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Bosentan in inoperable chronic thromboembolic pulmonary hypertension
  1. R Hughes,
  2. P George,
  3. J Parameshwar,
  4. F Cafferty,
  5. J Dunning,
  6. N W Morrell,
  7. J Pepke-Zaba
  1. Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge CB3 8RE, UK
  1. Correspondence to:
    Dr J Pepke-Zaba
    Pulmonary Vascular Diseases Unit, Papworth Hospital, Cambridge CB3 8RE, UK; Joanna.pepkezabapapworth.nhs.uk

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a devastating disease in which the pulmonary vasculature becomes obstructed by organised fibrotic material, presumed to be the consequence of incomplete resolution of pulmonary emboli. The ensuing increased pulmonary vascular resistance and right ventricular dysfunction results in severe exercise limitation, symptomatic right heart failure, and markedly impaired survival.

Recent studies suggest that the frequency of this condition is higher than previously appreciated, occurring in up 3.8% of patients following acute pulmonary embolism after 2 years.1 The treatment of choice is pulmonary endarterectomy (PEA), a potentially curative surgical procedure in which the fibrotic material is removed from …

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