PULMONARY THROMBOENDARTERECTOMY IN A PATI ENT WITH PRIMARY ANTIPHOSPHOLIPID SYNDROME

Pulmonary arterial hypertension (PAH) is an infrequent manifestation o f the primary antiphospholipid syndrome (PAPS). It may appear due to d ifferent mechanisms although the most common cause is recurrent pulmon ary embolisms. In some cases the thrombi do not dissolve and organize to form fibrous masses which occlude the pulmonary veins giving place to chronic thromboembolic pulmonary hypertension, When the thrombi are located in the proximal arteries, thromboendarterectomy may be curati ve. The first case of a patient with PAPS diagnosed with PAH secondary to chronic thrombosis of the proximal pulmonary arteries, in whom a s uccessful pulmonary thromboendarterectomy was performed is herein repo rted.