Download PDF
Dtsch Med Wochenschr 2014; 139(S 04): S142-S150
DOI: 10.1055/s-0034-1387489
Übersicht | Review article
Pneumologie
© Georg Thieme Verlag KG Stuttgart · New York

Gezielte Therapie der pulmonal arteriellen Hypertonie (PAH)

Targeted therapy of pulmonary arterial hypertension (PAH)
H. Klose
1   Sektion Pneumologie, Universitätsklinikum Hamburg-Eppendorf, Deutschland
,
C. Opitz
2   Klinik für Innere Medizin, Schwerpunkt Kardiologie und Angiologie, DRK Kliniken Berlin Köpenick, Deutschland
,
H. Bremer
3   Lungenzentrum Donaueschingen, Deutschland
,
R. Ewert
4   Klinik für Pneumologie, Ernst-Moritz-Arndt Universität Greifswald, Deutschland
,
D. Bonderman
5   Abt. Innere Medizin II, Kardiologie, Medizinische Universitätsklinik Wien, Wien, Österreich
,
S. Rosenkranz
6   Abt. für Kardiologie, Universitätsklinikum Köln, Deutschland
,
W. Seeger
7   Lungenzentrum, Universitätsklinikum Gießen und Marburg, Standort Gießen, Deutschland
8   Deutsches Zentrum für Lungenforschung (DZL)
,
A. Schmeißer
9   Abt. Kardiologie, Universitätsklinikum Magdeburg, Deutschland
,
L. Harbaum
1   Sektion Pneumologie, Universitätsklinikum Hamburg-Eppendorf, Deutschland
,
M. Buerke
10   Abt. Kardiologie St. Marien-Krankenhaus Siegen, Deutschland
,
H. Ardeschir Ghofrani
7   Lungenzentrum, Universitätsklinikum Gießen und Marburg, Standort Gießen, Deutschland
8   Deutsches Zentrum für Lungenforschung (DZL)
,
M. M. Borst
11   Medizinische Klinik I, Caritas Krankenhaus Bad Mergentheim, Deutschland
,
H. H. Leuchte
12   II. Medizinische Abteilung, Krankenhaus Neuwittelsbach, München, Deutschland
,
T. J. Lange
13   Klinik und Poliklinik für Innere Medizin II, Universitätsklinikum Regensburg, Deutschland
,
J. Behr
14   Medizinische Klinik und Poliklinik V, Klinikum Großhadern, München, und Asklepios Fachkliniken München-Gauting, Deutschland
8   Deutsches Zentrum für Lungenforschung (DZL)
,
S. Ulrich
15   Klinik für Pneumologie, Universitätsspital Zürich, Schweiz
,
I. Lang
16   Abteilung für Kardiologie, Medizinische Universitätsklinik Wien, Österreich
,
H. Olschewski
17   Abteilung für Pneumologie, Universitätsklinikum Graz, Österreich
,
H. Gall
7   Lungenzentrum, Universitätsklinikum Gießen und Marburg, Standort Gießen, Deutschland
8   Deutsches Zentrum für Lungenforschung (DZL)
,
H.-J. Kabitz
18   II. Medizinische Klinik, Klinikum Konstanz, Deutschland
,
F.-X. Kleber
19   Cardio-Centrum Berlin, Akademische Lehrpraxis der Charité, Universitätsmedizin Berlin, Deutschland
,
M. Held
20   Abteilung Innere Medizin, Missionsärztliche Klinik Würzburg, Deutschland
,
M. M. Hoeper
21   Klinik für Pneumologie, Medizinische Hochschule Hannover und Deutsches Zentrum für Lungenforschung (DZL), Hannover, Deutschland
,
E. Grünig
22   Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg, Deutschland
8   Deutsches Zentrum für Lungenforschung (DZL)
› Author Affiliations
Further Information

Publication History

04 November 2014

06 November 2014

Publication Date:
09 December 2014 (online)

Also available at
    Permissions and Reprints

Schlüsselwörter

Pulmonale Hypertonie - Pulmonal arterielle Hypertonie - Gezielte Therapie der PAH - Therapiealgorithmus - Kombinationstherapie

Keywords

pulmonary hypertension - pulmonary arterial hypertension - targeted therapy - treatment algorithm - combination therapy
 

