pulmoПульмонологияPULMONOLOGIYA0869-01892541-9617Scientific and Practical Journal “PULMONOLOGIYA” LLC10.18093/0869-0189-2017-27-3-321-345pulmo-875Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESЛегочная гипертензия. Диагностика и лечение. Европейские клинические рекомендации (2015) (часть 1-я)Pulmonary hypertension. Diagnosis and therapy. European guidelines, 2015 (Part 1)РедакционнаястатьяEditorialArticle201728082017273321345Copyright © Редакционная с., 20172017Редакционная с.Editorial a.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.pulmonology.ru/pulm/article/view/875

Легочная гипертензия (ЛГ) – это патофизиологическое состояние, которое может развиваться при многих заболеваниях и осложнять большинство сердечно-сосудистых и бронхолегочных заболеваний. Целью создания рекомендаций Европейского общества кардиологов (European Society of Cardiology (ESC)) и Европейского респираторного общества (European Respiratory Society (ERS)) явилось не только объединение большинства исследований последних лет, но и разработка образовательного инструмента и программ внедрения данных рекомендаций в клиническую практику. Данный документ продолжает 2 предшествующих варианта клинических рекомендаций ESC и ERS (2004, 2009) и концентрируется на клиническом ведении больных с ЛГ, однако при этом отмечены значительные отличия от версии 2009 г., суммированы все существующие на сегодняшний день доказательства по диагностике и лечению ЛГ для оказания помощи врачам при повседневном выборе оптимальной стратегии ведения конкретного пациента с учетом желаемого исхода и соотношения риск / польза конкретных диагностических и терапевтических мероприятий. Систематический обзор литературы выполнен по публикациям базы MEDLINE, в которой искали новые исследования по соответствующей теме, опубликованные после 2009 г. При создании рекомендаций тщательно обсуждалась вся научная и медицинская информация и доступные доказательства. Ожидаемые исходы заболевания оценены для больших популяций, насколько позволяли существующие данные. Подчеркивается, что ESC поддерживает национальные кардиологические общества в переводе всех рекомендаций ESC на национальные языки и внедрении их в практику этих стран, поскольку показано, что исход ЛГ напрямую зависит от выполнения действующих клинических рекомендаций. Окончательный вариант данных рекомендаций утвержден Комитетом ESC и ERS и опубликован в журналах European Heart Journal и European Respiratory Journal.

Pulmonary hypertension (PH) is a pathophysiological condition which could develop in many diseases. PH could also complicate majority of cardiovascular and pulmonary diseases. This guideline was developed by European Society of Cardiology (ESC) and European Respiratory Society (ERS). The aim of this guideline was to integrate majority of recent studies and to create educational tools and programmes for implementation of these recommendations into clinical practice. The authors made a systematic review of studies published in Medline database since 2009. An updated common classification for PH in adults and children is included in this guideline. New haemodynamic definition of pulmonary arterial hypertension involves pulmonary vascular resistance. This guideline includes novel findings on pathology, pathobiology, genetics, epidemiology, and risk factors. The authors updated a diagnostic algorithm and screening strategy, and described recent achievements in evaluating severity and therapy of PH including combined treatment. Also, a new short chapter on PH with unclear and/ or multifactorial mechanisms was added.

легочная гипертензиялегочная артериальная гипертензияхроническая тромбоэмболическая легочная гипертензиякардиореспираторное нагрузочное тестированиеpulmonary hypertensionclassificationpulmonary arterial hypertensionchronic thromboembolic pulmonary hypertensiondiagnostic algorithmcombined therapyReferencesHoeper M.M., Bogaard H.J., Condliffe R. et al. Definitions and diagnosis of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (Suppl.): D42–D50. DOI: 10.1016/j.jacc.2013.10.032.Hoeper M.M., Bogaard H.J., Condliffe R. et al. Definitions and diagnosis of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (Suppl.): D42–D50. DOI: 10.1016/j.jacc.2013.10.032.Kovacs G., Berghold A., Scheidl S. et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: A systematic review. Eur. Respir. J. 2009; 34 (4): 888–894. DOI: 10.1183/09031936.00145608.Kovacs G., Berghold A., Scheidl S. et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: A systematic review. Eur. Respir. J. 2009; 34 (4): 888–894. DOI: 10.1183/09031936.00145608.Herve P., Lau E., Sitbon O. et al. Criteria for diagnosis of exercise pulmonary hypertension. Eur. Respir. J. 2015; 46 (3): 728–737. DOI: 10.1183/09031936.00021915.Herve P., Lau E., Sitbon O. et al. Criteria for diagnosis of exercise pulmonary hypertension. Eur. Respir. J. 2015; 46 (3): 728–737. DOI: 10.1183/09031936.00021915.Vachiery J.L., Adir Y., Barbera J.A. et al. Pulmonary hypertension due to left heart disease. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D100–D108. DOI: 10.1016/j.jacc.2013.10.033.Vachiery J.L., Adir Y., Barbera J.A. et al. Pulmonary hypertension due to left heart disease. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D100–D108. DOI: 10.1016/j.jacc.2013.10.033.Simonneau G., Galiè N., Rubin L.J. et al. Clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2004; 43 (Suppl. 1): S5–S12.Simonneau G., Galiè N., Rubin L.J. et al. Clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2004; 43 (Suppl. 1): S5–S12.Simonneau G., Gatzoulis M.A., Adatia I. et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D34–D41. DOI: 10.1016/j.jacc.2013.10.029.Simonneau G., Gatzoulis M.A., Adatia I. et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D34–D41. DOI: 10.1016/j.jacc.2013.10.029.Dhillon R. The management of neonatal pulmonary hypertension. Arch. Dis. Child Fetal. Neonatal. Ed. 2012; 97 (3): F223–F228. DOI: 10.1136/adc.2009.180091.Dhillon R. The management of neonatal pulmonary hypertension. Arch. Dis. Child Fetal. Neonatal. Ed. 2012; 97 (3): F223–F228. DOI: 10.1136/adc.2009.180091.Porta N.F., Steinhorn R.H. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin. Perinatol. 2012; 39 (1): 149–164. DOI: 10.1016/j.clp.2011.12.006.Porta N.F., Steinhorn R.H. Pulmonary vasodilator therapy in the NICU: inhaled nitric oxide, sildenafil, and other pulmonary vasodilating agents. Clin. Perinatol. 