A 39-year-old woman with atypical variant of Klippel-Trénaunay syndrome and progressive thromboembolic pulmonary hypertension, successfully treated by pulmonary thromboendarterectomy (RCD code: II-1A.5)

Abstract

We present a case of 39-year old female with Klippel-Trenaunay syndrome (KTS) complicated by chronic thromboembolic pulmonary hypertension (CTEPH). Our patient was operated in adolescence by vascular surgeons and severe varicosities of her leg were excised. After second pregnancy she manifested pulmonary embolism (PE) and was treated with vitamin K antagonists (VKA) and implantation of vena cava filter. Despite chronic VKA therapy she developed pulmonary embolism again. We present our TTE studies, X-ray and CT scans confirming diagnosis of pulmonary hypertension. Because of worsening of her cardiopulmonary status she was send to Cardiac Surgery Department and underwent pulmonary thromboendarterectomy. Extensive quantities of chronic thromboembolic material were cut out from both pulmonary arteries. At one year follow-up her functional status returned to NYHA class I. Control TTE demonstrated normal diameter and function of her right cardiac chambers. We conclude that vascular anomalies existing in KTS although present locally are responsible for general hypercoagulability. We also wonder what should be proper treatment and which diagnostic tests should be perform to prevent from severe thromboembolic pulmonary hypertension. JRCD 2013; 1 (2): 61-68