Skip to main content
Top
Published in: Heart Failure Reviews 6/2019

01-11-2019 | Bosentan

Advances in targeted therapy for chronic thromboembolic pulmonary hypertension

Authors: Yi Zhang, Xue Yu, Qi Jin, Qin Luo, Zhihui Zhao, Qing Zhao, Lu Yan, Zhihong Liu

Published in: Heart Failure Reviews | Issue 6/2019

Login to get access

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by unresolved thrombi in the pulmonary arteries and microvasculopathy in nonoccluded areas. If left untreated, progressive pulmonary hypertension will induce right heart failure and, finally, death. Currently, pulmonary endarterectomy (PEA) remains the only method that has the potential to cure CTEPH. Unfortunately, up to 40% of patients are ineligible for this procedure for various reasons. In recent years, refined balloon pulmonary angioplasty (BPA) has become an alternative option for inoperable CTEPH patients, and it may be another curative treatment in the future, particularly in combination with prior PEA. Nevertheless, 23% of patients still suffer from persistent PH after BPA. Given that CTEPH shares many similarities with idiopathic pulmonary arterial hypertension (PAH), targeted drugs developed for PAH are also attractive options for CTEPH, especially for inoperable or persistent/recurrent CTEPH patients. To date, riociguat, macitentan, and subcutaneous treprostinil are the only drugs proven by randomized control trials to be capable of improving the exercise capacity (6-min walking distance) of CTEPH patients. In this review, we summarize the achievements and unresolved problems of PAH-targeted therapy for CTEPH over the last decade.
Literature
2.
5.
go back to reference Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M (2016) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37:67–119. https://doi.org/10.1093/eurheartj/ehv317 CrossRefPubMed Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M (2016) 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37:67–119. https://​doi.​org/​10.​1093/​eurheartj/​ehv317 CrossRefPubMed
6.
10.
go back to reference Berger G, Azzam ZS, Hardak E, Tavor Y, Yigla M (2011) Idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: can we be certain? Isr Med Assoc J 13:106–110PubMed Berger G, Azzam ZS, Hardak E, Tavor Y, Yigla M (2011) Idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: can we be certain? Isr Med Assoc J 13:106–110PubMed
13.
21.
go back to reference Jamieson SW, Kapelanski DP, Sakakibara N, Manecke GR, Thistlethwaite PA, Kerr KM, Channick RN, Fedullo PF, Auger WR (2003) Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 76:1457–1462 discussion 1462-1454 CrossRef Jamieson SW, Kapelanski DP, Sakakibara N, Manecke GR, Thistlethwaite PA, Kerr KM, Channick RN, Fedullo PF, Auger WR (2003) Pulmonary endarterectomy: experience and lessons learned in 1,500 cases. Ann Thorac Surg 76:1457–1462 discussion 1462-1454 CrossRef
23.
go back to reference Simonneau G, D’Armini AM, Ghofrani HA, Grimminger F, Jansa P, Kim NH, Mayer E, Pulido T, Wang C, Colorado P, Fritsch A, Meier C, Nikkho S, Hoeper MM (2016) Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial. Lancet Respir Med 4:372–380. https://doi.org/10.1016/s2213-2600(16)30022-4 CrossRef Simonneau G, D’Armini AM, Ghofrani HA, Grimminger F, Jansa P, Kim NH, Mayer E, Pulido T, Wang C, Colorado P, Fritsch A, Meier C, Nikkho S, Hoeper MM (2016) Predictors of long-term outcomes in patients treated with riociguat for chronic thromboembolic pulmonary hypertension: data from the CHEST-2 open-label, randomised, long-term extension trial. Lancet Respir Med 4:372–380. https://​doi.​org/​10.​1016/​s2213-2600(16)30022-4 CrossRef
25.