2012; 39 (1): 149–164. DOI: 10.1016/j.clp.2011.12.006.Ivy D.D., Abman S.H., Barst R.J. et al. Pediatric pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D117–D126. DOI: 10.1016/j.jacc.2013.10.028.Ivy D.D., Abman S.H., Barst R.J. et al. Pediatric pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D117–D126. DOI: 10.1016/j.jacc.2013.10.028.Humbert M., Sitbon O., Chaouat A. et al. Pulmonary arterial hypertension in France: results from a national registry. Am. J. Respir. Crit. Care Med. 2006; 173 (9): 1023–1030. DOI: 10.1164/rccm.200510-1668OC.Humbert M., Sitbon O., Chaouat A. et al. Pulmonary arterial hypertension in France: results from a national registry. Am. J. Respir. Crit. Care Med. 2006; 173 (9): 1023–1030. DOI: 10.1164/rccm.200510-1668OC.Peacock A.J., Murphy N.F., McMurray J.J.V. et al. An epidemiological study of pulmonary arterial hypertension. Eur. Respir. J. 2007; 30 (1): 104–109. DOI: 10.1183/09031936.00092306.Peacock A.J., Murphy N.F., McMurray J.J.V. et al. An epidemiological study of pulmonary arterial hypertension. Eur. Respir. J. 2007; 30 (1): 104–109. DOI: 10.1183/09031936.00092306.McGoon M.D., Benza R.L., Escribano-Subias P. et al. Pulmonary arterial hypertension: epidemiology and registries. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D51–D59.McGoon M.D., Benza R.L., Escribano-Subias P. et al. Pulmonary arterial hypertension: epidemiology and registries. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D51–D59.Simonneau G., Robbins I., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2009; 54 (Suppl.): S43–S54.Simonneau G., Robbins I., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J. Am. Coll. Cardiol. 2009; 54 (Suppl.): S43–S54.Montani D., Bergot E., Günther S. et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation. 2012; 125 (17): 2128–2137. DOI: 10.1161/CIRCULATIONAHA.111.079921.Montani D., Bergot E., Günther S. et al. Pulmonary arterial hypertension in patients treated by dasatinib. Circulation. 2012; 125 (17): 2128–2137. DOI: 10.1161/CIRCULATIONAHA.111.079921.Savale L., Chaumais M.C., Cottin V. et al. Pulmonary hypertension associated with benfluorex exposure. Eur. Respir. J. 2012; 40 (5): 1164–1172. DOI: 10.1183/09031936.00188611.Savale L., Chaumais M.C., Cottin V. et al. Pulmonary hypertension associated with benfluorex exposure. Eur. Respir. J. 2012; 40 (5): 1164–1172. DOI: 10.1183/09031936.00188611.Savale L., Sattler C., Gunther S. et al. Pulmonary arterial hypertension in patients treated with interferon. Eur. Respir. J. 2014; 44 (6): 1627–1634. DOI: 10.1183/09031936.00057914.Savale L., Sattler C., Gunther S. et al. Pulmonary arterial hypertension in patients treated with interferon. Eur. Respir. J. 2014; 44 (6): 1627–1634. DOI: 10.1183/09031936.00057914.Badesch B.D., Champion H.C., Gomez-Sanchez M.A. et al. Diagnosis and assessment of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2009; 54 (Suppl.): S55–S56.Badesch B.D., Champion H.C., Gomez-Sanchez M.A. et al. Diagnosis and assessment of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2009; 54 (Suppl.): S55–S56.Oudiz R.J. Pulmonary hypertension associated with left-sided heart disease. Clin. Chest Med. 2007; 28 (1): 233–241. DOI: 10.1016/j.ccm.2006.12.001.Oudiz R.J. Pulmonary hypertension associated with left-sided heart disease. Clin. Chest Med. 2007; 28 (1): 233–241. DOI: 10.1016/j.ccm.2006.12.001.Vahanian A., Alfieri O., Andreotti F. et al. Guidelines on the management of valvular heart disease (version 2012). Eur. Heart J. 2012; 33 (19): 2451–2496. DOI: 10.1093/eurheartj/ehs109.Vahanian A., Alfieri O., Andreotti F. et al. Guidelines on the management of valvular heart disease (version 2012). Eur. Heart J. 2012; 33 (19): 2451–2496. DOI: 10.1093/eurheartj/ehs109.Seeger W., Adir Y., Barberà J.A. et al. Pulmonary hypertension in chronic lung diseases. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D109–D116.Seeger W., Adir Y., Barberà J.A. et al. Pulmonary hypertension in chronic lung diseases. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D109–D116.Hurdman J., Condliffe R., Elliot C.A. et al. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur. Respir. J. 2013; 41 (6): 1292–1301. DOI: 10.1183/09031936.00079512.Hurdman J., Condliffe R., Elliot C.A. et al. Pulmonary hypertension in COPD: results from the ASPIRE registry. Eur. Respir. J. 2013; 41 (6): 1292–1301. DOI: 10.1183/09031936.00079512.Cottin V., Nunes H., Brillet P.Y. et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur. Respir. J. 2005; 26 (4): 586–593. DOI: 10.1183/09031936.05.00021005.Cottin V., Nunes H., Brillet P.Y. et al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur. Respir. J. 2005; 26 (4): 586–593. DOI: 10.1183/09031936.05.00021005.Escribano-Subias P., Blanco I., Lopez-Meseguer M. et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur. Respir. J. 2012; 40 (3): 596–603. DOI: 10.1183/09031936.00101211.Escribano-Subias P., Blanco I., Lopez-Meseguer M. et al. Survival in pulmonary hypertension in Spain: insights from the Spanish registry. Eur. Respir. J. 2012; 40 (3): 596–603. DOI: 10.1183/09031936.00101211.Pengo V., Lensing A.W., Prins M.H. et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N. Engl. J. Med. 2004; 350 (22): 2257–2264. DOI: 10.1056/NEJMoa032274.Pengo V., Lensing A.W., Prins M.H. et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N. Engl. J. Med. 2004; 350 (22): 2257–2264. DOI: 10.1056/NEJMoa032274.Pepke-Zaba J., Delcroix M., Lang I. et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011; 124 (18): 1973–1981. DOI: 10.1161/CIRCULATIONAHA.110.015008.Pepke-Zaba J., Delcroix M., Lang I. et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation. 2011; 124 (18): 1973–1981. DOI: 10.1161/CIRCULATIONAHA.110.015008.Soubrier F., Chung W.K., Machado R. et al. Genetics and genomics of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D13–D21.Soubrier F., Chung W.K., Machado R. et al. Genetics and genomics of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D13–D21.Ma L., Roman-Campos D., Austin E.D. et al. A novel channelopathy in pulmonary arterial hypertension. N. Engl. J. Med. 2013; 369 (4): 351–361. DOI: 10.1056/NEJMoa1211097.Ma L., Roman-Campos D., Austin E.D. et al. A novel channelopathy in pulmonary arterial hypertension. N. Engl. J. Med. 2013; 369 (4): 351–361. DOI: 10.1056/NEJMoa1211097.Eyries M., Montani D., Girerd B. et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat. Genet. 2014; 46 (1): 65–69. DOI: 10.1038/ng.2844.Eyries M., Montani D., Girerd B. et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat. Genet. 2014; 46 (1): 65–69. DOI: 10.1038/ng.2844.Eddahibi S., Chaouat A., Morrell N. et al. Polymorphism of the serotonin transporter gene and pulmonary hypertension in chronic obstructive pulmonary disease. Circulation. 2003; 108 (15): 1839–1844. DOI: 10.1161/01.CIR.0000091409.53101.E8.Eddahibi S., Chaouat A., Morrell N. et al. Polymorphism of the serotonin transporter gene and pulmonary hypertension in chronic obstructive pulmonary disease. Circulation. 