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10.1183/09031936.00201612

http://scihub22266oqcxt.onion/10.1183/09031936.00201612
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23397304!ä!23397304

suck abstract from ncbi


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pmid23397304      Eur+Respir+J 2013 ; 41 (4): 985-90
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  • Chronic thromboembolic pulmonary hypertension: role of medical therapy #MMPMID23397304
  • Pepke-Zaba J; Jansa P; Kim NH; Naeije R; Simonneau G
  • Eur Respir J 2013[Apr]; 41 (4): 985-90 PMID23397304show ga
  • Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease with poor prognosis if not treated. The treatment of choice is surgery with pulmonary endarterectomy. However, a significant percentage of patients are deemed non-operable due to distal distribution of the disease and arteriopathy in the non-occluded areas that is indistinguishable from pulmonary arterial hypertension (PAH). The overlap in clinical presentation, pathological features and pathogenesis between PAH and CTEPH provides a compelling rationale for exploring the efficacy of PAH-targeted therapies in CTEPH. These therapies are often considered for non-operable patients and are also used in operable patients as a bridge to surgery or as post-pulmonary endarterectomy therapy for persistent pulmonary hypertension, despite the fact they are not licensed for CTEPH. Two randomised clinical trials have been performed in non-operable CTEPH patients. The BENEFiT study, with the endothelin receptor antagonist bosentan, did not show improvement in walking distance. Recently, the CHEST-1 trial, with the soluble guanylate cyclase stimulator riociguat, met study end-point and demonstrated significant improvement in walking distance in patients with non-operable CTEPH. There is an urgent need for more randomised clinical trials designed to clarify whether administration of PAH-targeted therapies improves clinically meaningful end-points in various CTEPH populations.
  • |Anticoagulants/therapeutic use[MESH]
  • |Chronic Disease[MESH]
  • |Diagnosis, Differential[MESH]
  • |Disease Progression[MESH]
  • |Endarterectomy[MESH]
  • |Endothelin Receptor Antagonists[MESH]
  • |Familial Primary Pulmonary Hypertension[MESH]
  • |Humans[MESH]
  • |Hypertension, Pulmonary/diagnosis/*therapy[MESH]
  • |Phosphodiesterase Inhibitors/therapeutic use[MESH]
  • |Prognosis[MESH]
  • |Prostaglandins/therapeutic use[MESH]
  • |Randomized Controlled Trials as Topic[MESH]
  • |Thromboembolism/diagnosis/*therapy[MESH]


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