Presentation Highlights

  • Chronic thromboembolic pulmonary hypertension, or CTEPH, is a rare sequela of venous thromboembolism (VTE) with a cumulative risk of 0.1-10%.
  • While CTEPH has long been thought to have a direct relationship with VTE, additional mechanisms and causation for CTEPH are now under investigation.
  • Exploration of inflammatory mechanisms in vasculature occurring during thrombosis or during thrombus resolution are now the targets of investigation.
  • It is important to note that a linear process between VTE and CTEPH does not necessarily exist and that up to 50% of patients with CTEPH may have no previous VTE history.

Irene Lang, M.D. discussed her work in this field at Monday’s State-of-the Art Session.


Monday’s State-of-the-Art Session, “What Happens After Acute VTE?” included an important discussion about chronic thromboembolic pulmonary hypertension (CTEPH) led by Irene Lang, M.D. During this session, VTE experts and experts from related hematologic fields engaged in a very interesting discussion about events that occur after a patient experiences an acute VTE. Specifically, consequences of acute VTE such as post-thrombotic syndrome and CTEPH were discussed.

CTEPH is a rare sequala of VTE; the reported cumulative risk of CTEPH following VTE widely varies in the literature, from 0.1% to 10%. This variation in reported risk is probably due to variations in study design and methods used to define VTE and CTEPH diagnoses.

There is a likely mechanistic link between VTE and CTEPH, however the mechanism leading to development of CTEPH is not completely understood. The development of CTEPH is likely related to inflammatory processes in pulmonary vasculature during the acute event or as part of thrombus resolution.

As noted by current risk scores, patients who have recurrent VTE are at the highest risk of developing CTEPH. Other risk factors for CTEPH include prior splenectomy or existing inflammatory bowel disease, presence of antiphospholipid antibodies, thyroid hormone replacement therapy, and active or past history of malignancy.

Lang’s primary message for today’s presentation was that there is not always a clear connection between VTE and CTEPH, with up to 50% of patients with CTEPH having no previous VTE history. Other mechanisms such as inflammatory modifiers of thrombus resolution and/or vascular inflammation are under exploration. There remains much to learn about this condition.

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