2003; 108 (15): 1839–1844. DOI: 10.1161/01.CIR.0000091409.53101.E8.Bonderman D., Wexberg P., Martischnig A.M. et al. A noninvasive algorithm to exclude pre-capillary pulmonary hypertension. Eur. Respir. J. 2011; 37 (5): 1096–1103. DOI: 10.1183/09031936.00089610.Bonderman D., Wexberg P., Martischnig A.M. et al. A noninvasive algorithm to exclude pre-capillary pulmonary hypertension. Eur. Respir. J. 2011; 37 (5): 1096–1103. DOI: 10.1183/09031936.00089610.Rich J.D., Thenappan T., Freed B. et al. QTc prolongation is associated with impaired right ventricular function and predicts mortality in pulmonary hypertension. Int. J. Cardiol. 2013; 167 (3): 669–676. DOI: 10.1016/j.ijcard.2012.03.071.Rich J.D., Thenappan T., Freed B. et al. QTc prolongation is associated with impaired right ventricular function and predicts mortality in pulmonary hypertension. Int. J. Cardiol. 2013; 167 (3): 669–676. DOI: 10.1016/j.ijcard.2012.03.071.Sun P.Y., Jiang X., Gomberg-Maitland M. et al. Prolonged QRS duration: a new predictor of adverse outcome in idiopathic pulmonary arterial hypertension. Chest. 2012; 141: 374–380.Sun P.Y., Jiang X., Gomberg-Maitland M. et al. Prolonged QRS duration: a new predictor of adverse outcome in idiopathic pulmonary arterial hypertension. Chest. 2012; 141: 374–380.Olsson K.M., Nickel N.P., Tongers J. et al. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int. J. Cardiol. 2013; 167 (5): 2300–2305. DOI: 10.1016/j.ijcard.2012.06.024.Olsson K.M., Nickel N.P., Tongers J. et al. Atrial flutter and fibrillation in patients with pulmonary hypertension. Int. J. Cardiol. 2013; 167 (5): 2300–2305. DOI: 10.1016/j.ijcard.2012.06.024.Rich S., Dantzker D.R., Ayres S.M. et al. Primary pulmonary hypertension: a national prospective study. Ann. Intern. Med. 1987; 107 (2): 216–223.Rich S., Dantzker D.R., Ayres S.M. et al. Primary pulmonary hypertension: a national prospective study. Ann. Intern. Med. 1987; 107 (2): 216–223.Milne E.N. Forgotten gold in diagnosing pulmonary hypertension: the plain chest radiograph. Radiographics. 2012; 32 (4): 1085–1087. DOI: 10.1148/rg.324125021.Milne E.N. Forgotten gold in diagnosing pulmonary hypertension: the plain chest radiograph. Radiographics. 2012; 32 (4): 1085–1087. DOI: 10.1148/rg.324125021.Trip P., Nossent E.J., de Man F.S. et al. Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses. Eur. Respir. J. 2013; 42 (6): 1575–1585. DOI: 10.1183/09031936.00184412.Trip P., Nossent E.J., de Man F.S. et al. Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses. Eur. Respir. J. 2013; 42 (6): 1575–1585. DOI: 10.1183/09031936.00184412.Sun X.G., Hansen J.E., Oudiz R.J. et al. Pulmonary function in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2003; 41 (6): 1028–1035.Sun X.G., Hansen J.E., Oudiz R.J. et al. Pulmonary function in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2003; 41 (6): 1028–1035.Hoeper M.M., Pletz M.W., Golpon H. et al. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur. Respir. J. 2007; 29 (5): 944–950. DOI: 10.1183/09031936.00134506.Hoeper M.M., Pletz M.W., Golpon H. et al. Prognostic value of blood gas analyses in patients with idiopathic pulmonary arterial hypertension. Eur. Respir. J. 2007; 29 (5): 944–950. DOI: 10.1183/09031936.00134506.Pellegrino R., Viegi G., Brusasco V. et al. Interpretative strategies for lung function tests. Eur. Respir. J. 2005; 26 (5): 948–968. DOI: 10.1183/09031936.05.00035205.Pellegrino R., Viegi G., Brusasco V. et al. Interpretative strategies for lung function tests. Eur. Respir. J. 2005; 26 (5): 948–968. DOI: 10.1183/09031936.05.00035205.Holverda S., Bogaard H.J., Groepenhoff H. et al. Cardiopulmonary exercise test characteristics in patients with chronic obstructive pulmonary disease and associated pulmonary hypertension. Respiration. 2008; 76 (2): 160–167. DOI: 10.1159/000110207.Holverda S., Bogaard H.J., Groepenhoff H. et al. Cardiopulmonary exercise test characteristics in patients with chronic obstructive pulmonary disease and associated pulmonary hypertension. Respiration. 2008; 76 (2): 160–167. DOI: 10.1159/000110207.Jilwan F.N., Escourrou P., Garcia G. et al. High occurrence of hypoxemic sleep respiratory disorders in precapillary pulmonary hypertension and mechanisms. Chest. 2013; 143 (1): 47–55. DOI: 10.1378/chest.11-3124.Jilwan F.N., Escourrou P., Garcia G. et al. High occurrence of hypoxemic sleep respiratory disorders in precapillary pulmonary hypertension and mechanisms. Chest. 2013; 143 (1): 47–55. DOI: 10.1378/chest.11-3124.Rafanan A.L., Golish J.A., Dinner D.S. et al. Nocturnal hypoxemia is common in primary pulmonary hypertension. Chest. 2001; 120 (3): 894–899.Rafanan A.L., Golish J.A., Dinner D.S. et al. Nocturnal hypoxemia is common in primary pulmonary hypertension. Chest. 2001; 120 (3): 894–899.Rudski L.G., Lai W.W., Afilalo J. et al. 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 (7): 685–713. DOI: 10.1016/j.echo.2010.05.010.Rudski L.G., Lai W.W., Afilalo J. et al. 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 (7): 685–713. DOI: 10.1016/j.echo.2010.05.010.Lang R.M., Badano L.P., Mor-Avi V. et al. 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 (3): 233–270. DOI: 10.1093/ehjci/jev014.Lang R.M., Badano L.P., Mor-Avi V. et al. 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 (3): 233–270. DOI: 10.1093/ehjci/jev014.Foale R., Nihoyannopoulos P., McKenna W. et al. Echocardiographic measurement of the normal adult right ventricle. Br. Heart J. 1986; 56 (1): 33–44.Foale R., Nihoyannopoulos P., McKenna W. et al. Echocardiographic measurement of the normal adult right ventricle. Br. Heart J. 1986; 56 (1): 33–44.Hachulla E., Gressin V., Guillevin L. et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005; 52 (12): 3792–3800. DOI: 10.1002/art.21433.Hachulla E., Gressin V., Guillevin L. et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum. 2005; 52 (12): 3792–3800. DOI: 10.1002/art.21433.Tunariu N., Gibbs S.J.R., Win Z. et al. 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 (5): 680–684. DOI: 10.2967/jnumed.106.039438.Tunariu N., Gibbs S.J.R., Win Z. et al. 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 (5): 680–684. DOI: 10.2967/jnumed.106.039438.Meng J.J., Zhang L.J., Wang Q. et al. 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 (3): 177–181.Meng J.J., Zhang L.J., Wang Q. et al. 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 (3): 177–181.Rajaram S., Swift A.J., Telfer A. et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax. 2013; 68 (7): 677–678. DOI: 10.1136/thoraxjnl-2012-203020.Rajaram S., Swift A.J., Telfer A. et al. 3D contrast-enhanced lung perfusion MRI is an effective screening tool for chronic thromboembolic pulmonary hypertension: results from the ASPIRE Registry. Thorax. 