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Vascular malformations as underlying cause of chronic thromboembolism and pulmonary hypertension
Charlene E U Oduber1, Victor E A Gerdes, Chantal M A M van der Horst
1Department of Plastic, Reconstructive and Hand surgery, Academic Medical Centre, Amsterdam, The Netherlands. ceoduber@gmail.com
Abstract:
We report four patients with chronic thromboembolic pulmonary hypertension (CTEPH) presumably due to recurrent pulmonary embolism from low-flow vascular malformations, and give a review of the literature. Venous malformations, such as those observed in Klippel- Trenaunay syndrome (KTS) can be associated with hypercoagulability, thrombosis and recurrent pulmonary embolism and ultimately CTEPH. Since many physicians appear unfamiliar with these potential complications, patients may experience a delayed diagnosis of this progressive and potentially life-threatening CTEPH.
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