg 2010;38:277-284.
269. de Perrot M, Granton JT, McRae K, Pierre AF, Singer LG, Waddell TK, Keshavjee S. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: A 14-year single-center experience. J Thorac Cardiovasc Surg 2012;143:910-918.
270. Christie JD, Edwards LB, Kucheryavaya AY, Benden C, Dipchand AI, Dobbels F, Kirk R, Rahmel AO, Stehlik J, Hertz MI. The Registry of the International Society for Heart and Lung Transplantation: 29th adult lung and heart-lung transplant report- 2012. J Heart Lung Transplant 2012;31:1073-1086.
271. Waddell TK, Bennett L, Kennedy R, Todd TR, Keshavjee SH. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002;21: 731-737.
272. Choong CK, Sweet SC, Guthrie TJ, Mendeloff EN, Haddad FJ, Schuler P, De LaMorena M, Huddleston CB. Repair of congenital heart lesions combined with lung transplantation for the treatment of severe pulmonary hypertension: A 13-year experience. J Thorac Cardiovasc Surg 2005;129:661-669.
273. Fleming TR, Powers JH. Biomarkers and surrogate endpoints in clinical trials. Statistic Med 2012;31:2973-2984.
274. Fleming TR. Surrogate endpoints and FDA accelerated approval process. Health Affairs 2005;24:67-78.
275. Tongers J, Schwerdtfeger B, Klein G, Kempf T, Schaefer A, Knapp JM, Niehaus M, Korte T, Hoeper MM. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J 2007;153:127-132.
276. Hoeper MM, Galiè N, Murali S, Olschewski H, Rubenfire M, Robbins IM, Farber HW, McLaughlin V, Shapiro S, Pepke-Zaba J, Winkler J, Ewert R, Opitz C, Westerkamp V, Vachiery JL, Torbicki A, Behr J, Barst RJ. Outcome after cardiopulmonary resuscitation in patients with pulmonary arterial hypertension. Am J Respir Crit Care Med 2002;165:341-344.
277. Showkathali R, Tayebjee MH, Grapsa J, Alzetani M, Nihoyannopoulos P, Howard LS, Lefroy DC, Gibbs JS. Right atrial flutter isthmus ablation is feasible and results in acute clinical improvement in patients with persistent atrial flutter and severe pulmonary arterial hypertension. Int J Cardiol 2011;149:279-280.
278. Zylkowska J, Kurzyna M, Pietura R, Fijalkowska A, Florczyk M, Czajka C, Torbicki A. Recurrent hemoptysis: An emerging life-threatening complication in idiopathic pulmonary arterial hypertension. Chest 2011;139:690-693.
279. Zylkowska J, Kurzyna M, Florczyk M, Burakowska B, Grzegorczyk F, Burakowski J, Wieteska M, Oniszh K, Biederman A, Wawrzynska L, Szturmowicz M, Fijalkowska A, Torbicki A. Pulmonary artery dilatation correlates with the risk of unexpected death in chronic arterial or thromboembolic pulmonary hypertension. Chest 2012;142:1406-1416.
280. Russo V, Zompatori M, Galiè N. Extensive right pulmonary artery dissection in a young patient with chronic pulmonary hypertension. Heart 2012;98:265-266.
281. Demerouti EA, Manginas AN, Athanassopoulos GD, Karatasakis GT. Complications leading to sudden cardiac death in pulmonary arterial hypertension. Respiratory Care 2013;58:1246-1254.
282. Lee MS, Oyama J, Bhatia R, Kim YH, Park SJ. Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: A contemporary review and argument for percutaneous revascularization. Catheter Cardiovasc Interv 2010;76:543-550.
283. Barst RJ, Ertel SI, Beghetti M, Ivy DD. Pulmonary arterial hypertension: A comparison between children and adults. Eur Respir J 2011;37:665-677.
284. Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension. Circulation 2012;125:113-122.
285. van Loon RL, Roofthooft MTR, Hillege HL, ten Harkel ADJ, van Osch-Gevers M, Delhaas T, Kapusta L, Strengers JLM, Rammeloo L, Clur SA, Mulder BJM, Berger RMF. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation 2011;124: 1755-1764.
286. Moledina S, Hislop AA, Foster H, Schulze-Neick I, Haworth SG. Childhood idiopathic pulmonary arterial hypertension: A national cohort study. Heart 2010;96: 1401-1406.
287. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, Bonnet D, Schulze-Neick I, Barst RJ. Clinical features of paediatric pulmonary hypertension: A registry study. Lancet 2012;379:537-546.
288. Hansmann G, Hoeper MM. Registries for paediatric pulmonary hypertension. Eur Respir J 2013;42:580-583.
289. Cerro MJ, Abman S, Diaz G, Freudenthal AH, Freudenthal F, Harikrishnan S, Haworth SG, Ivy D, Lopes AA, Raj JU, Sandoval J, Stenmark K, Adatia I. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ 2011;1:286-298.
290. Beghetti M, Berger RM, Schulze-Neick I, Day RW, Pulido T, Feinstein J, Barst RJ, Humpl T. Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice. Eur Respir J 2013;42:689-700.
291. Schulze-Neick I, Beghetti M. Issues related to the management and therapy of paediatric pulmonary hypertension. Eur Respir Rev 2010;19:331-339.
292. Adatia I, Haworth SG, Wegner M, Barst RJ, Ivy D, Stenmark KR, Karkowsky A, Rosenzweig E, Aguilar C. Clinical trials in neonates and children: report of the Pulmonary Hypertension Academic Research Consortium Pediatric Advisory Committee. Pulm Circ 2013;3:252-266.
293. Lammers AE, Hislop AA, Flynn Y, Haworth SG. Epoprostenol treatment in children with severe pulmonary hypertension. Heart 2007;93:739-743.