2013; 68 (7): 677–678. DOI: 10.1136/thoraxjnl-2012-203020.Rajaram S., Swift A.J., Condliffe R. et al. CT features of pulmonary arterial hypertension and its major subtypes: a systematic CT evaluation of 292 patients from the ASPIRE Registry. Thorax. 2015; 70 (4): 382–387. DOI: 10.1136/thoraxjnl-2014-206088.Rajaram S., Swift A.J., Condliffe R. et al. CT features of pulmonary arterial hypertension and its major subtypes: a systematic CT evaluation of 292 patients from the ASPIRE Registry. Thorax. 2015; 70 (4): 382–387. DOI: 10.1136/thoraxjnl-2014-206088.Shen Y., Wan C., Tian P. et al. CT-base pulmonary artery measurement in the detection of pulmonary hypertension: a meta-analysis and systematic review. Medicine (Baltimore). 2014; 93 (27): e256. DOI: 10.1097/MD.0000000000000256.Shen Y., Wan C., Tian P. et al. CT-base pulmonary artery measurement in the detection of pulmonary hypertension: a meta-analysis and systematic review. Medicine (Baltimore). 2014; 93 (27): e256. DOI: 10.1097/MD.0000000000000256.Tan R.T., Kuzo R., Goodman L.R. et al. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Chest. 1998; 113 (5): 1250–1256.Tan R.T., Kuzo R., Goodman L.R. et al. Utility of CT scan evaluation for predicting pulmonary hypertension in patients with parenchymal lung disease. Chest. 1998; 113 (5): 1250–1256.Resten A., Maitre S., Humbert M. et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am. J. Roentgenol. 2004; 183 (1): 65–70. DOI: 10.2214/ajr.183.1.1830065.Resten A., Maitre S., Humbert M. et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. Am. J. Roentgenol. 2004; 183 (1): 65–70. DOI: 10.2214/ajr.183.1.1830065.Dartevelle P., Fadel E., Mussot S. et al. Chronic thromboembolic pulmonary hypertension. Eur. Respir. J. 2004; 23 (4): 637–648.Dartevelle P., Fadel E., Mussot S. et al. Chronic thromboembolic pulmonary hypertension. Eur. Respir. J. 2004; 23 (4): 637–648.Reichelt A., Hoeper M.M., Galanski M. et al. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur. J. Radiol. 2008; 71 (1): 49–54. DOI: 10.1016/j.ejrad.2008.03.016.Reichelt A., Hoeper M.M., Galanski M. et al. Chronic thromboembolic pulmonary hypertension: evaluation with 64-detector row CT versus digital substraction angiography. Eur. J. Radiol. 2008; 71 (1): 49–54. DOI: 10.1016/j.ejrad.2008.03.016.Fedullo P.F., Auger W.R., Kerr K.M. et al. Chronic thromboembolic pulmonary hypertension. N. Engl. J. Med. 2001; 345 (20): 1465–1472. DOI: 10.1056/NEJMra010902.Fedullo P.F., Auger W.R., Kerr K.M. et al. Chronic thromboembolic pulmonary hypertension. N. Engl. J. Med. 2001; 345 (20): 1465–1472. DOI: 10.1056/NEJMra010902.Fukui S., Ogo T., Morita Y. et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur. Respir. J. 2014; 43 (5): 1394–1402. DOI: 10.1183/09031936.00012914.Fukui S., Ogo T., Morita Y. et al. Right ventricular reverse remodelling after balloon pulmonary angioplasty. Eur. Respir. J. 2014; 43 (5): 1394–1402. DOI: 10.1183/09031936.00012914.Castaner E., Alguersuari A., Andreu M. et al. Imaging findings in pulmonary vasculitis. Semin. Ultrasound. CT MR. 2012; 33 (6): 567–579. DOI: 10.1053/j.sult.2012.05.001.Castaner E., Alguersuari A., Andreu M. et al. Imaging findings in pulmonary vasculitis. Semin. Ultrasound. CT MR. 2012; 33 (6): 567–579. DOI: 10.1053/j.sult.2012.05.001.Nawaz A., Litt H.I., Stavropoulos S.W. et al. Digital subtraction pulmonary arteriography versus multidetector CT in the detection of pulmonary arteriovenous malformations. J. Vasc. Interv. Radiol. 2008; 19 (11): 1582–1588. DOI: 10.1016/j.jvir.2008.07.011.Nawaz A., Litt H.I., Stavropoulos S.W. et al. Digital subtraction pulmonary arteriography versus multidetector CT in the detection of pulmonary arteriovenous malformations. J. Vasc. Interv. Radiol. 2008; 19 (11): 1582–1588. DOI: 10.1016/j.jvir.2008.07.011.Peacock A.J., Vonk Noordegraaf A. Cardiac magnetic resonance imaging in pulmonary arterial hypertension. Eur. Respir. Rev. 2013; 22 (130): 526–534. DOI: 10.1183/09059180.00006313.Peacock A.J., Vonk Noordegraaf A. Cardiac magnetic resonance imaging in pulmonary arterial hypertension. Eur. Respir. Rev. 2013; 22 (130): 526–534. DOI: 10.1183/09059180.00006313.Swift A.J., Rajaram S., Condliffe R. et al. 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. DOI: 10.1186/1532-429X-14-40.Swift A.J., Rajaram S., Condliffe R. et al. 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. DOI: 10.1186/1532-429X-14-40.Swift A.J., Rajaram S., Hurdman J. et al. 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 (10): 1036–1047. DOI: 10.1016/j.jcmg.2013.01.013.Swift A.J., Rajaram S., Hurdman J. et al. 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 (10): 1036–1047. DOI: 10.1016/j.jcmg.2013.01.013.Ley S., Kauczor H.U., Heussel C.P. et al. Value of contrast-enhanced MR angiography and helical CT angiography in chronic thromboembolic pulmonary hypertension. Eur. Radiol. 2003; 13 (10): 2365–2371. DOI: 10.1007/s00330-003-1878-8.Ley S., Kauczor H.U., Heussel C.P. et al. Value of contrast-enhanced MR angiography and helical CT angiography in chronic thromboembolic pulmonary hypertension. Eur. Radiol. 2003; 13 (10): 2365–2371. DOI: 10.1007/s00330-003-1878-8.van Wolferen S.A., Marcus J.T., Boonstra A. et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur. Heart J. 2007; 28 (10): 1250–1257. DOI: 10.1093/eurheartj/ehl477.van Wolferen S.A., Marcus J.T., Boonstra A. et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur. Heart J. 2007; 28 (10): 1250–1257. DOI: 10.1093/eurheartj/ehl477.Peacock A.J., Crawley S., McLure L. et al. 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 (1): 107–114. DOI: 10.1161/CIRCIMAGING.113.000629.Peacock A.J., Crawley S., McLure L. et al. 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 (1): 107–114. DOI: 10.1161/CIRCIMAGING.113.000629.van de Veerdonk M.C., Kind T., Marcus J.T. et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J. Am. Coll. Cardiol. 2011; 58 (24): 2511–2519. DOI: 10.1016/j.jacc.2011.06.068.van de Veerdonk M.C., Kind T., Marcus J.T. et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J. Am. Coll. Cardiol. 2011; 58 (24): 2511–2519. DOI: 10.1016/j.jacc.2011.06.068.Albrecht T., Blomley M.J., Cosgrove D.O. et al. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet. 1999; 353 (9164): 1579–1583. DOI: 10.1016/S0140-6736(98)06373-9.Albrecht T., Blomley M.J., Cosgrove D.O. et al. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet. 1999; 353 (9164): 1579–1583. DOI: 10.1016/S0140-6736(98)06373-9.Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. Swiss. Med. Wkly. 2003; 133 (11-12): 163–169. DOI: 2003/11/smw-10016.Naeije R. Hepatopulmonary syndrome and portopulmonary hypertension. Swiss. Med. Wkly. 2003; 133 (11-12): 163–169. DOI: 2003/11/smw-10016.Hoeper M.M., Lee S.H., Voswinckel R. et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J. Am. Coll. Cardiol. 