Successful pulmonary thromboendarterectomy in a patient with Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by a triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and va ricose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported is the development of chronic thromboembolic pulmon ary hypertension (CTEPH) and subsequent death from right ventricular failur e. We report the first patient with KTS to undergo a successful pulmonary t hromboendarterectomy for CTEPH.