294. Barst RJ, Maislin G, Fishman AP. Vasodilator therapy for primary pulmonary hypertension in children. Circulation 1999;99:1197-1208.
295. Levy M, Celermajer DS, Bourges-Petit E, Del Cerro MJ, Bajolle F, Bonnet D. Add-on therapy with subcutaneous treprostinil for refractory pediatric pulmonary hypertension. J Pediatr 2011;158:584-588.
296. Ivy DD, Doran AK, Smith KJ, Mallory GB Jr, Beghetti M, Barst RJ, Brady D, Law Y, Parker D, Claussen L, Abman SH. Short-and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol 2008; 51:161-169.
297. Barst R, Ivy D, Widlitz AC, Moore K, Doran A, Nguyen N, Gaitonde M. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther 2003;73:372-382.
298. Rosenzweig EB, Ivy DD, Widlitz A, Doran A, Claussen LR, Yung D, Abman SH, Morganti A, Nguyen N, Barst RJ. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol 2005;46:697-704.
299. Beghetti M, Haworth SG, Bonnet D, Barst RJ, Acar P, Fraisse A, Ivy DD, Jaïs X, Schulze-Neick I, Galiè N, Morganti A, Dingemanse J, Kusic-Pajic A, Berger RM. Pharmacokinetic and clinical profile of a novel formulation of bosentan in children with pulmonary arterial hypertension: The FUTURE-1 study. Br J Clin Pharmacol 2009;68:948-955.
300. Barst RJ, Ivy DD, Gaitan G, Szatmari A, Rudzinski A, Garcia AE, Sastry BKS, Pulido T, Layton GR, Serdarevic-Pehar M, Wessel DL. A randomized, doubleblind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation 2012; 125:324-334.
301. Barst RJ, Beghetti M, Pulido T, Layton G, Konourina I, Zhang M, Ivy DD. STARTS-2: long-term survival with oral sildenafil monotherapy in treatmentnaive pediatric pulmonary arterial hypertension. Circulation 2014;129: 1914-1923.
302. Takatsuki S, Calderbank M, Ivy DD. Initial experience with tadalafil in pediatric pulmonary arterial hypertension. Pediatr Cardiol 2012;33:683-688.
303. Douwes JM, Roofthooft MT, Van Loon RL, Ploegstra MJ, Bartelds B, Hillege HL, Berger RM. Sildenafil add-on therapy in paediatric pulmonary arterial hypertension, experiences of a national referral centre. Heart 2014;100:224-230.
304. Micheletti A, Hislop AA, Lammers A, Bonhoeffer P, Derrick G, Rees P, Haworth SG. Role of atrial septostomy in the treatment of children with pulmonary arterial hypertension. Heart 2006;92:969-972.
305. Boudjemline Y, Patel M, Malekzadeh-Milani S, Szezepanski I, Levy M, Bonnet D. Patent ductus arteriosus stenting (transcatheter Potts shunt) for palliation of suprasystemic pulmonary arterial hypertension: A case series. Circ Cardiovasc Interv 2013;6:e18-e20.
306. Baruteau AE, Belli E, Boudjemline Y, Laux D, Levy M, Simonneau G, Carotti A, Humbert M, Bonnet D. Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg 2015;47:e105-e110.
307. Esch JJ, Shah PB, Cockrill BA, Farber HW, Landzberg MJ, Mehra MR, Mullen MP, Opotowsky AR, Waxman AB, Lock JE, Marshall AC. Transcatheter Potts shunt creation in patients with severe pulmonary arterial hypertension: initial clinical experience. J Heart Lung Transplant 2013;32:381-387.
308. Ploegstra MJ, Douwes JM, Roofthooft MT, Zijlstra WM, Hillege HL, Berger RM. Identification of treatment goals in paediatric pulmonary arterial hypertension. Eur Respir J 2014;44:1616-1626.
309. Galiè N, Manes A, Palazzini M, Negro L, Marinelli A, Gambetti S, Mariucci E, Donti A, Branzi A, Picchio FM. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome. Drugs 2008;68:1049-1066.
310. Engelfriet PM, Duffels MGJ, Moller T, Boersma E, Tijssen JGP, Thaulow E, Gatzoulis MA, Mulder BJM. Pulmonary arterial hypertension in adults born with a heart septal defect: The Euro Heart Survey on adult congenital heart disease. Heart 2007;93:682-687.
311. Beghetti M, Galiè N. Eisenmenger syndrome: A clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol 2009;53:733-740.
312. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19:1845-1855.
313. 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;15:100-105.
314. Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galiè N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: A comparison between clinical subgroups. Eur Heart J 2014;35: 716-724.
315. Hopkins WE. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron Artery Dis 2005;16:19-25.
316. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007;115:1039-1050.
317. Moller JH, Patton C, Varco RL, Lillehei CW. Late results (30 to 35 years) after operative closure of isolated ventricular septal defect from 1954 to 1960. Am J Cardiol 1991;68:1491-1497.
318. van Albada ME, Berger RM. Pulmonary arterial hypertension in congenital cardiac disease-the need for refinement of the Evian-Venice classification. Cardiol Young 2008;18:10-17.
319. Schulze-Neick I, Beghetti M. Classifying pulmonary hypertension in the setting of the congenitally malformed heart-cleaning up a dog's dinner. Cardiol Young 2008; 18:22-25.
320. Lopes AA, Leary PW. Measurement, interpretation and use of hemodynamic parameters. Cardiol Young 2009;19(Suppl 1):8-12.
321. Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, Davidson SJ, Bouzas B, Gibbs JS, Burman J, Gatzoulis MA. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol 2007;50:634-642.
322. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RMF, Lauer A, Chiossi E, Landzberg M. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol 2007;127:27-32.
