title: Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry.
abstract: BACKGROUND
  Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated.
  
  
  RESULTS
  The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate.
  
  
  CONCLUSIONS
  Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments.
authors.affiliation: Papworth Hospital, Cambridge, UK. joanna.pepkezaba@papworth.nhs.uk
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.forename: Marion
authors.initials: M
authors.lastname: Delcroix
authors.forename: Irene
authors.initials: I
authors.lastname: Lang
authors.forename: Eckhard
authors.initials: E
authors.lastname: Mayer
authors.forename: Pavel
authors.initials: P
authors.lastname: Jansa
authors.forename: David
authors.initials: D
authors.lastname: Ambroz
authors.forename: Carmen
authors.initials: C
authors.lastname: Treacy
authors.forename: Andrea M
authors.initials: AM
authors.lastname: D'Armini
authors.forename: Marco
authors.initials: M
authors.lastname: Morsolini
authors.forename: Repke
authors.initials: R
authors.lastname: Snijder
authors.forename: Paul
authors.initials: P
authors.lastname: Bresser
authors.forename: Adam
authors.initials: A
authors.lastname: Torbicki
authors.forename: Bent
authors.initials: B
authors.lastname: Kristensen
authors.forename: Jerzy
authors.initials: J
authors.lastname: Lewczuk
authors.forename: Iveta
authors.initials: I
authors.lastname: Simkova
authors.forename: Joan A
authors.initials: JA
authors.lastname: Barberà
authors.forename: Marc
authors.initials: M
authors.lastname: de Perrot
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.forename: Sean
authors.initials: S
authors.lastname: Gaine
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.forename: Miguel A
authors.initials: MA
authors.lastname: Gomez-Sanchez
authors.forename: Gabor
authors.initials: G
authors.lastname: Kovacs
authors.forename: Abdul Monem
authors.initials: AM
authors.lastname: Hamid
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D000368:Aged; D002908:Chronic Disease; D004691:Endarterectomy / Q000401:mortality; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery*; D015994:Incidence; D038622:Internationality; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011446:Prospective Studies; D044062:Prostaglandins I / Q000627:therapeutic use; D012008:Recurrence; D012042:Registries; D012307:Risk Factors; D016306:Vena Cava Filters / Q000706:statistics & numerical data; D054556:Venous Thromboembolism / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery*
chemical_list: D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D044062:Prostaglandins I