2006; 48 (12): 2546–2552. DOI: 10.1016/j.jacc.2006.07.061.Hoeper M.M., Lee S.H., Voswinckel R. et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J. Am. Coll. Cardiol. 2006; 48 (12): 2546–2552. DOI: 10.1016/j.jacc.2006.07.061.Kovacs G., Avian A., Pienn M. et al. 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.Kovacs G., Avian A., Pienn M. et al. 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.Hoeper M.M., Maier R., Tongers J. et al. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1999; 160 (2): 535–541. DOI: 10.1164/ajrccm.160.2.9811062.Hoeper M.M., Maier R., Tongers J. et al. Determination of cardiac output by the Fick method, thermodilution, and acetylene rebreathing in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1999; 160 (2): 535–541. DOI: 10.1164/ajrccm.160.2.9811062.Frost A.E., Farber H.W., Barst R.J. et al. Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16 to 18 mm Hg: insights from the REVEAL Registry. Chest. 2013; 143 (1): 185–195. DOI: 10.1378/chest.11-1387.Frost A.E., Farber H.W., Barst R.J. et al. Demographics and outcomes of patients diagnosed with pulmonary hypertension with pulmonary capillary wedge pressures 16 to 18 mm Hg: insights from the REVEAL Registry. Chest. 2013; 143 (1): 185–195. DOI: 10.1378/chest.11-1387.Abraham W.T., Adamson P.B., Bourge R.C. et al. Wireless pulmonary artery haemodynamic monitoring in chronic heart failure: a randomised controlled trial. Lancet. 2011; 377 (9766): 658–666. DOI: 10.1016/S0140-6736(11)60101-3.Abraham W.T., Adamson P.B., Bourge R.C. et al. Wireless pulmonary artery haemodynamic monitoring in chronic heart failure: a randomised controlled trial. Lancet. 2011; 377 (9766): 658–666. DOI: 10.1016/S0140-6736(11)60101-3.Prasad A., Hastings J.L., Shibata S. et al. Characterization of static and dynamic left ventricular diastolic function in patients with heart failure with a preserved ejection fraction. Circ. Heart Fail. 2010; 3 (5): 617–626. DOI: 10.1161/CIRCHEARTFAILURE.109.867044.Prasad A., Hastings J.L., Shibata S. et al. Characterization of static and dynamic left ventricular diastolic function in patients with heart failure with a preserved ejection fraction. Circ. Heart Fail. 2010; 3 (5): 617–626. DOI: 10.1161/CIRCHEARTFAILURE.109.867044.Fujimoto N., Borlaug B.A., Lewis G.D. et al. Hemodynamic responses to rapid saline loading: the impact of age, sex, and heart failure. Circulation. 2013; 127 (1): 55–62. DOI: 10.1161/CIRCULATIONAHA.112.111302.Fujimoto N., Borlaug B.A., Lewis G.D. et al. Hemodynamic responses to rapid saline loading: the impact of age, sex, and heart failure. Circulation. 2013; 127 (1): 55–62. DOI: 10.1161/CIRCULATIONAHA.112.111302.Fox B.D., Shimony A., Langleben D. et al. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur. Respir. J. 2013; 42 (4): 1083–1091. DOI: 10.1183/09031936.00091212.Fox B.D., Shimony A., Langleben D. et al. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur. Respir. J. 2013; 42 (4): 1083–1091. DOI: 10.1183/09031936.00091212.Robbins I.M., Hemnes A.R., Pugh M.E. et al. High prevalence of occult pulmonary venous hypertension revealed by fluid challenge in pulmonary hypertension. Circ. Heart Fail. 2014; 7 (1): 116–122. DOI: 10.1161/CIRCHEARTFAILURE.113.000468.Robbins I.M., Hemnes A.R., Pugh M.E. et al. High prevalence of occult pulmonary venous hypertension revealed by fluid challenge in pulmonary hypertension. Circ. Heart Fail. 2014; 7 (1): 116–122. DOI: 10.1161/CIRCHEARTFAILURE.113.000468.Borlaug B.A., Nishimura R.A., Sorajja P. et al. Exercise hemodynamics enhance diagnosis of early heart failure with preserved ejection fraction. Circ. Heart Fail. 2010; 3 (5): 588–595. DOI: 10.1161/CIRCHEARTFAILURE.109.930701.Borlaug B.A., Nishimura R.A., Sorajja P. et al. Exercise hemodynamics enhance diagnosis of early heart failure with preserved ejection fraction. Circ. Heart Fail. 2010; 3 (5): 588–595. DOI: 10.1161/CIRCHEARTFAILURE.109.930701.Hager W.D., Collins I., Tate J.P. et al. Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients. Clin. Respir. J. 2013; 7 (3): 227–236. DOI: 10.1111/j.1752-699X.2012.00310.x.Hager W.D., Collins I., Tate J.P. et al. Exercise during cardiac catheterization distinguishes between pulmonary and left ventricular causes of dyspnea in systemic sclerosis patients. Clin. Respir. J. 2013; 7 (3): 227–236. DOI: 10.1111/j.1752-699X.2012.00310.x.Halpern S.D., Taichman D.B. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest. 2009; 136 (1): 37–43. DOI: 10.1378/chest.08-2784.Halpern S.D., Taichman D.B. Misclassification of pulmonary hypertension due to reliance on pulmonary capillary wedge pressure rather than left ventricular end-diastolic pressure. Chest. 2009; 136 (1): 37–43. DOI: 10.1378/chest.08-2784.Naeije R., Vachiery J.L., Yerly P. et al. The transpulmonary pressure gradient for the diagnosis of pulmonary vascular disease. Eur. Respir. J. 2013; 41 (1): 217–223. DOI: 10.1183/09031936.00074312.Naeije R., Vachiery J.L., Yerly P. et al. The transpulmonary pressure gradient for the diagnosis of pulmonary vascular disease. Eur. Respir. J. 2013; 41 (1): 217–223. DOI: 10.1183/09031936.00074312.Provencher S., Herve P., Sitbon O. et al. Changes in exercise haemodynamics during treatment in pulmonary arterial hypertension. Eur. Respir. J. 2008; 32 (2): 393–398. DOI: 10.1183/09031936.00009008.Provencher S., Herve P., Sitbon O. et al. Changes in exercise haemodynamics during treatment in pulmonary arterial hypertension. Eur. Respir. J. 2008; 32 (2): 393–398. DOI: 10.1183/09031936.00009008.Tedford R.J., Beaty C.A., Mathai S.C. et al. 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. DOI: 10.1056/NEJM199207093270203.Tedford R.J., Beaty C.A., Mathai S.C. et al. 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. DOI: 10.1056/NEJM199207093270203.Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension [see comments]. N. Engl. J. Med. 1992; 327 (2): 76–81.Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension [see comments]. N. Engl. J. Med. 1992; 327 (2): 76–81.Sitbon O., Humbert M., Jaïs X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111 (23): 3105–3111. DOI: 10.1161/CIRCULATIONAHA.104.488486.Sitbon O., Humbert M., Jaïs X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111 (23): 3105–3111. DOI: 10.1161/CIRCULATIONAHA.104.488486.Barst R., McGoon M., Torbicki A. et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2004; 43 (Suppl. 1): S40–S47.Barst R., McGoon M., Torbicki A. et al. Diagnosis and differential assessment of pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2004; 43 (Suppl. 