323. ZuckermanWA, Leaderer D, Rowan CA, Mituniewicz JD, Rosenzweig EB. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. J Am Coll Cardiol 2011;107:1381-1385.
324. Mukhopadhyay S, Sharma M, Ramakrishnan S, Yusuf J, Gupta MD, Bhamri N, Trehan V, Tyagi S. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: A preliminary observational study. Circulation 2006;114:1807-1810.
325. Khanna D, Gladue H, Channick R, Chung L, Distler O, Furst DE, Hachulla E, Humbert M, Langleben D, Mathai SC, Saggar R, Visovatti S, Altorok N, Townsend W, FitzGerald J, McLaughlin VV. Recommendations for screening and detection of connective tissue disease-associated pulmonary arterial hypertension. Arthritis Rheum 2013;65:3194-3201.
326. Gashouta MA, Humbert M, Hassoun PM. Update in systemic sclerosis-associated pulmonary arterial hypertension. Presse Med 2014;43:e293-e304.
327. Coghlan JG, Denton CP, Gruenig E, Bonderman D, Distler O, Khanna D, Muller-Ladner U, Pope JE, Vonk MC, Doelberg M, Chadha-Boreham H, Heinzl H, Rosenberg DM, McLaughlin VV, Seibold JR. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: The DETECT study. Ann Rheum Dis 2014;73:1340-1349.
328. Humbert M, Yaici A, de Groote P, Montani D, Sitbon O, Launay D, Gressin V, Guillevin L, Clerson P, Simonneau G, Hachulla E. Screening for pulmonary arterial hypertension in patients with systemic sclerosis: clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 2011;63:3522-3530.
329. Hao YJ, Jiang X, ZhouW, Wang Y, Gao L, Wang Y, Li GT, Hong T, Huo Y, Jing ZC, Zhang ZL. Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients. Eur Respir J 2014;44:963-972.
330. Hachulla E, Carpentier P, Gressin V, Diot E, Allanore Y, Sibilia J, Launay D, Mouthon L, Jego P, Cabane J, de Groote P, Chabrol A, Lazareth I, Guillevin L, Clerson P, Humbert M, ItinerAIR-Sclerodermie Study Investigators. Risk factors for death and the 3-year survival of patients with systemic sclerosis: The French ItinerAIR-Sclerodermie study. Rheumatology 2009;48:304-308.
331. Avouac J, Airo P, Meune C, Beretta C, Dieude P, Caramaschi P, Cappelli S, Diot E, Vacca A, Cracowski JL, Sibilia J, Kahan A, Matucci-Cerinic M, Allanore Y. Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies. J Rheumatol 2010;37:2290-2298.
332. Gunther S, Jaïs X, Maitre S, Berezne A, Dorfmuller P, Seferian A, Savale L, Mercier O, Fadel E, Sitbon O, Mouthon L, Simonneau G, Humbert M, Montani D. Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension. Arthritis Rheum 2012;64:2995-3005.
333. Coghlan G. Does left heart disease cause most systemic sclerosis associated pulmonary hypertension? Eur Respir J 2013;42:888-890.
334. Humbert M, Sitbon O, Yaïci A, Montani D, O'Callaghan DS, Jaïs X, Parent F, Savale L, Natali D, Günther S, Chaouat A, Chabot F, Cordier JF, Habib G, Gressin V, Jing ZC, Souza R, Simonneau G. Survival in incident and prevalent cohorts of patients with pulmonary arterial hypertension. Eur Respir J 2010;36: 549-555.
335. Fisher MR, Mathai SC, Champion HC, Girgis RE, Housten-Harris T, Hummers L, Krishnan JA, Wigley F, Hassoun PM. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006;54: 3043-3050.
336. Launay D, Sitbon O, Hachulla E, Mouthon L, Gressin V, Rottat L, Clerson P, Cordier JF, Simonneau G, Humbert M. Survival in systemic sclerosis-associated pulmonary arterial hypertension in the modern management era. Ann Rheum Dis 2013;72:1940-1946.
337. O'Callaghan DS, Dorfmuller P, Jaïs X, Mouthon L, Sitbon O, Simonneau G, Humbert M, Montani D. Pulmonary veno-occlusive disease: The bete noire of pulmonary hypertension in connective tissue diseases? Presse Med 2011;40: e65-e78.
338. Demling RH, Smith M, Gunther R, Flynn JT, Gee MH. Pulmonary injury and prostaglandin production during endotoxemia in conscious sheep. Am J Physiol 1981; 240:H348-H353.
339. Jaïs X, Launay D, Yaici A, Le PJ, Tcherakian C, Sitbon O, Simonneau G, Humbert M. Immunosuppressive therapy in lupus-and mixed connective tissue disease-associated pulmonary arterial hypertension: A retrospective analysis of twenty-three cases. Arthritis Rheum 2008;58:521-531.
340. Avouac J, Wipff J, Kahan A, Allanore Y. Effects of oral treatments on exercise capacity in systemic sclerosis related pulmonary arterial hypertension: A meta-analysis of randomised controlled trials. Ann Rheum Dis 2008;67:808-814.
341. Launay D, Savale L, Berezne A, Le PJ, Hachulla E, Mouthon L, Sitbon O, Lambert B, Gaudric M, Jaïs X, Stephan F, Hatron PY, Lamblin N, Vignaux O, Cottin V, Farge D, Wallaert B, Guillevin L, Simonneau G, Mercier O, Fadel E, Dartevelle P, Humbert M, Mussot S. Lung and heart-lung transplantation for systemic sclerosis patients. A monocentric experience of 13 patients, review of the literature and position paper of a multidisciplinary working group. Presse Med 2014;43: e345-e363.
342. Hoeper MM, Krowka MJ, Strassburg CP. Portopulmonary hypertension and hepatopulmonary syndrome. Lancet 2004;363:1461-1468.
