Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry

Circulation. 2011 Nov 1;124(18):1973-81. doi: 10.1161/CIRCULATIONAHA.110.015008. Epub 2011 Oct 3.


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.

Methods and 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Chronic Disease
  • Endarterectomy / mortality
  • Endothelin Receptor Antagonists
  • Female
  • Humans
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / surgery*
  • Incidence
  • Internationality
  • Male
  • Middle Aged
  • Phosphodiesterase 5 Inhibitors / therapeutic use
  • Prospective Studies
  • Prostaglandins I / therapeutic use
  • Recurrence
  • Registries*
  • Risk Factors
  • Vena Cava Filters / statistics & numerical data
  • Venous Thromboembolism / drug therapy
  • Venous Thromboembolism / mortality*
  • Venous Thromboembolism / surgery*


  • Endothelin Receptor Antagonists
  • Phosphodiesterase 5 Inhibitors
  • Prostaglandins I