Papillary fibroelastoma of the aortic valve - a case report and literature review |
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Authors: | Neerod K Jha Michael Khouri Donogh M Murphy Alessandro Salustri Javed A Khan Moataz A Saleh Friederike Von Canal Norbert Augustin |
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Affiliation: | 1.Division of Adult Cardiac Surgery, Institute of Cardiac Sciences,Sheikh Khalifa Medical City (Managed by Cleveland Clinic),Abu Dhabi,UAE;2.Division of Cardiology, Institute of Cardiac Sciences,Sheikh Khalifa Medical City (Managed by Cleveland Clinic),Abu Dhabi,UAE;3.Department of Laboratory Medicine, Sheikh Khalifa Medical City,(Managed by Cleveland Clinic),Abu Dhabi,UAE;4.Department of Anaesthesiology, Sheikh Khalifa Medical City,(Managed by Cleveland Clinic),Abu Dhabi,UAE |
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Abstract: | The prevalence of primary cardiac tumour ranges from 0.0017-0.28% and papillary fibroelastoma is rare but not uncommon benign cardiac neoplasm. Currently, with the advent of higher-resolution imaging technology especially transoesophageal echocardiography such cases being recognized frequently. The clinical presentation of these tumours varies from asymptomatic to severe ischaemic or embolic complications. We herein, present a 50-year-old female patient with a papillary fibroelastoma of the aortic valve arising from the endocardium of the right coronary cusp very close to the commissure between the right and non-coronary cusps. The patient presented with angina-like chest pain and was investigated using echocardiography and CT angiographic modalities in addition to the usual investigations. The differential diagnosis considered was a thrombus, myxoma, Lambl's excrescence and infective vegetation. The surgical management included a prompt resection of the tumour on cardiopulmonary bypass avoiding injury to the aortic valve. The patient recovered well. A review of the literature suggests that the cardiac papillary fibroelastoma is a rare but potentially treatable cause of embolic stroke and other fatal complications, therefore, a strong suspicion; appropriate use of imaging modality, preoperative anticoagulation and urgent surgical resection is warranted. Also, possibility of this diagnosis should be kept in mind while managing cardiac or valvular tumours. |
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