  • Referenzen

  • 1 Galie N, Hoeper MM, Humbert M et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. The 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Respir J 2009; 34: 1219-63
    CrossrefPubMedSearch in Google Scholar
  • 2 Galie N, Manes A, Negro L et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30: 394-403
    CrossrefPubMedSearch in Google Scholar
  • 3 Galie N, Corris PA, Frost A et al. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013; 62: D60-72
    CrossrefPubMedSearch in Google Scholar
  • 4 McLaughlin VV, Gaine SP, Howard LS et al. Treatment goals of pulmonary hypertension. J Am Coll Cardiol 2013; 62: D73-81
    CrossrefPubMedSearch in Google Scholar
  • 5 Mathai SC, Bueso M, Hummers LK et al. Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension. Eur Respir J 2010; 35: 95-104
    CrossrefPubMedSearch in Google Scholar
  • 6 Jais X, Launay D, Yaici A et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum 2008; 58: 521-31
    CrossrefPubMedSearch in Google Scholar
  • 7 Macchia A, Marchioli R, Tognoni G et al. Systematic review of trials using vasodilators in pulmonary arterial hypertension: why a new approach is needed. Am Heart J 2010; 159: 245-57
    CrossrefPubMedSearch in Google Scholar
  • 8 Sitbon O, Humbert M, Jais X et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105-11
    CrossrefPubMedSearch in Google Scholar
  • 9 Galie N, Rubin L, Hoeper M et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet 2008; 371: 2093-2100
    CrossrefPubMedSearch in Google Scholar
  • 10 Simonneau G, Rubin LJ, Galie N et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149: 521-530
    CrossrefPubMedSearch in Google Scholar
  • 11 Pulido T, Adzerikho I, Channick RN et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. The New England journal of medicine 2013; 369: 809-18
    CrossrefPubMedSearch in Google Scholar
  • 12 Thelin (sitaxentan) to be withdrawn due to cases of unpredictable serious liver injury.. EMA Europe; 2014. http://www.ema.europa.eu/ema/index.jsp?curl=/pages/news_and_events/news/2010/12/news_detail_001161.jsp&murl=menus/news_and_events/news_and_events.jsp&mid=WC0b01ac058004d5c1 (Accessed 2014-10-20)
  • 13 Galie N, Ghofrani HA, Torbicki A et al. Sildenafil Citrate Therapy for Pulmonary Arterial Hypertension. The New England journal of medicine 2005; 353: 2148-57
    CrossrefPubMedSearch in Google Scholar
  • 14 Galie N, Brundage BH, Ghofrani HA et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119: 2894-903
    CrossrefPubMedSearch in Google Scholar
  • 15 Olschewski H, Simonneau G, Galie N et al. Inhaled iloprost for severe pulmonary hypertension. NEJM 2002; 347: 322-9
    CrossrefPubMedSearch in Google Scholar
  • 16 Hoeper MM, Gall H, Seyfarth HJ et al. Long-term outcome with intravenous iloprost in pulmonary arterial hypertension. Eur Respir J 2009; 34: 132-7
    CrossrefPubMedSearch in Google Scholar
  • 17 Rubin LJ, Mendoza J, Hood M et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990; 112: 485-91
    CrossrefPubMedSearch in Google Scholar
  • 18 Barst RJ. Diagnosis and treatment of pulmonary artery hypertension. Curr Opin Pediatr 1996; 8: 512-9
    CrossrefPubMedSearch in Google Scholar
  • 19 Badesch DB, Tapson VF, McGoon MD et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000; 132: 425-34
    PubMedSearch in Google Scholar
  • 20 Simonneau G, Barst RJ, Galie N et al. 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: 800-4
    CrossrefPubMedSearch in Google Scholar
  • 21 Hiremath J, Thanikachalam S, Parikh K et al. Exercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trial. J Heart Lung Transplant 2010; 29: 137-49
    CrossrefPubMedSearch in Google Scholar
  • 22 Benza RL, Seeger W, McLaughlin VV et al. Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: the Treprostinil Sodium Inhalation Used in the Management of Pulmonary Arterial Hypertension (TRIUMPH) study open-label extension. J Heart Lung Transplant 2011; 30: 1327-33