1): S40–S47.Morgan J.M., McCormack D.G., Griffiths M.J. et al. Adenosine as a vasodilator in primary pulmonary hypertension [see comments]. Circulation. 1991; 84 (83): 1145–1149.Morgan J.M., McCormack D.G., Griffiths M.J. et al. Adenosine as a vasodilator in primary pulmonary hypertension [see comments]. Circulation. 1991; 84 (83): 1145–1149.Nootens M., Schrader B., Kaufmann E. et al. Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension. Chest. 1995; 107 (1): 54–57.Nootens M., Schrader B., Kaufmann E. et al. Comparative acute effects of adenosine and prostacyclin in primary pulmonary hypertension. Chest. 1995; 107 (1): 54–57.Hoeper M.M., Olschewski H., Ghofrani H.A. et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2000; 35 (1): 176–182.Hoeper M.M., Olschewski H., Ghofrani H.A. et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2000; 35 (1): 176–182.Opitz C.F., Wensel R., Bettmann M. et al. 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.Opitz C.F., Wensel R., Bettmann M. et al. 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.Kim N.H., Delcroix M., Jenkins D.P. et al. Chronic thromboembolic pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D92–D99. DOI: 10.1016/j.jacc.2013.10.024.Kim N.H., Delcroix M., Jenkins D.P. et al. Chronic thromboembolic pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D92–D99. DOI: 10.1016/j.jacc.2013.10.024.Montani D., Price L.C., Dorfmuller P. et al. Pulmonary veno-occlusive disease. Eur. Respir. J. 2009; 33 (1): 189–200. DOI: 10.1183/09031936.00090608.Montani D., Price L.C., Dorfmuller P. et al. Pulmonary veno-occlusive disease. Eur. Respir. J. 2009; 33 (1): 189–200. DOI: 10.1183/09031936.00090608.He J., Fang W., Lv B. et al. 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 (5): 459–463. DOI: 10.1097/MNM.0b013e32835085d9.He J., Fang W., Lv B. et al. 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 (5): 459–463. DOI: 10.1097/MNM.0b013e32835085d9.Cottin V., Le Pavec J., Prevot G. et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur. Respir. J. 2010; 35 (1): 105–111. DOI: 10.1183/09031936.00038709.Cottin V., Le Pavec J., Prevot G. et al. Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome. Eur. Respir. J. 2010; 35 (1): 105–111. DOI: 10.1183/09031936.00038709.Taichman D.B., McGoon M.D., Harhay M.O. et al. 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 (7): 586–592. DOI: 10.1016/S0025-6196(11)60747-7.Taichman D.B., McGoon M.D., Harhay M.O. et al. 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 (7): 586–592. DOI: 10.1016/S0025-6196(11)60747-7.Sitbon O., Humbert M., Nunes H. et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J. Am. Coll. Cardiol. 2002; 40 (4): 780–788.Sitbon O., Humbert M., Nunes H. et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J. Am. Coll. Cardiol. 2002; 40 (4): 780–788.Nickel N., Golpon H., Greer M. et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur. Respir. J. 2012; 39 (3): 589–596. DOI: 10.1183/09031936.00092311.Nickel N., Golpon H., Greer M. et al. The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension. Eur. Respir. J. 2012; 39 (3): 589–596. DOI: 10.1183/09031936.00092311.Barst R.J., Chung L., Zamanian R.T. et al. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest. 2013; 144 (1): 160–168. DOI: 10.1378/chest.12-2417.Barst R.J., Chung L., Zamanian R.T. et al. Functional class improvement and 3-year survival outcomes in patients with pulmonary arterial hypertension in the REVEAL Registry. Chest. 2013; 144 (1): 160–168. DOI: 10.1378/chest.12-2417.Benza R.L., Miller D.P., Gomberg-Maitland M. et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010; 122 (2): 164–172. DOI: 10.1161/CIRCULATIONAHA.109.898122.Benza R.L., Miller D.P., Gomberg-Maitland M. et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation. 2010; 122 (2): 164–172. DOI: 10.1161/CIRCULATIONAHA.109.898122.McLaughlin V.V., Sitbon O., Badesch D.B. et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur. Respir. J. 2005; 25 (2): 244–249. DOI: 10.1183/09031936.05.00054804.McLaughlin V.V., Sitbon O., Badesch D.B. et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur. Respir. J. 2005; 25 (2): 244–249. DOI: 10.1183/09031936.05.00054804.Sachdev A., Villarraga H.R., Frantz R.P. et al. Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension. Chest. 2011; 139 (6): 1299–1309. DOI: 10.1378/chest.10-2015.Sachdev A., Villarraga H.R., Frantz R.P. et al. Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension. Chest. 2011; 139 (6): 1299–1309. DOI: 10.1378/chest.10-2015.Raymond R.J., Hinderliter A.L., Willis P.W. et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2002; 39 (7): 1214–1219.Raymond R.J., Hinderliter A.L., Willis P.W. et al. Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension. J. Am. Coll. Cardiol. 2002; 39 (7): 1214–1219.Bustamante-Labarta M., Perrone S., De La Fuente R.L. et al. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J. Am. Soc. Echocardiogr. 2002; 15 (10, Pt 2): 1160–1164.Bustamante-Labarta M., Perrone S., De La Fuente R.L. et al. Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension. J. Am. Soc. Echocardiogr. 2002; 15 (10, Pt 2): 1160–1164.Forfia P.R., Fisher M.R., Mathai S.C. et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 2006; 174 (9): 1034–1041. DOI: 10.1164/rccm.200604-547OC.Forfia P.R., Fisher M.R., Mathai S.C. et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am. J. Respir. Crit. Care Med. 2006; 174 (9): 1034–1041. DOI: 10.1164/rccm.200604-547OC.Thenappan T., Shah S.J., Rich S. et al. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur. Respir. J. 2010; 35 (5): 1079–1087. DOI: 10.1183/09031936.00072709.Thenappan T., Shah S.J., Rich S. et al. Survival in pulmonary arterial hypertension: a reappraisal of the NIH risk stratification equation. Eur. Respir. J. 2010; 35 (5): 1079–1087. DOI: 10.1183/09031936.00072709.Wensel R., Opitz C.F., Anker S.D. et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation. 2002; 106 (3): 319–324.Wensel R., Opitz C.F., Anker S.D. et al. Assessment of survival in patients with primary pulmonary hypertension: importance of cardiopulmonary exercise testing. Circulation. 2002; 106 (3): 319–324.McLaughlin V.V., Shillington A., Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002; 106 (12): 1477–1482.McLaughlin V.V., Shillington A., Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002; 106 (12): 1477–1482.Fine N.M., Chen L., Bastiansen P.M. et al. Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension. Circ. Cardiovasc. Imaging. 2013; 6 (5): 711–721. DOI: 10.1161/CIRCIMAGING.113.000640.Fine N.M., Chen L., Bastiansen P.M. et al. Outcome prediction by quantitative right ventricular function assessment in 575 subjects evaluated for pulmonary hypertension. Circ. Cardiovasc. Imaging. 