343. Zopey R, Susanto I, Barjaktarevic I, Wang T. Transition from hepatopulmonary syndrome to portopulmonary hypertension: A case series of 3 patients. Case Rep Pulmonol 2013;2013:561870.
344. Krowka MJ, Swanson KL, Frantz RP, McGoon MD, Wiesner RH. Portopulmonary hypertension: results from a 10-year screening algorithm. Hepatology 2006;44: 1502-1510.
345. Kawut SM, Krowka MJ, Trotter JF, Roberts KE, Benza RL, Badesch DB, Taichman DB, Horn EM, Zacks S, Kaplowitz N, Brown RS Jr, Fallon MB. Clinical risk factors for portopulmonary hypertension. Hepatology 2008;48:196-203.
346. Roberts KE, Fallon MB, Krowka MJ, Brown RS, Trotter JF, Peter I, Tighiouart H, Knowles JA, Rabinowitz D, Benza RL, Badesch DB, Taichman DB, Horn EM, Zacks S, Kaplowitz N, Kawut SM. Genetic risk factors for portopulmonary hypertension in patients with advanced liver disease. Am J Respir Crit Care Med 2009;179: 835-842.
347. Krowka MJ, Miller DP, Barst RJ, Taichman D, Dweik RA, Badesch DB, McGoon MD. Portopulmonary hypertension: A report from the US-based REVEAL Registry. Chest 2012;141:906-915.
348. Le Pavec J, Souza R, Herve P, Lebrec D, Savale L, Tcherakian C, Jaïs X, Yaici A, Humbert M, Simonneau G, Sitbon O. Portopulmonary hypertension: survival and prognostic factors. Am J Respir Crit Care Med 2008;178:637-643.
349. Provencher S, Herve P, Jaïs X, Lebrec D, Humbert M, Simonneau G, Sitbon O. Deleterious effects of beta-blockers on exercise capacity and hemodynamics in patients with portopulmonary hypertension. Gastroenterology 2006;130:120-126.
350. Hoeper MM, Seyfarth HJ, Hoeffken G, Wirtz H, Spiekerkoetter E, Pletz MW, Welte T, Halank M. Experience with inhaled iloprost and bosentan in portopulmonary hypertension. Eur Respir J 2007;30:1096-1102.
351. Halank M, Knudsen L, Seyfarth HJ, Ewert R, Wiedemann B, Kolditz M, Hoffken G, Hoeper MM. Ambrisentan improves exercise capacity and symptoms in patients with portopulmonary hypertension. Z Gastroenterol 2011;49:1258-1262.
352. Reichenberger F, Voswinckel R, Steveling E, Enke B, Kreckel A, Olschewski H, Grimminger F, SeegerW, Ghofrani HA. Sildenafil treatment for portopulmonary hypertension. Eur Respir J 2006;28:563-567.
353. 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;63:604-606.
354. Swanson KL, Wiesner RH, Nyberg SL, Rosen CB, Krowka MJ. Survival in portopulmonary hypertension: Mayo Clinic experience categorized by treatment subgroups. Am J Transplant 2008;8:2445-2453.
355. Sakai T, Planinsic RM, Mathier MA, de Vera ME, Venkataramanan R. Initial experience using continuous intravenous treprostinil to manage pulmonary arterial hypertension in patients with end-stage liver disease. Transpl Int 2009;22: 554-561.
356. Savale L, Magnier R, Le Pavec J, Jaïs X, Montani D, O'Callaghan DS, Humbert M, Dingemanse J, Simonneau G, Sitbon O. Efficacy, safety and pharmacokinetics of bosentan in portopulmonary hypertension. Eur Respir J 2013;41:96-103.
357. Humbert M, Segal ES, Kiely DG, Carlsen J, Schwierin B, Hoeper MM. Results of European post-marketing surveillance of bosentan in pulmonary hypertension. Eur Respir J 2007;30:338-344.
358. Kia L, Shah SJ, Wang E, Sharma D, Selvaraj S, Medina C, Cahan J, Mahon H, Levitsky J. Role of pretransplant echocardiographic evaluation in predicting outcomes following liver transplantation. Am J Transplant 2013;13:2395-2401.
359. Krowka MJ, Plevak DJ, Findlay JY, Rosen CB, Wiesner RH, Krom RA. Pulmonary hemodynamics and perioperative cardiopulmonary-related mortality in patients with portopulmonary hypertension undergoing liver transplantation. Liver Transplant 2000;6:443-450.
360. AshfaqM, Chinnakotla S, Rogers L, Ausloos K, Saadeh S, Klintmalm GB, Ramsay M, Davis GL. The impact of treatment of portopulmonary hypertension on survival following liver transplantation. Am J Transplant 2007;7:1258-1264.
361. Raevens S, De Pauw M, Reyntjens K, Geerts A, Verhelst X, Berrevoet F, Rogiers X, Troisi RI, Van Vlierberghe H, Colle I. Oral vasodilator therapy in patients with moderate to severe portopulmonary hypertension as a bridge to liver transplantation. Eur J Gastroenterol Hepatol 2013;25:495-502.
362. Plotkin JS, Kuo PC, Rubin LJ, Gaine S, Howell CD, Laurin J, Njoku MJ, Lim JW, Johnson LB. Successful use of chronic epoprostenol as a bridge to liver transplantation in severe portopulmonary hypertension. Transplantation 1998;65:457-459.
363. Austin MJ, McDougall NI, Wendon JA, Sizer E, Knisely AS, Rela M, Wilson C, Callender ME, O'Grady JG, Heneghan MA. Safety and efficacy of combined use of sildenafil, bosentan, and iloprost before and after liver transplantation in severe portopulmonary hypertension. Liver Transplant 2008;14:287-291.
