Clinical Research in Cardiology

, Volume 99, Issue 9, pp 553–556

Standard PAH therapy improves long term survival in CTEPH patients

  • Hans-Juergen Seyfarth
  • Michael Halank
  • Heinrike Wilkens
  • Hans-Joachim Schäfers
  • Ralf Ewert
  • Martin Riedel
  • Ernst Schuster
  • Hans Pankau
  • Stefan Hammerschmidt
  • Hubert Wirtz
Original Paper



Chronic thromboembolic pulmonary hypertension (CTEPH), subsequent to pulmonary embolism is a relatively frequent cause of pulmonary hypertension. Similar to patients with pulmonary arterial hypertension (PAH), CTEPH carries a poor prognosis. There is no hard evidence for any other therapy except pulmonary endarterectomy and none for those patients that are not eligible for this procedure.

Patients and methods

Fifty patients with confirmed, inoperable CTEPH receiving specific vasodilative therapy (prostanoids, endothelin receptor antagonists, PDE 5-inhibitors or combination) were included in this retrospective study (mean age 55 years, range 16–76 years; 36 female, 14 male). Kaplan–Meier plots of these patients were compared with Kaplan–Meier plots of two historical CTEPH patient groups without any specific vasodilative treatment by log rank tests.


CTEPH patients treated with specific vasodilative compounds as used for therapy of PAH were followed up for 52 ± 30 months and had a significantly improved survival compared with patients treated without PAH type vasodilators (p ≤ 0.0002).


Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.


