Cardiac myxomas extending from the right ventricle are uncommon and this rare condition is potentially associated with several risks and poor outcomes. We report the case of a patient with a right ventricular myxoma who had a recent haemorrhagic stroke due to an arteriovenous malformation. The preoperative and intraoperative managements were challenging and required several difficult choices by the medical team and consequently, a multidisciplinary approach and a benefit-risk evaluation were necessary to determinate timely surgical resection in an urgent situation, simultaneously avoiding delayed or improper treatments, achieving a successful outcome in a very high-risk situation.
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