2013; 6 (5): 711–721. DOI: 10.1161/CIRCIMAGING.113.000640.Shimada Y.J., Shiota M., Siegel R.J. et al. 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 (9): 943–953. DOI: 10.1016/j.echo.2010.06.029.Shimada Y.J., Shiota M., Siegel R.J. et al. 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 (9): 943–953. DOI: 10.1016/j.echo.2010.06.029.Smith B.C., Dobson G., Dawson D. et al. Three-dimensional speckle tracking of the right ventricle: toward optimal quantification of right ventricular dysfunction in pulmonary hypertension. J. Am. Coll. Cardiol. 2014; 64 (1): 41–51. DOI: 10.1016/j.jacc.2014.01.084.Smith B.C., Dobson G., Dawson D. et al. Three-dimensional speckle tracking of the right ventricle: toward optimal quantification of right ventricular dysfunction in pulmonary hypertension. J. Am. Coll. Cardiol. 2014; 64 (1): 41–51. DOI: 10.1016/j.jacc.2014.01.084.Grunig E., Tiede H., Enyimayew E.O. et al. Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension. Circulation. 2013; 128 (18): 2005–2015. DOI: 10.1161/CIRCULATIONAHA.113.001573.Grunig E., Tiede H., Enyimayew E.O. et al. Assessment and prognostic relevance of right ventricular contractile reserve in patients with severe pulmonary hypertension. Circulation. 2013; 128 (18): 2005–2015. DOI: 10.1161/CIRCULATIONAHA.113.001573.Swift A.J., Rajaram S., Marshall H. et al. Black blood MRI has diagnostic and prognostic value in the assessment of patients with pulmonary hypertension. Eur. Radiol. 2012; 22 (3): 695–702. DOI: 10.1007/s00330-011-2306-0.Swift A.J., Rajaram S., Marshall H. et al. Black blood MRI has diagnostic and prognostic value in the assessment of patients with pulmonary hypertension. Eur. Radiol. 2012; 22 (3): 695–702. DOI: 10.1007/s00330-011-2306-0.Swift A.J., Rajaram S., Campbell M.J. et al. Prognostic value of cardiovascular magnetic resonance imaging measurements corrected for age and sex in idiopathic pulmonary arterial hypertension. Circ. Cardiovasc. Imaging. 2014; 7 (1): 100–106. DOI: 10.1161/CIRCIMAGING.113.000338.Swift A.J., Rajaram S., Campbell M.J. et al. Prognostic value of cardiovascular magnetic resonance imaging measurements corrected for age and sex in idiopathic pulmonary arterial hypertension. Circ. Cardiovasc. Imaging. 2014; 7 (1): 100–106. DOI: 10.1161/CIRCIMAGING.113.000338.Sitbon O., McLaughlin V.V., Badesch D.B. et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax. 2005; 60 (12): 1025–1030. DOI: 10.1136/thx.2005.040618.Sitbon O., McLaughlin V.V., Badesch D.B. et al. Survival in patients with class III idiopathic pulmonary arterial hypertension treated with first line oral bosentan compared with an historical cohort of patients started on intravenous epoprostenol. Thorax. 2005; 60 (12): 1025–1030. DOI: 10.1136/thx.2005.040618.Rich J.D., Archer S.L., Rich S. Noninvasive cardiac output measurements in patients with pulmonary hypertension. Eur. Respir. J. 2013; 42 (1): 125–133. DOI: 10.1183/09031936.00102212.Rich J.D., Archer S.L., Rich S. Noninvasive cardiac output measurements in patients with pulmonary hypertension. Eur. Respir. J. 2013; 42 (1): 125–133. DOI: 10.1183/09031936.00102212.Savarese G., Paolillo S., Costanzo P. et al. 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 (13): 1192–1201. DOI: 10.1016/j.jacc.2012.01.083.Savarese G., Paolillo S., Costanzo P. et al. 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 (13): 1192–1201. DOI: 10.1016/j.jacc.2012.01.083.Gabler N.B., French B., Strom B.L. et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation. 2012; 126 (3): 349–356. DOI: 10.1161/CIRCULATIONAHA.112.105890.Gabler N.B., French B., Strom B.L. et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation. 2012; 126 (3): 349–356. DOI: 10.1161/CIRCULATIONAHA.112.105890.Fritz J.S., Blair C., Oudiz R.J. et al. Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest. 2013; 143 (2): 315–323. DOI: 10.1378/chest.12-0270.Fritz J.S., Blair C., Oudiz R.J. et al. Baseline and follow-up 6-min walk distance and brain natriuretic peptide predict 2-year mortality in pulmonary arterial hypertension. Chest. 2013; 143 (2): 315–323. DOI: 10.1378/chest.12-0270.Paciocco G., Martinez F., Bossone E. et al. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur. Respir. J. 2001; 17 (4): 647–652.Paciocco G., Martinez F., Bossone E. et al. Oxygen desaturation on the six-minute walk test and mortality in untreated primary pulmonary hypertension. Eur. Respir. J. 2001; 17 (4): 647–652.Provencher S., Chemla D., Herve P. et al. Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur. Respir. J. 2006; 27 (1): 114–120. DOI: 10.1183/09031936.06.00042705.Provencher S., Chemla D., Herve P. et al. Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension. Eur. Respir. J. 2006; 27 (1): 114–120. DOI: 10.1183/09031936.06.00042705.Sun X.G., Hansen J.E., Oudiz R.J. et al. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation. 2001; 104 (4): 429–435.Sun X.G., Hansen J.E., Oudiz R.J. et al. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation. 2001; 104 (4): 429–435.Wensel R., Francis D.P., Meyer F.J. et al. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int. J. Cardiol. 2013; 167 (4): 1193–1198. DOI: 10.1016/j.ijcard.2012.03.135.Wensel R., Francis D.P., Meyer F.J. et al. Incremental prognostic value of cardiopulmonary exercise testing and resting haemodynamics in pulmonary arterial hypertension. Int. J. Cardiol. 2013; 167 (4): 1193–1198. DOI: 10.1016/j.ijcard.2012.03.135.Blumberg F.C., Arzt M., Lange T. et al. Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension. Eur. J. Heart Fail. 2013; 15 (7): 771–775. DOI: 10.1093/eurjhf/hft044.Blumberg F.C., Arzt M., Lange T. et al. Impact of right ventricular reserve on exercise capacity and survival in patients with pulmonary hypertension. Eur. J. Heart Fail. 2013; 15 (7): 771–775. DOI: 10.1093/eurjhf/hft044.Diller G.P., Dimopoulos K., Okonko D. et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005; 112 (6): 828–835. DOI: 10.1161/CIRCULATIONAHA.104.529800.Diller G.P., Dimopoulos K., Okonko D. et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005; 112 (6): 828–835. DOI: 10.1161/CIRCULATIONAHA.104.529800.Arena R., Lavie C.J., Milani R.V. et al. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: an evidence-based review. J. Heart Lung Transplant. 2010; 29 (2): 159–173. DOI: 10.1016/j.healun.2009.09.003.Arena R., Lavie C.J., Milani R.V. et al. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: an evidence-based review. J. Heart Lung Transplant. 