Pulmonary hypertension Pulmonary embolism Survival Vasodilator agents 



Cardiac index


Chronic thromboembolic pulmonary hypertension


Idiopathic pulmonary arterial hypertension


Mean pulmonary arterial pressure


Pulmonary arterial hypertension


Pulmonary endarterectomy


Pulmonary vascular resistance


  1. 1.
    Bonderman D, Jakowitsch J, Adlbrecht C, Schemper M, Kyrle PA, Schonauer V, Exner M, Klepetko W, Kneussl MP, Maurer G, Lang I (2005) Medical conditions increasing the risk of chronic thromboembolic pulmonary hypertension. Thromb Haemost 93:512–516PubMedGoogle Scholar
  2. 2.
    Bonderman D, Nowotny R, Skoro-Sajer N, Jakowitsch J, Adlbrecht C, Klepetko W, Lang IM (2005) Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 128:2599–2603CrossRefPubMedGoogle Scholar
  3. 3.
    Bresser P, Fedullo PF, Auger WR, Channick RN, Robbins IM, Kerr KM, Jamieson SW, Rubin LJ (2004) Continuous intravenous epoprostenol for chronic thromboembolic pulmonary hypertension. Eur Respir J 23:595–600CrossRefPubMedGoogle Scholar
  4. 4.
    Bresser P, Pepke-Zaba J, Jais X, Humbert M, Hoeper MM (2006) Medical therapies for chronic thromboembolic pulmonary hypertension. Proc Am Thorac Soc 3:594–600CrossRefPubMedGoogle Scholar
  5. 5.
    Dartevelle P, Fadel E, Mussot S, Chapelier A, Hervé P, de Perrot M, Cerrina J, Ladurie FL, Lehouerou D, Humbert M, Sitbon O, Simonneau G (2004) Chronic thromboembolic pulmonary hypertension. Eur Respir J 23:637–648CrossRefPubMedGoogle Scholar
  6. 6.
    Doyle RL, McC Douglas, Channick RN, Simmonneau G, Conte J (2004) Surgical treatments/interventions for pulmonary arterial hypertension. ACCP evidence-based clinical practice guidelines. Chest 126:63S–71SCrossRefPubMedGoogle Scholar
  7. 7.
    Egermayer P, Peacock AJ (2000) Is pulmonary embolism a common cause of chronic pulmonary hypertension? Limitations of the embolic hypothesis. Eur Respir J 15:440–448CrossRefPubMedGoogle Scholar
  8. 8.
    Ewert R, Opitz CF, Wensel R, Winkler J, Halank M, Felix SB (2007) Continous intravenous iloprost to revert treatment failure of first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Clin Res Cardiol 96:207–211CrossRefGoogle Scholar
  9. 9.
    Fedullo PF, Auger WR, Kerr KM, Rubin LJ (2001) Chronic thromboembolic pulmonary hypertension. N Engl J Med 345:1465–1472CrossRefPubMedGoogle Scholar
  10. 10.
    Ghofrani HA, Schermuly RT, Rose F, Wiedemann R, Kohstall MG, Kreckel A, Olschewski H, Weissmann N, Enke B, Ghofrani S, Seeger W, Grimminger F (2003) Sildenafil for long-term treatment of nonoperable chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 167:1139–1141CrossRefPubMedGoogle Scholar
  11. 11.
    Hoeper MM, Kramm T, Wilkens H, Schulze C, Schaefers HJ, Welte T, Mayer E (2005) Bosentan therapy for inoperable chronic thromboembolic pulmonary hypertension. Chest 128:2363–2367CrossRefPubMedGoogle Scholar
  12. 12.
    Hughes RJ, Jais X, Bonderman D, Suntharalingam J, Humbert M, Lang I, Simonneau G, Pepke-Zaba J (2006) The efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension: a 1-year follow-up study. Eur Respir J 28:138–143CrossRefPubMedGoogle Scholar
  13. 13.
    Jaïs X, D’Armini AM, Jansa P, Torbicki A, Delcroix M, Ghofrani HA, Hoeper MM, Lang IM, Mayer E, Pepke-Zaba J, Perchenet L, Morganti A, Simonneau G, Rubin LJ, for the BENEFiT Study Group (2008) Bosentan for treatment of inoperable chronic thromboembolic pulmonary hypertension. JACC 52:2127–2134Google Scholar
  14. 14.
    Mc Laughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N, Rainisio M, Simonneau G, Rubin LJ (2005) Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 25:244–249CrossRefGoogle Scholar
  15. 15.
    Moser KM, Bloor CM (1993) Pulmonary vascular lesions occuring in patients with chronic major vessel thromboembolic pulmonary hypertension. Chest 103:685–692CrossRefPubMedGoogle Scholar
  16. 16.
    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 87:340–345CrossRefPubMedGoogle Scholar
  17. 17.
    Olschewski H, Hoeper MM, Borst MM, Ewert R, Grünig E, Kleber FX, Kopp B, Opitz C, Reichenberger F, Schmeisser A, Schranz D, Schulze-Neick I, Wilkens H, Winkler J, Worth H (2007) Diagnosis and therapy of chronic pulmonary hypertension. Clin Res Cardiol 96:301–330CrossRefPubMedGoogle Scholar
  18. 18.
    Olschewski H, Simonneau G, Galie N, Higgenbottam 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 347:322–329CrossRefPubMedGoogle Scholar
  19. 19.
    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 123:1583–1588CrossRefPubMedGoogle Scholar
  20. 20.
    Pengo V, Lensing AW, Prins MH, Marchiori A, Davidson BL, Tiozzo F, Albanese P, Biasiolo A, Pegoraro C, Iliceto S, Prandoni P (2004) Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 350:2257–2264CrossRefPubMedGoogle Scholar
  21. 21.
    Riedel M, Stanek V, Widimsky J, Prerovsky I (1982) Longterm follow-up of patients with pulmonary thromboembolism. Chest 81:151–158CrossRefPubMedGoogle Scholar
  22. 22.
    Rosenkranz S (2007) Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol 96:527–541CrossRefPubMedGoogle Scholar
  23. 23.
    Seyfarth HJ, Hammerschmidt S, Gessner C, Halank M, Wirtz H (2006) Chronic thromboembolic pulmonary hypertension—therapeutic options. Curr Respir Med Rev 2:431–438Google Scholar
  24. 24.
    Seyfarth HJ, Hammerschmidt S, Pankau H, Winkler J, Wirtz H (2006) Long-term bosentan in chronic thromboembolic pulmonary hypertension. Respiration 569:1–6Google Scholar
  25. 25.
    Seyfarth HJ, Pankau H, Hammerschmidt S, Schauer J, Wirtz H, Winkler J (2005) Bosentan improves exercise tolerance and Tei index in pulmonary hypertension patients with prostanoid therapy. Chest 128:709–713CrossRefPubMedGoogle Scholar
  26. 26.
    Sitbon O, McLaughlin VV, Badesch DB, Barst RJ, Black C, Galie N, Humbert M, Rainisio M, Rubin LJ, Simonneau G (2005) 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 60:1025–1030CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Hans-Juergen Seyfarth
    • 1
  • Michael Halank
    • 2
  • Heinrike Wilkens
    • 3
  • Hans-Joachim Schäfers
    • 3
  • Ralf Ewert
    • 4
  • Martin Riedel
    • 5
  • Ernst Schuster
    • 6
  • Hans Pankau
    • 1
  • Stefan Hammerschmidt
    • 1
  • Hubert Wirtz
    • 1
  1. 1.Department of Respiratory MedicineUniversity of LeipzigLeipzigGermany
  2. 2.Internal Medicine IUniversity of DresdenDresdenGermany
  3. 3.Universitätsklinikum Homburg/SaarHomburgGermany
  4. 4.University of GreifswaldGreifswaldGermany
  5. 5.Deutsches Herzzentrum, Klinikum Der TU MünchenMunichGermany
  6. 6.Institut for Medical Informatics, Statistics and EpidemiologyUniversity of LeipzigLeipzigGermany

Personalised recommendations