Aortic valve fibroelastoma: a rare cause of stroke

Abstract

The prevalence of primary cardiac tumours varies from 0.02% to 0.45%. Cardiac papillary fibroelastoma (CPF) is a rare tumour diagnosed incidentally on imaging. The clinical manifestations result from thromboembolisation and include transient ischaemic attack, stroke and sudden cardiac death. We present a patient aged 57 years with CPF arising from the aortic valve. The patient presented with right hemiparesis due to acute stroke. He received tissue plasminogen activator with complete resolution of neurological symptoms. Echocardiography revealed a broad-based, gelatinous, non-mobile mass on the left aortic cusp. The tumour was excised sparing the aortic valve. The patient recovered rapidly without any complications. The histopathological examination confirmed the diagnosis of CPF. A review of the literature suggests that CPF is a rare but treatable cause of stroke. The course is not clear and there are no tumours or patient-related characteristics which could predict the risk of thromboembolisation. Surgical treatment is definite and is relatively safe.

The importance of early completion of cardiac investigations after ischaemic stroke: a case and systematic review of reperfusion therapy in stroke due to cardiac fibroelastoma

“…6 Along with this case, a detailed search of PUBMED and EMBASE revealed 11 additional cases (supplementary material S1) that had described good outcomes with patients undergoing reperfusion therapy in strokes secondary to CPFs. 2,3,[8][9][10][11][12][13][14] However, all but one had a histological examination of the retrieved clot that confirmed a fragment of the tumour, and hence one could not exclude the possibility that some of the embolised clots in these studies contained predominantly thrombus rather than the tumour material, which could in turn explain the good prognosis. 11 Once identified, CPFs should be removed as soon as possible due to the risk of recurrent embolic events, while balancing the increased risk of bleeding during cardiopulmonary bypass.…”

“…CPFs are benign tumours, accounting for <10% of all cardiac tumours but the most common type of valvular tumour. 1,2 The majority of CPFs are found at the aortic valve, with the left ventricle as the most common non-valvular site. [3][4][5] The mean age of onset was 66 years and the size ranged from 4-70 mm.…”

Giant and multiple cardiac papillary fibroelastomas masquerading as acute cerebrovascular events

“…Cardiac papillary fibroelastomas (CPFs) are rare benign tumors, usually small and unique, arising most often from the valvular endothelium. Their prevalence varies from 0.02% to 0.08% in the medical literature [ 1 , 2 ]. Their clinical significance is due to the very high embolization propensity, which is usually in the cerebral circulation [ 1 , 3 , 4 , 5 ] but also in the coronary arteries [ 6 , 7 ].…”

“…Their prevalence varies from 0.02% to 0.08% in the medical literature [ 1 , 2 ]. Their clinical significance is due to the very high embolization propensity, which is usually in the cerebral circulation [ 1 , 3 , 4 , 5 ] but also in the coronary arteries [ 6 , 7 ]. While these tumors are mostly solitary, there have been reports of fibroelastomas in multiple locations [ 8 , 9 , 10 ], but this situation remains exceptional.…”

Papillary fibroelastoma on the aortic valve presenting as multiple cardiac arrests from electrical storm due to ischemia in patient without previous cardiac history

“…The prevalence of primary cardiac tumors varies from 0.02% to 0.45% 1 . Cardiac papillary fibroelastoma (PFE) is a benign endocardial papilloma.…”