    CrossrefPubMedSearch in Google Scholar
  • 23 McLaughlin VV, Benza RL, Rubin LJ et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010; 55: 1915-22
    CrossrefPubMedSearch in Google Scholar
  • 24 Tapson VF, Torres F, Kermeen F et al. 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: 1383-90
    CrossrefPubMedSearch in Google Scholar
  • 25 Tapson VF, Jing ZC, Xu KF et al. Oral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trial. Chest 2013; 144: 952-8
    CrossrefPubMedSearch in Google Scholar
  • 26 Jing ZC, Parikh K, Pulido T et al. Efficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trial. Circulation 2013; 127: 624-33
    CrossrefPubMedSearch in Google Scholar
  • 27 Simonneau G, Torbicki A, Hoeper MM et al. Selexipag: an oral, selective prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension. Eur Respir J 2012; 40: 874-80
    CrossrefPubMedSearch in Google Scholar
  • 28 Speich R, Treder U, Domenighetti G et al. Weaning from intravenous prostanoids and normalization of hemodynamics by long-term imatinib therapy in severe idiopathic pulmonary arterial hypertension. Int J Clin Pharm 2014; 36: 256-60
    CrossrefPubMedSearch in Google Scholar
  • 29 Giaid A, Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. NEJM 1995; 333: 214-21
    CrossrefPubMedSearch in Google Scholar
  • 30 Ghofrani HA, Galie N, Grimminger F et al. Riociguat for the treatment of pulmonary arterial hypertension. NEJM 2013; 369: 330-40
    CrossrefPubMedSearch in Google Scholar
  • 31 Ghofrani HA, D'Armini AM, Grimminger F et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. NEJM 2013; 369: 319-29
    CrossrefPubMedSearch in Google Scholar
  • 32 Hoeper MM, Markevych I, Spiekerkoetter E et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26: 858-63
    CrossrefPubMedSearch in Google Scholar
  • 33 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: 589-96
    CrossrefPubMedSearch in Google Scholar
  • 34 Bai Y, Sun L, Hu S et al. Combination therapy in pulmonary arterial hypertension: a meta-analysis. Cardiology 2011; 120: 157-65
    CrossrefPubMedSearch in Google Scholar
  • 35 Galie 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: 2080-6
    CrossrefPubMedSearch in Google Scholar
  • 36 First-Line Combination of Ambrisentan and Tadalafil Reduces Risk of Clinical Failure Compared to Monotherapy in Pulmonary Arterial Hypertension Outcomes Study. Gilead Sciences Inc; 2014. http://www.gilead.com/news/press-releases/2014/9/firstline-combination-of-ambrisentan-and-tadalafil-reduces-risk-of-clinical-failure-compared-to-monotherapy-in-pulmonary-arterial-hypertension-outcomes-study (Accessed 2014-10-13, 2014)
    Search in Google Scholar
  • 37 Sitbon O, Jais X, Savale L et al. Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study. Eur Respir J 2014; 43: 1691-7
    CrossrefPubMedSearch in Google Scholar
  • 38 Behr J, Borst MM, Winkler J et al. [A role for combination therapy in pulmonary arterial hypertension]. Pneumologie (Stuttgart, Germany) 2005; 59: 730-5
    Thieme ConnectPubMedSearch in Google Scholar
  • 39 de Perrot M, Granton JT, McRae K et al. Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience. J Thorac Cardiovasc Surg 2012; 143: 910-8
    CrossrefPubMedSearch in Google Scholar
  • 40 McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation 2006; 114: 1417-31
    CrossrefPubMedSearch in Google Scholar
  • 41 Ghofrani HA, Distler O, Gerhardt F et al. [Treatment of pulmonary arterial hypertension (PAH): recommendations of the Cologne Consensus Conference 2010]. Dtsch Med Wochenschr 2010; 135 (Suppl. 03) S87-101
    Thieme ConnectPubMedSearch in Google Scholar
  • 42 Opitz CF, Blindt R, Blumberg F et al. [Pulmonary hypertension: hemodynamic evaluation: recommendations of the Cologne Consensus Conference 2010]. Dtsch Med Wochenschr 2010; 135 (Suppl. 03) S78-86
    Thieme ConnectPubMedSearch in Google Scholar