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CTEPH Risk Factors

Multiple risk factors for CTEPH have been identified.

As reviewed by Piazza and Goldhaber in 20111 and by Kim and Lang in 2012,14 multiple factors related to pulmonary embolism (PE) can increase the risk of developing CTEPH:

  • Recurrent, unprovoked, or idiopathic PE
  • Large perfusion defects when PE was detected
  • Younger or older age when PE was detected
  • Pulmonary-artery systolic pressure >50 mmHg at PE’s first manifestation
  • Persistent PH evident when echocardiography is performed 6 months after acute PE was detected


As many as 1 out of every 25 of your previously treated PE patients (>3 months of anticoagulation5) could go on to develop CTEPH.2,7*

*Based on a study with 223 patients in which 3.8% were diagnosed with CTEPH within 2 years of their first episode of pulmonary embolism with or without prior deep-vein thrombosis (95% CI, 1.1 to 6.5). CTEPH did not develop after two years in any of the 132 remaining patients with more than 2 years of follow up.

In addition to these PE-related risk factors, some medical conditions independent of PE have been associated with increased risk of CTEPH:

  • Infected surgical cardiac shunts or pacemaker or defibrillator leads
  • Splenectomy
  • Chronic inflammatory disorders
  • Antiphospholipid syndrome
  • Thyroid-replacement therapy
  • Cancer

Thrombotic and genetic factors may also increase risk of CTEPH:

  • Lupus anticoagulant or antiphospholipid antibodies
  • Increased levels of factor VIII
  • Dysfibrinogenemia
  • ABO blood groups other than O
  • HLA polymorphisms
  • Abnormal endogenous fibrinolysis
  • Elevated plasma levels of lipoprotein(a)

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Multiple factors related to PE can increase the risk of CTEPH.

Potential Precursors to CTEPH


Paul Forfia, MD, of Temple University Hospital, Philadelphia, discusses how venous thromboembolism can be a potential precursor to CTEPH.

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