Abstract
Abstract Rendu-Osler-Weber (ROW) syndrome or hereditary hemorrhagic telangiectasia is a rare autossomic dominant disease characterized by vascular dysplasia involving multiple systems and associated with an increased bleeding risk. The presence of atrial fibrillation in this population becomes a challenge, regarding the evaluation of bleeding and thrombotic risks and the best approach for the patient management. A 72 years-old female with ROW syndrome, atrial fibrillation and a CHA2DS2-VASc risk score of four, was admitted to our hospital for left atrial appendage (LAA) closure after a period of novel oral anticoagulation crowed with multiple haemorrhagic events and blood transfusions. After the implantation of a Watchman LAA number 33 closure device (Image 1A), and because of the concomitant diagnose of chronic pulmonary embolism, she was restarted on anticoagulation therapy in lower doses, with initial tolerance. For evaluation of LAA closure device during follow-up, after an unsuccessful attempt of transoesophageal echocardiography (TEE) because of severe epistaxis subsequent to oropharyngeal anaesthesia, the patient underwent a Cardiac Computed Tomography (CT) that showed a moderate peri-device leak (4.4x11mm, 0.73cm2) – (Image 1B). For better characterization, the patient underwent a new attempt of TEE with the support of an anaesthesiologist and the need of local vasoconstrictor agents and tranexamic acid for epistaxis and oropharyngeal bleeding control. TEE confirmed a moderate posterior device-leak (7.1mm) with absence of cavitary thrombus (Image 1C). After a year of anticoagulation with new significant bleeding events, TEE was repeated with the anaesthetic and pharmacologic preparation. This TEE showed a decrease in peri-device leak (<5mm wide – Image 1D), which was fundamental for supporting the multidisciplinary team decision of interruption of anticoagulation therapy. With this case we want to highlight the challenging decisions regarding patients with ROW syndrome and atrial fibrillation. Specifically, we must be alert about possible complications in trying to perform TEE in this population, once the frequent occurrence of telangiectasias in the oropharynges that may occur, as in this patient. Abstract P182 Figure. Image 1
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