2010; 29 (2): 159–173. DOI: 10.1016/j.healun.2009.09.003.Pullamsetti S., Kiss L., Ghofrani H.A. et al. Increased levels and reduced catabolism of asymmetric and symmetric dimethylarginines in pulmonary hypertension. FASEB J. 2005; 19 (9): 1175–1177. DOI: 10.1096/fj.04-3223fje.Pullamsetti S., Kiss L., Ghofrani H.A. et al. Increased levels and reduced catabolism of asymmetric and symmetric dimethylarginines in pulmonary hypertension. FASEB J. 2005; 19 (9): 1175–1177. DOI: 10.1096/fj.04-3223fje.Kielstein J.T., Bode-Boger S.M., Hesse G. et al. Asymmetrical dimethylarginine in idiopathic pulmonary arterial hypertension. Arterioscler. Thromb. Vasc. Biol. 2005; 25 (7): 1414–1418. DOI: 10.1161/01.ATV.0000168414.06853.f0.Kielstein J.T., Bode-Boger S.M., Hesse G. et al. Asymmetrical dimethylarginine in idiopathic pulmonary arterial hypertension. Arterioscler. Thromb. Vasc. Biol. 2005; 25 (7): 1414–1418. DOI: 10.1161/01.ATV.0000168414.06853.f0.Kielstein J.T., Impraim B., Simmel S. et al. Cardiovascular effects of systemic nitric oxide synthase inhibition with asymmetrical dimethylarginine in humans. Circulation. 2004; 109 (2): 172–177. DOI: 10.1161/01.CIR.0000105764.22626.B1.Kielstein J.T., Impraim B., Simmel S. et al. Cardiovascular effects of systemic nitric oxide synthase inhibition with asymmetrical dimethylarginine in humans. Circulation. 2004; 109 (2): 172–177. DOI: 10.1161/01.CIR.0000105764.22626.B1.Kawut S.M., Horn E.M., Berekashvili K.K. et al. von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension. Chest. 2005; 128 (4): 2355–2362. DOI: 10.1378/chest.128.4.2355.Kawut S.M., Horn E.M., Berekashvili K.K. et al. von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension. Chest. 2005; 128 (4): 2355–2362. DOI: 10.1378/chest.128.4.2355.Kümpers P., Nickel N., Lukasz A. et al. Circulating angiopoietins in idiopathic pulmonary arterial hypertension. Eur. Heart J. 2010; 31 (18): 2291–2300. DOI: 10.1093/eurheartj/ehq226.Kümpers P., Nickel N., Lukasz A. et al. Circulating angiopoietins in idiopathic pulmonary arterial hypertension. Eur. Heart J. 2010; 31 (18): 2291–2300. DOI: 10.1093/eurheartj/ehq226.Rubens C., Ewert R., Halank M. et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001; 120 (5): 1562–1569.Rubens C., Ewert R., Halank M. et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001; 120 (5): 1562–1569.Quarck R., Nawrot T., Meyns B. et al. C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2009; 53 (14): 1211–1218. DOI: 10.1016/j.jacc.2008.12.038.Quarck R., Nawrot T., Meyns B. et al. C-reactive protein: a new predictor of adverse outcome in pulmonary arterial hypertension. J. Am. Coll. Cardiol. 2009; 53 (14): 1211–1218. DOI: 10.1016/j.jacc.2008.12.038.Balabanian K., Foussat A., Dorfmuller P. et al. CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2002; 165 (10): 1419–1425. DOI: 10.1164/rccm.2106007.Balabanian K., Foussat A., Dorfmuller P. et al. CX(3)C chemokine fractalkine in pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2002; 165 (10): 1419–1425. DOI: 10.1164/rccm.2106007.Dorfmuller P., Zarka V., Durand-Gasselin I, et al. Chemokine RANTES in severe pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2002; 165 (4): 534–539. DOI: 10.1164/ajrccm.165.4.2012112.Dorfmuller P., Zarka V., Durand-Gasselin I, et al. Chemokine RANTES in severe pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2002; 165 (4): 534–539. DOI: 10.1164/ajrccm.165.4.2012112.Humbert M., Monti G., Brenot F. et al. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1995; 151 (5): 1628–1631. DOI: 10.1164/ajrccm.151.5.7735624.Humbert M., Monti G., Brenot F. et al. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1995; 151 (5): 1628–1631. DOI: 10.1164/ajrccm.151.5.7735624.Nagaya N., Nishikimi T., Uematsu M. et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000; 102 (8): 865–870.Nagaya N., Nishikimi T., Uematsu M. et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000; 102 (8): 865–870.Leuchte H.H., El Nounou M., Tuerpe J.C. et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest. 2007; 131 (2): 402–409. DOI: 10.1378/chest.06-1758.Leuchte H.H., El Nounou M., Tuerpe J.C. et al. N-terminal pro-brain natriuretic peptide and renal insufficiency as predictors of mortality in pulmonary hypertension. Chest. 2007; 131 (2): 402–409. DOI: 10.1378/chest.06-1758.Fijalkowska A., Kurzyna M., Torbicki A. et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest. 2006; 129 (5): 1313–1321. DOI: 10.1378/chest.129.5.1313.Fijalkowska A., Kurzyna M., Torbicki A. et al. Serum N-terminal brain natriuretic peptide as a prognostic parameter in patients with pulmonary hypertension. Chest. 2006; 129 (5): 1313–1321. DOI: 10.1378/chest.129.5.1313.Torbicki A., Kurzyna M., Kuca P. et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003; 108 (7): 844–848. DOI: 10.1161/01.CIR.0000084544.54513.E2.Torbicki A., Kurzyna M., Kuca P. et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. 2003; 108 (7): 844–848. DOI: 10.1161/01.CIR.0000084544.54513.E2.Nickel N., Kempf T., Tapken H. et al. Growth differentiation factor-15 in idiopathic pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2008; 178 (5): 534–541. DOI: 10.1164/rccm.200802-235OC.Nickel N., Kempf T., Tapken H. et al. Growth differentiation factor-15 in idiopathic pulmonary arterial hypertension. Am. J. Respir. Crit. Care Med. 2008; 178 (5): 534–541. DOI: 10.1164/rccm.200802-235OC.Nagaya N., Uematsu M., Satoh T. et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1999; 160 (2): 487–492. DOI: 10.1164/ajrccm.160.2.9812078.Nagaya N., Uematsu M., Satoh T. et al. Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am. J. Respir. Crit. Care Med. 1999; 160 (2): 487–492. DOI: 10.1164/ajrccm.160.2.9812078.Lorenzen J.M., Nickel N., Kramer R. et al. Osteopontin in patients with idiopathic pulmonary hypertension. Chest. 2011; 139 (5): 1010–1017. DOI: 10.1378/chest.10-1146.Lorenzen J.M., Nickel N., Kramer R. et al. Osteopontin in patients with idiopathic pulmonary hypertension. Chest. 2011; 139 (5): 1010–1017. DOI: 10.1378/chest.10-1146.Warwick G., Thomas P.S., Yates D.H. Biomarkers in pulmonary hypertension. Eur. Respir. J. 2008; 32 (2): 503–512. DOI: 10.1183/09031936.00160307.Warwick G., Thomas P.S., Yates D.H. Biomarkers in pulmonary hypertension. Eur. Respir. J. 2008; 32 (2): 503–512. DOI: 10.1183/09031936.00160307.Hoeper M.M., Markevych I., Spiekerkoetter E. et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur. Respir. J. 2005; 26 (5): 858–863. DOI: 10.1183/09031936.05.00075305.Hoeper M.M., Markevych I., Spiekerkoetter E. et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur. Respir. J. 2005; 26 (5): 858–863. DOI: 10.1183/09031936.05.00075305.McLaughlin V.V., Gaine S.P., Howard L.S. et al. Treatment goals of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D73–D81.McLaughlin V.V., Gaine S.P., Howard L.S. et al. Treatment goals of pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25, Suppl.): D73–D81.

The authors declare that there are no conflicts of interest present.