364. Grannas G, Neipp M, Hoeper MM, Gottlieb J, Luck R, Becker T, Simon A, Strassburg CP, Manns MP, Welte T, Haverich A, Klempnauer J, Nashan B, Strueber M. Indications for and outcomes after combined lung and liver transplantation: A single-center experience on 13 consecutive cases. Transplantation 2008;85:524-531.
365. Robalino BD, Moodie DS. Association between primary pulmonary hypertension and portal hypertension: Analysis of its pathophysiology and clinical, laboratory and hemodynamic manifestations. J Am Coll Cardiol 1991;17:492-498.
366. Barbaro G, Lucchini A, Pellicelli AM, Grisorio B, Giancaspro G, Barbarini G. Highly active antiretroviral therapy compared with HAART and bosentan in combination in patients with HIV-associated pulmonary hypertension. Heart 2006;92: 1164-1166.
367. Degano B. HIV-associated pulmonary arterial hypertension: survival and prognostic factors in the modern therapeutic era. AIDS 2010;24:67-75.
368. Opravil M, Pechère M, Speich R, Joller Jemelka HI, Jenni R, Russi EW, Hirschel B, Lüthy R. HIV-associated primary pulmonary hypertension. A case control study. Swiss HIV Cohort Study. Am J Respir Crit Care Med 1997;155:990-995.
369. Sitbon O, Lascoux-Combe C, Delfraissy JF, Yeni PG, Raffi F, De Zuttere D, Gressin V, Clerson P, Sereni D, Simonneau G. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med 2008;177:108-113.
370. 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;170:1212-1217.
371. Montani D, Achouh L, Dorfmuller P, Le Pavec J, Sztrymf B, Tcherakian C, Rabiller A, Haque R, Sitbon O, Jaïs X, Dartevelle P, Maitre S, Capron F, Musset D, Simonneau G, Humbert M. Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology. Medicine (Baltimore) 2008;87:220-233.
372. Lantuejoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG. Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A clinicopathologic study of 35 cases. Am J Surg Pathol 2006;30:850-857.
373. Humbert M, Mâtre S, Capron F, Rain B, Musset D, Simonneau G. Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis. Am J Respir Crit Care Med 1998;157:1681-1685.
374. Mandel J, Mark EJ, Hales CA. Pulmonary veno-occlusive disease. Am J Respir Crit Care Med 2000;162:1964-1973.
375. Langleben D, Heneghan JM, Batten AP, Wang NS, Fitch N, Schlesinger RD, Guerraty A, Rouleau JL. Familial pulmonary capillary hemangiomatosis resulting in primary pulmonary hypertension. Ann Intern Med 1988;109:106-109 [published erratum appears in Ann Intern Med 1988;109:439].
376. Best DH, Sumner KL, Austin ED, Chung WK, Brown LM, Borczuk AC, Rosenzweig EB, Bayrak-Toydemir P, Mao R, Cahill BC, Tazelaar HD, Leslie KO, Hemnes AR, Robbins IM, Elliott CG. EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest 2014;145:231-236.
377. Seferian A, Helal B, Jaïs X, Girerd B, Price LC, Gunther S, Savale L, Dorfmuller P, Parent F, Sitbon O, Humbert M, Simonneau G, Montani D. Ventilation/perfusion lung scan in pulmonary veno-occlusive disease. Eur Respir J 2012;40:75-83.
378. Rabiller A, Jaïs X, Hamid A, Resten A, Parent F, Haque R, Capron F, Sitbon O, Simonneau G, Humbert M. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J 2006;27:108-113.
379. Montani D, Jaïs X, Price LC, Achouh L, Degano B, Mercier O, Mussot S, Fadel E, Dartevelle P, Sitbon O, Simonneau G, Humbert M. Cautious epoprostenol therapy is a safe bridge to lung transplantation in pulmonary veno-occlusive disease. Eur Respir J 2009;34:1348-1356.
380. Fang JC, DeMarco T, Givertz MM, Borlaug BA, Lewis GD, Rame JE, Gomberg-Maitland M, Murali S, Frantz RP, McGlothlin D, Horn EM, Benza RL. World Health Organization Pulmonary Hypertension Group 2: pulmonary hypertension due to left heart disease in the adult-a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2012;31:913-933.
381. Ghio S, Gavazzi A, Campana C, Inserra C, Klersy C, Sebastiani R, Arbustini E, Recusani F, Tavazzi L. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol 2001;37:183-188.
382. Thenappan T, Shah SJ, Gomberg-Maitland M, Collander B, Vallakati A, Shroff P, Rich S. Clinical characteristics of pulmonary hypertension in patients with heart failure and preserved ejection fraction. Circ Heart Fail 2011;4:257-265.
383. Robbins IM, Newman JH, Johnson RF, Hemnes AR, Fremont RD, Piana RN, Zhao DX, Byrne DW. Association of the metabolic syndrome with pulmonary venous hypertension. Chest 2009;136:31-36.
384. Bursi F, McNallan SM, Redfield MM, Nkomo VT, Lam CS, Weston SA, Jiang R, Roger VL. Pulmonary pressures and death in heart failure: A community study. J Am Coll Cardiol 2012;59:222-231.
385. Gerges C, Gerges M, Lang MB, Zhang Y, Jakowitsch J, Probst P, Maurer G, Lang IM. Diastolic pulmonary vascular pressure gradient: A predictor of prognosis in "out-of-proportion" pulmonary hypertension. Chest 2013;143:758-766.
386. Moraes DL, ColucciWS, Givertz MM. Secondary pulmonary hypertension in chronic heart failure: The role of the endothelium in pathophysiology and management. Circulation 2000;102:1718-1723.
