Summary

Papillary fibroelastomas are rare benign tumours arising from the cardiac endothelium. The rationale behind the treatment of fibroelastomas is to eliminate the risk of embolic events and the associated morbidity and mortality. These tumours present predominantly in males during the seventh decade of life and most commonly affect the aortic valve. We report a case of aortic valve papillary fibroelastoma with an atypical clinical presentation, treated with valve-sparing surgical excision. Our case highlights the management of this rare but serious condition and emphasises the need to develop clear guidelines regarding the treatment of asymptomatic patients with a papillary fibroelastoma.