Chronic thromboembolic pulmonary hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH)

Chronic thromboembolic pulmonary hypertension (CTEPH) may occur in about 2-4% of patients presenting with acute pulmonary embolism. It is considered when mean pulmonary artery pressure persists at or above 25 mm Hg after an episode of pulmonary thromboembolism [1]. Though it is a rare complication of pulmonary embolism, mortality and morbidity are significant. Upto 90% three year mortality has been reported in those with mean pulmonary artery pressures above 50 mm Hg. Majority of those with CTEPH may not give a history suggestive of previous presentation with acute pulmonary embolism.

In those who developed CTEPH after pulmonary embolism, it occurred within a year in 80% of cases in study of 325 patients followed up for a mean of 16.3 months [2]. Occurrence after 2 years was not noted in that study. Sheet anchor of treatment in CTEPH is pulmonary thromboendarterctomy in those who can tolerate the prolonged surgery. In those who are deemed unfit for surgery, in addition to anticoagulation, specific therapy for pulmonary hypertension will be considered. The medications include endothelin receptor antagonists like bosentan and ambrisentan, phosphodiesterase inhibitors like sildenafil and tadalafil, and protacyclin analogues.

Riociguat, a stimulator of soluble guanylate cyclase is one of the latest medications for CTEPH.


  1. Ozsu S, Cinarka H. Chronic thromboembolic pulmonary hypertension: Medical treatment. Pulm Circ. 2013 Apr;3(2):341-4.
  2. Korkmaz A, Ozlu T, Ozsu S, Kazaz Z, Bulbul Y. Clin Appl Thromb Hemost. 2012 Jun;18(3):281-8.