go back to reference Benza RL, Miller DP, Foreman AJ, Frost AE, Badesch DB, Benton WW, McGoon MD (2015) Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant 34:356–361. https://doi.org/10.1016/j.healun.2014.09.016 CrossRefPubMed Benza RL, Miller DP, Foreman AJ, Frost AE, Badesch DB, Benton WW, McGoon MD (2015) Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: a Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis. J Heart Lung Transplant 34:356–361. https://​doi.​org/​10.​1016/​j.​healun.​2014.​09.​016 CrossRefPubMed
26.
go back to reference 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 (2010) Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 122:164–172. https://doi.org/10.1161/circulationaha.109.898122 CrossRefPubMed 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 (2010) Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 122:164–172. https://​doi.​org/​10.​1161/​circulationaha.​109.​898122 CrossRefPubMed
29.
go back to reference McLaughlin VV, Jansa P, Nielsen-Kudsk JE, Halank M, Simonneau G, Grunig E, Ulrich S, Rosenkranz S, Gomez Sanchez MA, Pulido T, Pepke-Zaba J, Barbera JA, Hoeper MM, Vachiery JL, Lang I, Carvalho F, Meier C, Mueller K, Nikkho S, D’Armini AM (2017) Riociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study. BMC Pulm Med 17:216. https://doi.org/10.1186/s12890-017-0563-7 CrossRefPubMedPubMedCentral McLaughlin VV, Jansa P, Nielsen-Kudsk JE, Halank M, Simonneau G, Grunig E, Ulrich S, Rosenkranz S, Gomez Sanchez MA, Pulido T, Pepke-Zaba J, Barbera JA, Hoeper MM, Vachiery JL, Lang I, Carvalho F, Meier C, Mueller K, Nikkho S, D’Armini AM (2017) Riociguat in patients with chronic thromboembolic pulmonary hypertension: results from an early access study. BMC Pulm Med 17:216. https://​doi.​org/​10.​1186/​s12890-017-0563-7 CrossRefPubMedPubMedCentral
35.
go back to reference Delcroix M, Lang I, Pepke-Zaba J, Jansa P, D’Armini AM, Snijder R, Bresser P, Torbicki A, Mellemkjaer S, Lewczuk J, Simkova I, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Jais X, Ambroz D, Treacy C, Morsolini M, Jenkins D, Lindner J, Dartevelle P, Mayer E, Simonneau G (2016) Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 133:859–871. https://doi.org/10.1161/circulationaha.115.016522 CrossRefPubMed Delcroix M, Lang I, Pepke-Zaba J, Jansa P, D’Armini AM, Snijder R, Bresser P, Torbicki A, Mellemkjaer S, Lewczuk J, Simkova I, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Jais X, Ambroz D, Treacy C, Morsolini M, Jenkins D, Lindner J, Dartevelle P, Mayer E, Simonneau G (2016) Long-term outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry. Circulation 133:859–871. https://​doi.​org/​10.​1161/​circulationaha.​115.​016522 CrossRefPubMed
36.
go back to reference 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, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jais X, Simonneau G (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981. https://doi.org/10.1161/circulationaha.110.015008 CrossRef 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, Barbera JA, de Perrot M, Hoeper MM, Gaine S, Speich R, Gomez-Sanchez MA, Kovacs G, Hamid AM, Jais X, Simonneau G (2011) Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 124:1973–1981. https://​doi.​org/​10.​1161/​circulationaha.​110.​015008 CrossRef
37.
go back to reference Marra AM, Egenlauf B, Ehlken N, Fischer C, Eichstaedt C, Nagel C, Bossone E, Cittadini A, Halank M, Gall H, Olsson KM, Lange TJ, Grunig E (2015) Change of right heart size and function by long-term therapy with riociguat in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Int J Cardiol 195:19–26. https://doi.org/10.1016/j.ijcard.2015.05.105 CrossRef Marra AM, Egenlauf B, Ehlken N, Fischer C, Eichstaedt C, Nagel C, Bossone E, Cittadini A, Halank M, Gall H, Olsson KM, Lange TJ, Grunig E (2015) Change of right heart size and function by long-term therapy with riociguat in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Int J Cardiol 195:19–26. https://​doi.​org/​10.​1016/​j.​ijcard.​2015.​05.​105 CrossRef
38.