387. Rapp AH, Lange RA, Cigarroa JE, Keeley EC, Hillis LD. Relation of pulmonary arterial diastolic and mean pulmonary arterial wedge pressures in patients with and without pulmonary hypertension. Am J Cardiol 2001;88:823-824.
388. Miller WL, Grill DE, Borlaug BA. Clinical features, hemodynamics, and outcomes of pulmonary hypertension due to chronic heart failure with reduced ejection fraction: pulmonary hypertension and heart failure. JACC Heart Fail 2013;1: 290-299.
389. Tampakakis E, Leary PJ, Selby VN, De Marco T, Cappola TP, Felker GM, Russell SD, Kasper EK, Tedford RJ. The diastolic pulmonary gradient does not predict survival in patients with pulmonary hypertension due to left heart disease. JACC Heart Fail 2015;3:9-16.
390. Pellegrini P, Rossi A, Pasotti M, Raineri C, Cicoira M, Bonapace S, Dini FL, Temporelli PL, Vassanelli C, Vanderpool R, Naeije R, Ghio S. Prognostic relevance of pulmonary arterial compliance in patients with chronic heart failure. Chest 2014;145:1064-1070.
391. Chatterjee NA, Lewis GD. Characterization of pulmonary hypertension in heart failure using the diastolic pressure gradient: limitations of a solitary measurement. JACC Heart Fail 2015;3:17-21.
392. McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Bohm M, Dickstein K, Falk V, Filippatos G, Fonseca C, Gomez-Sanchez MA, Jaarsma T, Kober L, Lip GY, Maggioni AP, Parkhomenko A, Pieske BM, Popescu BA, Ronnevik PK, Rutten FH, Schwitter J, Seferovic P, Stepinska J, Trindade PT, Voors AA, Zannad F, Zeiher A. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2012: The Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2012 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association (HFA) of the ESC. Eur Heart J 2012;33:1787-1847.
393. Khush KK, Tasissa G, Butler J, McGlothlin D, De MT. Effect of pulmonary hypertension on clinical outcomes in advanced heart failure: Analysis of the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) database. Am Heart J 2009;157:1026-1034.
394. Patel ND, Weiss ES, Schaffer J, Ullrich SL, Rivard DC, Shah AS, Russell SD, Conte JV. Right heart dysfunction after left ventricular assist device implantation: A comparison of the pulsatile HeartMate I and axial-flow HeartMate II devices. Ann Thorac Surg 2008;86:832-840.
395. Torre-Amione G, Southard RE, Loebe MM, Youker KA, Bruckner B, Estep JD, Tierney M, Noon GP. Reversal of secondary pulmonary hypertension by axial and pulsatile mechanical circulatory support. J Heart Lung Transplant 2010;29: 195-200.
396. Barnett CF, DeMarco T. Pulmonary hypertension associated with left-sided heart disease. Heart Fail Clin 2012;8:447-459.
397. Bonderman D, Ghio S, Felix SB, Ghofrani HA, Michelakis E, Mitrovic V, Oudiz RJ, Boateng F, Scalise AV, Roessig L, Semigran MJ. Riociguat for patients with pulmonary hypertension caused by systolic left ventricular dysfunction: A phase IIb double-blind, randomized, placebo-controlled, dose-ranging hemodynamic study. Circulation 2013;128:502-511.
398. Oswald-Mammosser M, Weitzenblum E, Quoix E, Moser G, Chaouat A, Charpentier C, Kessler R. Prognostic factors in COPD patients receiving longterm oxygen therapy. Importance of pulmonary artery pressure. Chest 1995;107: 1193-1198.
399. 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;164:219-224.
400. Lettieri CJ, Nathan SD, Barnett SD, Ahmad S, Shorr AF. Prevalence and outcomes of pulmonary arterial hypertension in advanced idiopathic pulmonary fibrosis. Chest 2006;129:746-752.
401. 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;127:1531-1536.
402. 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;172:189-194.
403. Arcasoy SM, Christie JD, Ferrari VA, St John SM, Zisman DA, Blumenthal NP, Pochettino A, Kotloff RM. Echocardiographic assessment of pulmonary hypertension in patients with advance lung disease. Am J Respir Crit Care Med 2003;167: 735-740.
404. Fisher MR, Forfia PR, Chamera E, Housten-Harris T, Champion HC, Girgis RE, Corretti MC, Hassoun PM. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179:615-621.
405. Nathan SD, Shlobin OA, Barnett SD, Saggar R, Belperio JA, Ross DJ, Ahmad S, Saggar R, Libre E, Lynch JP III, Zisman DA. Right ventricular systolic pressure by echocardiography as a predictor of pulmonary hypertension in idiopathic pulmonary fibrosis. Respir Med 2008;102:1305-1310.
406. Agusti AG, Barbera JA, Roca J, Wagner PD, Guitart R, Rodriguez-Roisin R. Hypoxic pulmonary vasoconstriction and gas exchange during exercise in chronic obstructive pulmonary disease. Chest 1990;97:268-275.
407. Barbera JA, Roger N, Roca J, Rovira I, Higenbottam TW, Rodriguez-Roisin R. Worsening of pulmonary gas exchange with nitric oxide inhalation in chronic obstructive pulmonary disease. Lancet 1996;347:436-440.
408. Simonneau G, Escourrou P, Duroux P, Lockhart A. Inhibition of hypoxic pulmonary vasoconstriction by nifedipine. N Engl J Med 1981;304:1582-1585.
409. Morrell NW, Higham MA, Phillips PG, Shakur BH, Robinson PJ, Beddoes RJ. Pilot study of losartan for pulmonary hypertension in chronic obstructive pulmonary disease. Respir Res 2005;6:88-95.
410. Saadjian A, Philip-Joet F, Paganelli F, Arnaud A, Levy S. Long-term effects of cicletanine on secondary pulmonary hypertension. J Cardiovasc Pharmacol 1998;31: 364-371.
