Standard PAH therapy improves long term survival in CTEPH patients
- First Online:
- 247 Downloads
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.
KeywordsPulmonary hypertension Pulmonary embolism Survival Vasodilator agents
Chronic thromboembolic pulmonary hypertension
Idiopathic pulmonary arterial hypertension
Mean pulmonary arterial pressure
Pulmonary arterial hypertension
Pulmonary vascular resistance
- 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
- 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
- 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
- 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.Seyfarth HJ, Hammerschmidt S, Pankau H, Winkler J, Wirtz H (2006) Long-term bosentan in chronic thromboembolic pulmonary hypertension. Respiration 569:1–6Google Scholar
- 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