go back to reference Ahmadi A, Thornhill RE, Pena E, Renaud JM, Promislow S, Chandy G, Davies RA, Stewart DJ, Contreras-Dominguez V, Dunne R, Doyle-Cox C, Beanlands RS, deKemp RA, Mielniczuk LM (2018) Effects of riociguat on right ventricular remodelling in chronic thromboembolic pulmonary hypertension patients: a prospective study. Can J Cardiol 34:1137–1144. https://doi.org/10.1016/j.cjca.2018.06.007 CrossRef Ahmadi A, Thornhill RE, Pena E, Renaud JM, Promislow S, Chandy G, Davies RA, Stewart DJ, Contreras-Dominguez V, Dunne R, Doyle-Cox C, Beanlands RS, deKemp RA, Mielniczuk LM (2018) Effects of riociguat on right ventricular remodelling in chronic thromboembolic pulmonary hypertension patients: a prospective study. Can J Cardiol 34:1137–1144. https://​doi.​org/​10.​1016/​j.​cjca.​2018.​06.​007 CrossRef
40.
go back to reference Ahn HS, Foster M, Cable M, Pitts BJ, Sybertz EJ (1991) Ca/CaM-stimulated and cGMP-specific phosphodiesterases in vascular and non-vascular tissues. Adv Exp Med Biol 308:191–197CrossRef Ahn HS, Foster M, Cable M, Pitts BJ, Sybertz EJ (1991) Ca/CaM-stimulated and cGMP-specific phosphodiesterases in vascular and non-vascular tissues. Adv Exp Med Biol 308:191–197CrossRef
41.
45.
go back to reference Sekine A, Tanabe N, Sugiura T, Shigeta A, Jujo T, Nishimura R, Sakao S, Kasahara Y, Tatsumi K (2014) Polymorphism of the G protein beta3 subunit gene influences the efficacy of sildenafil in patients with pulmonary hypertension. Intern Med 53:291–297CrossRef Sekine A, Tanabe N, Sugiura T, Shigeta A, Jujo T, Nishimura R, Sakao S, Kasahara Y, Tatsumi K (2014) Polymorphism of the G protein beta3 subunit gene influences the efficacy of sildenafil in patients with pulmonary hypertension. Intern Med 53:291–297CrossRef
46.
go back to reference Nishimura R, Tanabe N, Sekine A, Kasai H, Suda R, Kato F, Jujo T, Sugiura T, Shigeta A, Sakao S, Tatsumi K (2016) Synergistic effects of ACE insertion/deletion and GNB3 C825T polymorphisms on the efficacy of PDE-5 inhibitor in patients with pulmonary hypertension. Respiration 91:132–140. https://doi.org/10.1159/000443772 CrossRefPubMed Nishimura R, Tanabe N, Sekine A, Kasai H, Suda R, Kato F, Jujo T, Sugiura T, Shigeta A, Sakao S, Tatsumi K (2016) Synergistic effects of ACE insertion/deletion and GNB3 C825T polymorphisms on the efficacy of PDE-5 inhibitor in patients with pulmonary hypertension. Respiration 91:132–140. https://​doi.​org/​10.​1159/​000443772 CrossRefPubMed
50.
go back to reference Reesink HJ, Meijer RC, Lutter R, Boomsma F, Jansen HM, Kloek JJ, Bresser P (2006) Hemodynamic and clinical correlates of endothelin-1 in chronic thromboembolic pulmonary hypertension. Circ J 70:1058–1063CrossRef Reesink HJ, Meijer RC, Lutter R, Boomsma F, Jansen HM, Kloek JJ, Bresser P (2006) Hemodynamic and clinical correlates of endothelin-1 in chronic thromboembolic pulmonary hypertension. Circ J 70:1058–1063CrossRef
51.
