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)
Journal Title: Journal of Rare Cardiovascular Diseases - Year 2013, Vol 1, Issue 2
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. JRCD2013; 1 (2): 25–32Key words: Klippel–Trénaunay syndrome, chronic thromboembolic pulmonary hypertension, pulmonary endartherectomy
Authors and Affiliations
Małgorzata Poręba, Marcin Misztal, Andrzej Biederman, Agnieska Skrzypek, Piotr Szatkowski, Ewelina Pirsztuk, Danuta Czernecka-Mroczek, Jadwiga Nessler
Keywords
Related Articles
- EP ID EP245379
- DOI 10.20418/jrcd.vol1no2.79
- Views 99
- Downloads 0
A 33-year-old man after sudden cardiac arrest as a first manifestation of Brugada syndrome (RCD code: V‑1A.1)
Brugada syndrome is an autosomal dominant genetic disease with variable expression characterized by abnormal electrocardiographic findings – right bundle branch block and ST-segment elevation in the anterior precordial l...
Facilitating recognition and grouping the expertise in the main fields of rare cardiovascular diseases
Dear Friends and Colleagues, Dear Readers,We are pleased to deliver at your hands the second issue of your quarterly Journal of Rare Cardiovascular Diseases. The feedback we have received, after launching the Journal in...
New Frontiers in Interventional Cardiology 2013 – Highlights from the biggest workshop in Central and Eastern Europe
Since 1999, at the end of each year, cardiologists, interventional cardiologists and cardiac surgeons from all over the World meet in Cracow to discuss the most challanging topics in recent cardiology. From the beginning...
Update on myocarditis – review (RCD code: III-6A.1)
Myocardits is an inflammatory disease of the myocardium diagnosed by established histological and immunohistochemical criteria. The true incidence of myocarditis is unknown. There are numerous potential etiologic factors...
Hemangioma of the left ventricle (RCD code: VI-1B.4)
Cardiac tumors are quite rare, and differential diagnosis of them is challenging.We present the case report of a young man complaining on non specific chest pain who was reported to Cardiology Ward due to suspicion of th...