Chronic thromboembolic pulmonary hypertension (CTEPH)

The spectrum of venous thromboembolism encompasses deep vein thrombosis, acute thrombus passing through the right heart, acute pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH). Pulmonary embolism may recur and have severe sequelae, such as the post-thrombotic syndrome and CTEPH. It is thought at present, based on a long-term prospective study of about 300 patients, that CTEPH results within two years in 3.8% of patients who had survived the first symptomatic pulmonary embolism. Previous incidence of pulmonary embolism, young age, a large perfusion deficit and no known cause were associated with an increased probability of subsequent CTEPH. The diagnosis and treatment of CTEPH call for an interdisciplinary approach, the former still relying on finding a positive perfusion scan together with pulmonary hypertension. Treatment of choice is surgical endarterectomy of the pulmonary obstruction which will result in functional healing in 80% of patients.