[A case of Klippel-Trenaunay-Weber syndrome associated with progressive pulmonary hypertension].

Abstract

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder characterized by varicose veins, cutaneous hemangiomas, hypertrophy of soft tissue and bone and arteriovenous malformations. We present a case of a 43-year-old man with KTWS. He experienced progressive pulmonary hypertension due to recurrent pulmonary embolism, which developed despite adequate anticoagulation. This case report suggests that patients with KTWS need more aggressive management and treatment of their thromboembolitic state and pulmonary hypertension.