411. Stolz D, Rasch H, Linka A, Di Valentino M, Meyer A, Brutsche M, Tamm M. A randomised, controlled trial of bosentan in severe COPD. Eur Respir J 2008;32: 619-628.
412. Blanco I, Gimeno E, Munoz PA, Pizarro S, Gistau C, Rodriguez-Roisin R, Roca J, Barbera JA. Hemodynamic and gas exchange effects of sildenafil in patients with chronic obstructive pulmonary disease and pulmonary hypertension. Am J Respir Crit Care Med 2010;181:270-278.
413. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, HunninghakeGW. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 2010;363:620-628.
414. Blanco I, Santos S, Gea J, Guell R, Torres F, Gimeno-Santos E, Rodriguez DA, Vilaro J, Gomez B, Roca J, Barbera JA. Sildenafil to improve respiratory rehabilitation outcomes in COPD: A controlled trial. Eur Respir J 2013;42:982-992.
415. Lederer DJ, Bartels MN, Schluger NW, Brogan F, Jellen P, Thomashow BM, Kawut SM. Sildenafil for chronic obstructive pulmonary disease: A randomized crossover trial. COPD 2012;9:268-275.
416. Goudie AR, Lipworth BJ, Hopkinson PJ, Wei L, Struthers AD. Tadalafil in patients with chronic obstructive pulmonary disease: A randomised, double-blind, parallel-group, placebo-controlled trial. Lancet Respir Med 2014;2:293-300.
417. Lang IM, Pesavento R, Bonderman D, Yuan JX. Risk factors and basic mechanisms of chronic thromboembolic pulmonary hypertension: A current understanding. Eur Respir J 2013;41:462-468.
418. Guerin L, Couturaud F, Parent F, Revel MP, Gillaizeau F, Planquette B, Pontal D, Guegan M, Simonneau G, Meyer G, Sanchez O. Prevalence of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. Prevalence of CTEPH after pulmonary embolism. Thromb Haemost 2014;112:598-605.
419. Pepke-Zaba J, Jansa P, Kim NH, Naeije R, Simonneau G. Chronic thromboembolic pulmonary hypertension: role of medical therapy. Eur Respir J 2013;41:985-990.
420. Madani MM, Wittine LM, Auger WR, Fedullo PF, Kerr KM, Kim NH, Test VJ, Kriett JM, Jamieson SW. Chronic thromboembolic pulmonary hypertension in pediatric patients. J Thorac Cardiovasc Surg 2011;141:624-630.
421. Pepke-Zaba J, Hoeper MM, Humbert M. Chronic thromboembolic pulmonary hypertension: Advances from bench to patient management. Eur Respir J 2013; 41:8-9.
422. Lang IM, Simonneau G, Pepke-Zaba JW, Mayer E, Ambroz D, Blanco I, Torbicki A, Mellemkjaer S, Yaici A, Delcroix M. Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study. Thromb Haemost 2013;110:83-91.
423. Lang IM, Plank C, Sadushi-Kolici R, Jakowitsch J, KlepetkoW, Maurer G. Imaging in pulmonary hypertension. JACC Cardiovasc Imaging 2010;3:1287-1295.
424. Hoeper MM, Mayer E, Simonneau G, Rubin LJ. Chronic thromboembolic pulmonary hypertension. Circulation 2006;113:2011-2020.
425. Sherrick AD, Swensen SJ, Hartman TE. Mosaic pattern of lung attenuation on CT scans: frequency among patients with pulmonary artery hypertension of different causes. Am J Roentgenol 1997;169:79-82.
426. Ley S, Ley-Zaporozhan J, Pitton MB, Schneider J, Wirth GM, Mayer E, Duber C, Kreitner KF. Diagnostic performance of state-of-the-art imaging techniques for morphological assessment of vascular abnormalities in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Eur Radiol 2012;22: 607-616.
427. Sugiyama M, Fukuda T, Sanda Y, Morita Y, Higashi M, Ogo T, Tsuji A, Demachi J, Nakanishi N, Naito H. Organized thrombus in pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension; imaging with cone beam computed tomography. Jpn J Radiol 2014;32:375-382.
428. Shure D, Gregoratos G, Moser KM. Fiberoptic angioscopy: role in the diagnosis of chronic pulmonary arterial obstruction. Ann Intern Med 1985;103:844-850.
429. Skoro-Sajer N, Marta G, Gerges C, Hlavin G, Nierlich P, Taghavi S, Sadushi-Kolici R, Klepetko W, Lang IM. Surgical specimens, haemodynamics and long-term outcomes after pulmonary endarterectomy. Thorax 2014;69: 116-122.
430. Mayer E, Jenkins D, Lindner J, D'Armini A, Kloek J, Meyns B, Ilkjaer LB, Klepetko W, Delcroix M, Lang I, Pepke-Zaba J, Simonneau G, Dartevelle P. Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. J Thorac Cardiovasc Surg 2011;141:702-710.
431. Madani MM, Auger WR, Pretorius V, Sakakibara N, Kerr KM, Kim NH, Fedullo PF, Jamieson SW. Pulmonary endarterectomy: recent changes in a single institution's experience of more than 2, 700 patients. Ann Thorac Surg 2012;94:97-103.
432. Jenkins D, Mayer E, Screaton N, Madani M. State-of-the-art chronic thromboembolic pulmonary hypertension diagnosis and management. Eur Respir Rev 2012;21:32-39.
433. Vuylsteke A, Sharples L, Charman G, Kneeshaw J, Tsui S, Dunning J, Wheaton E, Klein A, Arrowsmith J, Hall R, Jenkins D. Circulatory arrest versus cerebral perfusion during pulmonary endarterectomy surgery (PEACOG): A randomised controlled trial. Lancet 2011;378:1379-1387.