go back to reference Bauer M, Wilkens H, Langer F, Schneider SO, Lausberg H, Schafers HJ (2002) Selective upregulation of endothelin B receptor gene expression in severe pulmonary hypertension. Circulation 105:1034–1036CrossRef Bauer M, Wilkens H, Langer F, Schneider SO, Lausberg H, Schafers HJ (2002) Selective upregulation of endothelin B receptor gene expression in severe pulmonary hypertension. Circulation 105:1034–1036CrossRef
52.
go back to reference Jais 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 (2008) 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 52:2127–2134. https://doi.org/10.1016/j.jacc.2008.08.059 CrossRefPubMed Jais 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 (2008) 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 52:2127–2134. https://​doi.​org/​10.​1016/​j.​jacc.​2008.​08.​059 CrossRefPubMed
54.
go back to reference Post MC, Plokker HW, Kelder JC, Snijder RJ (2009) Long-term efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension. Neth Hear J 17:329–333CrossRef Post MC, Plokker HW, Kelder JC, Snijder RJ (2009) Long-term efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension. Neth Hear J 17:329–333CrossRef
55.
go back to reference Ulrich S, Speich R, Domenighetti G, Geiser T, Aubert JD, Rochat T, Huber L, Treder U, Fischler M (2007) Bosentan therapy for chronic thromboembolic pulmonary hypertension. A national open label study assessing the effect of bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (BOCTEPH-study). Swiss Med Wkly 137:573–580PubMed Ulrich S, Speich R, Domenighetti G, Geiser T, Aubert JD, Rochat T, Huber L, Treder U, Fischler M (2007) Bosentan therapy for chronic thromboembolic pulmonary hypertension. A national open label study assessing the effect of bosentan on haemodynamics, exercise capacity, quality of life, safety and tolerability in patients with chronic thromboembolic pulmonary hypertension (BOCTEPH-study). Swiss Med Wkly 137:573–580PubMed
61.
go back to reference Ghofrani HA, Simonneau G, D’Armini AM, Fedullo P, Howard LS, Jais X, Jenkins DP, Jing ZC, Madani MM, Martin N, Mayer E, Papadakis K, Richard D, Kim NH (2017) Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med 5:785–794. https://doi.org/10.1016/s2213-2600(17)30305-3 CrossRefPubMed Ghofrani HA, Simonneau G, D’Armini AM, Fedullo P, Howard LS, Jais X, Jenkins DP, Jing ZC, Madani MM, Martin N, Mayer E, Papadakis K, Richard D, Kim NH (2017) Macitentan for the treatment of inoperable chronic thromboembolic pulmonary hypertension (MERIT-1): results from the multicentre, phase 2, randomised, double-blind, placebo-controlled study. Lancet Respir Med 5:785–794. https://​doi.​org/​10.​1016/​s2213-2600(17)30305-3 CrossRefPubMed
63.
go back to reference Scelsi L, Ghio S, Campana C, D’Armini AM, Serio A, Klersy C, Piovella F, Vigano M, Tavazzi L (2004) Epoprostenol in chronic thromboembolic pulmonary hypertension with distal lesions. Ital Heart J 5:618–623PubMed Scelsi L, Ghio S, Campana C, D’Armini AM, Serio A, Klersy C, Piovella F, Vigano M, Tavazzi L (2004) Epoprostenol in chronic thromboembolic pulmonary hypertension with distal lesions. Ital Heart J 5:618–623PubMed
66.
