Chronic thromboembolic pulmonary hypertension due to upper-extremity deep vein thrombosis caused by thoracic outlet syndrome

Abstract

We report on a 20 year-old woman diagnosed with pulmonary embolism (PE) and right subclavian vein thrombosis attributable to stasis caused by right clavicular prominence. At the 10-months follow-up, the patient had developed chronic thromboembolic pulmonary hypertension (CTEPH), and treatment was begun with a dual endothelin receptor antagonist. Very few cases of deep venous thrombosis of upper limb have been reported in relation to anatomical abnormalities. This case is also exceptional because the patient developed a chronic thromboembolic pulmonary hypertension, whose incidence is estimated at 0.5% of all symptomatic PE.