434. Thistlethwaite PA, Madani MM, Kemp AD, Hartley M, Auger WR, Jamieson SW. Venovenous extracorporeal life support after pulmonary endarterectomy: indications, techniques, and outcomes. Ann Thorac Surg 2006;82:2139-2145.
435. Berman M, Tsui S, Vuylsteke A, Snell A, Colah S, Latimer R, Hall R, Arrowsmith JE, Kneeshaw J, Klein AA, Jenkins DP. Successful extracorporeal membrane oxygenation support after pulmonary thromboendarterectomy. Ann Thorac Surg 2008;86: 1261-1267.
436. Mydin M, Berman M, Klein A, Tsui S, Dunning J, Valchanov K, Vuylsteke A, Jenkins DP. Extracorporeal membrane oxygenation as a bridge to pulmonary endarterectomy. Ann Thorac Surg 2011;92:e101-e103.
437. Hughes RJ, Jaïs X, Bonderman D, Suntharalingam J, Humbert M, Lang I, Simonneau G, Pepke-Zaba J. The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: A 1-year follow-up study. Eur Respir J 2006;28:138-143.
438. Skoro-Sajer N, Bonderman D, Wiesbauer F, Harja E, Jakowitsch J, Klepetko W, Kneussl MP, Lang IM. Treprostinil for severe inoperable chronic thromboembolic pulmonary hypertension. Thromb Haemost 2007;5:483-489.
439. Reichenberger F, Voswinckel R, Enke B, Rutsch M, Fechtali EE, Schmehl T, Olschewski H, Schermuly R, Weissmann N, Ghofrani HA, Grimminger F, Mayer E, Seeger W. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension. Eur Respir J 2007;30:922-927.
440. Jaïs X, D'Armini AM, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, Hoeper MM, Lang IM, Mayer E, Pepke-Zaba J, Perchenet L, Morganti A, Simonneau G, Rubin LJ. Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension: BENEFiT (Bosentan Effects in iNopErable Forms of chronIc Thromboembolic pulmonary hypertension), a randomized, placebo-controlled trial. J Am Coll Cardiol 2008;52:2127-2134.
441. Ghofrani HA, D'Armini AM, Grimminger F, Hoeper MM, Jansa P, Kim NH, Mayer E, Simonneau G, Wilkins MR, Fritsch A, Neuser D, Weimann G, Wang C. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013;369:319-329.
442. Reesink HJ, Surie S, Kloek JJ, Tan HL, Tepaske R, Fedullo PF, Bresser P. Bosentan as a bridge to pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension. J Thorac Cardiovasc Surg 2010;139:85-91.
443. Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ. Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation 2001;103:10-13.
444. Kataoka M, Inami T, Hayashida K, Shimura N, Ishiguro H, Abe T, Tamura Y, Ando M, Fukuda K, Yoshino H, Satoh T. Percutaneous transluminal pulmonary angioplasty for the treatment of chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012;5:756-762.
445. Mizoguchi H, Ogawa A, Munemasa M, Mikouchi H, Ito H, Matsubara H. Refined balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension. Circ Cardiovasc Interv 2012;5:748-755.
446. Sugimura K, Fukumoto Y, Satoh K, Nochioka K, Miura Y, Aoki T, Tatebe S, Miyamichi-Yamamoto S, Shimokawa H. Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-termprognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J 2012; 76:485-488.
447. Inami T, Kataoka M, Shimura N, Ishiguro H, Yanagisawa R, Taguchi H, Fukuda K, Yoshino H, Satoh T. Pulmonary edema predictive scoring index (PEPSI), a new index to predict risk of reperfusion pulmonary edema and improvement of hemodynamics in percutaneous transluminal pulmonary angioplasty. JACC Cardiovasc Interv 2013;6:725-736.
448. Andreassen AK, Ragnarsson A, Gude E, Geiran O, Andersen R. Balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension. Heart 2013;99:1415-1420.
449. Rubin LJ, Hoeper MM, KlepetkoW, Galiè N, Lang IM, Simonneau G. Current and future management of chronic thromboembolic pulmonary hypertension: from diagnosis to treatment responses. Proc Am Thorac Soc 2006;3:601-607.
450. Peacock A. Pulmonary hypertension due to chronic hypoxia. BMJ 1990;300:763.
451. Recommendations on the management of pulmonary hypertension in clinical practice. Heart 2001;86(Suppl 1):I1-I13.
452. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Thorax 2008;63(Suppl 2):ii1-ii41.
453. Barbera JA, Escribano P, Morales P, Gomez MA, Oribe M, Martinez A, Roman A, Segovia J, Santos F, Subirana MT. [Standards of care in pulmonary hypertension. Consensus statement of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC)]. Rev Esp Cardiol 2008;61:170-184.
454. Armstrong I, Rochnia N, Harries C, Bundock S, Yorke J. The trajectory to diagnosis with pulmonary arterial hypertension: A qualitative study. BMJ Open 2012;2: e000806.
455. Ghofrani HA, Distler O, Gerhardt F, Gorenflo M, Grunig E, Haefeli WE, Held M, Hoeper MM, Kahler CM, Kaemmerer H, Klose H, Kollner V, Kopp B, Mebus S, Meyer A, Miera O, Pittrow D, Riemekasten G, Rosenkranz S, Schranz D, Voswinckel R, Olschewski H. Treatment of pulmonary arterial hypertension (PAH): updated Recommendations of the Cologne Consensus Conference 2011. Int J Cardiol 2011;154(Suppl 1):S20-S33.
456. Vachiery JL, Gaine S. Challenges in the diagnosis and treatment of pulmonary arterial hypertension. Eur Respir Rev 2012;21:313-320.