go back to reference Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, Nikkho S, Speich R, Hoeper MM, Behr J, Winkler J, Sitbon O, Popov W, Ghofrani HA, Manes A, Kiely DG, Ewert R, Meyer A, Corris PA, Delcroix M, Gomez-Sanchez M, Siedentop H, Seeger W (2002) Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 347:322–329. https://doi.org/10.1056/NEJMoa020204 CrossRefPubMed Olschewski H, Simonneau G, Galie N, Higenbottam T, Naeije R, Rubin LJ, Nikkho S, Speich R, Hoeper MM, Behr J, Winkler J, Sitbon O, Popov W, Ghofrani HA, Manes A, Kiely DG, Ewert R, Meyer A, Corris PA, Delcroix M, Gomez-Sanchez M, Siedentop H, Seeger W (2002) Inhaled iloprost for severe pulmonary hypertension. N Engl J Med 347:322–329. https://​doi.​org/​10.​1056/​NEJMoa020204 CrossRefPubMed
72.
go back to reference Sadushi-Kolici R, Jansa P, Kopec G, Torbicki A, Skoro-Sajer N, Campean IA, Halank M, Simkova I, Karlocai K, Steringer-Mascherbauer R, Samarzija M, Salobir B, Klepetko W, Lindner J, Lang IM (2019) Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med 7:239–248. https://doi.org/10.1016/s2213-2600(18)30367-9 CrossRefPubMed Sadushi-Kolici R, Jansa P, Kopec G, Torbicki A, Skoro-Sajer N, Campean IA, Halank M, Simkova I, Karlocai K, Steringer-Mascherbauer R, Samarzija M, Salobir B, Klepetko W, Lindner J, Lang IM (2019) Subcutaneous treprostinil for the treatment of severe non-operable chronic thromboembolic pulmonary hypertension (CTREPH): a double-blind, phase 3, randomised controlled trial. Lancet Respir Med 7:239–248. https://​doi.​org/​10.​1016/​s2213-2600(18)30367-9 CrossRefPubMed
73.
go back to reference Ono F, Nagaya N, Okumura H, Shimizu Y, Kyotani S, Nakanishi N, Miyatake K (2003) Effect of orally active prostacyclin analogue on survival in patients with chronic thromboembolic pulmonary hypertension without major vessel obstruction. Chest 123:1583–1588CrossRef Ono F, Nagaya N, Okumura H, Shimizu Y, Kyotani S, Nakanishi N, Miyatake K (2003) Effect of orally active prostacyclin analogue on survival in patients with chronic thromboembolic pulmonary hypertension without major vessel obstruction. Chest 123:1583–1588CrossRef
74.
go back to reference Nagaya N, Shimizu Y, Satoh T, Oya H, Uematsu M, Kyotani S, Sakamaki F, Sato N, Nakanishi N, Miyatake K (2002) Oral beraprost sodium improves exercise capacity and ventilatory efficiency in patients with primary or thromboembolic pulmonary hypertension. Heart 87:340–345CrossRef Nagaya N, Shimizu Y, Satoh T, Oya H, Uematsu M, Kyotani S, Sakamaki F, Sato N, Nakanishi N, Miyatake K (2002) Oral beraprost sodium improves exercise capacity and ventilatory efficiency in patients with primary or thromboembolic pulmonary hypertension. Heart 87:340–345CrossRef
77.
go back to reference Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galie N, Ghofrani HA, Hoeper MM, Lang IM, Preiss R, Rubin LJ, Di Scala L, Tapson V, Adzerikho I, Liu J, Moiseeva O, Zeng X, Simonneau G, McLaughlin VV (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373:2522–2533. https://doi.org/10.1056/NEJMoa1503184 CrossRefPubMed Sitbon O, Channick R, Chin KM, Frey A, Gaine S, Galie N, Ghofrani HA, Hoeper MM, Lang IM, Preiss R, Rubin LJ, Di Scala L, Tapson V, Adzerikho I, Liu J, Moiseeva O, Zeng X, Simonneau G, McLaughlin VV (2015) Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 373:2522–2533. https://​doi.​org/​10.​1056/​NEJMoa1503184 CrossRefPubMed
80.
go back to reference Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, Seeger W, Grimminger F (2002) Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 136:515–522CrossRef Ghofrani HA, Wiedemann R, Rose F, Olschewski H, Schermuly RT, Weissmann N, Seeger W, Grimminger F (2002) Combination therapy with oral sildenafil and inhaled iloprost for severe pulmonary hypertension. Ann Intern Med 136:515–522CrossRef
81.
85.
go back to reference Nagaya N, Sasaki N, Ando M, Ogino H, Sakamaki F, Kyotani S, Nakanishi N (2003) Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 123:338–343CrossRef Nagaya N, Sasaki N, Ando M, Ogino H, Sakamaki F, Kyotani S, Nakanishi N (2003) Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Chest 123:338–343CrossRef
88.
go back to reference Kramm T, Eberle B, Krummenauer F, Guth S, Oelert H, Mayer E (2003) Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: effects before and after pulmonary thromboendarterectomy. Ann Thorac Surg 76:711–718CrossRef Kramm T, Eberle B, Krummenauer F, Guth S, Oelert H, Mayer E (2003) Inhaled iloprost in patients with chronic thromboembolic pulmonary hypertension: effects before and after pulmonary thromboendarterectomy. Ann Thorac Surg 76:711–718CrossRef
91.
go back to reference Inami T, Kataoka M, Shimura N, Ishiguro H, Yanagisawa R, Taguchi H, Fukuda K, Yoshino H, Satoh T (2013) 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 6:725–736. https://doi.org/10.1016/j.jcin.2013.03.009 CrossRefPubMed Inami T, Kataoka M, Shimura N, Ishiguro H, Yanagisawa R, Taguchi H, Fukuda K, Yoshino H, Satoh T (2013) 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 6:725–736. https://​doi.​org/​10.​1016/​j.​jcin.​2013.​03.​009 CrossRefPubMed
93.
go back to reference Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ (2001) Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation 103:10–13CrossRef Feinstein JA, Goldhaber SZ, Lock JE, Ferndandes SM, Landzberg MJ (2001) Balloon pulmonary angioplasty for treatment of chronic thromboembolic pulmonary hypertension. Circulation 103:10–13CrossRef
95.
go back to reference Sugimura K, Fukumoto Y, Satoh K, Nochioka K, Miura Y, Aoki T, Tatebe S, Miyamichi-Yamamoto S, Shimokawa H (2012) Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J 76:485–488CrossRef Sugimura K, Fukumoto Y, Satoh K, Nochioka K, Miura Y, Aoki T, Tatebe S, Miyamichi-Yamamoto S, Shimokawa H (2012) Percutaneous transluminal pulmonary angioplasty markedly improves pulmonary hemodynamics and long-term prognosis in patients with chronic thromboembolic pulmonary hypertension. Circ J 76:485–488CrossRef
96.
go back to reference Galie 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 (2008) Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 117:3010–3019. https://doi.org/10.1161/circulationaha.107.742510 CrossRefPubMed Galie 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 (2008) Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 117:3010–3019. https://​doi.​org/​10.​1161/​circulationaha.​107.​742510 CrossRefPubMed
97.
go back to reference Divers C, Platt D, Wang E, Lin J, Lingohr-Smith M, Mathai SC (2017) A review of clinical trial endpoints of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension and how they relate to patient outcomes in the United States. J Manag Care Spec Pharm 23:92–104. https://doi.org/10.18553/jmcp.2017.23.1.92 CrossRef Divers C, Platt D, Wang E, Lin J, Lingohr-Smith M, Mathai SC (2017) A review of clinical trial endpoints of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension and how they relate to patient outcomes in the United States. J Manag Care Spec Pharm 23:92–104. https://​doi.​org/​10.​18553/​jmcp.​2017.​23.​1.​92 CrossRef
Metadata
Title
Advances in targeted therapy for chronic thromboembolic pulmonary hypertension
Authors
Yi Zhang
Xue Yu
Qi Jin
Qin Luo
Zhihui Zhao
Qing Zhao
Lu Yan
Zhihong Liu
Publication date
01-11-2019

Other articles of this Issue 6/2019

Heart Failure Reviews 6/2019 Go to the issue