A case of embolic stroke and failed treatment with anticoagulation with papillary fibroelastoma

Papillary Fibroelastoma (PFE) is a primary, benign cardiac tumour that carries a high embolic risk. 1 It is a rare but increasingly recognised cause of stroke. 1-3 We present the case of a patient presenting with embolic strokes caused by a PFE and will discuss the relevant literature regarding diagnosis and management. A 38 year old woman initially presented to our service in 2017 with a transient headache, associated with left arm weakness and reduced sensation in the left upper limb. MRI revealed multiple embolic infarcts in both cerebral hemispheres. She was subsequently diagnosed with a PFE on a transthoracic echocardiogram (TTE) and commenced on Apixaban for anticoagulation. A transoesophageal echocardiogram (TOE) was performed to evaluate the lesion further. Despite compliance with anticoagulation she had a further embolic episode in 2022, with symptoms of right hand weakness and poor coordination. MRI revealed acute infarcts in the right cerebellum and left parietal regions. The patient underwent successful surgical removal of the PFE following this. We reviewed the current evidence base for the diagnosis and management of PFE. Current literature suggests surgical excision should be considered first line treatment for symptomatic PFE as patients remain at risk of embolic stroke despite anticoagulation. 2 Surgical intervention with resection of the tumour is a curative measure, avoiding long-term anticoagulation. 1 The long-term outcomes have been shown to be favourable. 1,2,4 Evidence also suggests the importance of TOE in the evaluation of cryptogenic embolic stroke; it is estimated that the cause of stroke remains unclear in up to 30% of patients with an ischaemic stroke, and it is apparent that TTE may miss cases of PFE. 5–7 We conclude that first line treatment of PFE should be surgical excision if the patient is fit for surgery. We suggest that a TOE should be considered in young people with cryptogenic stroke in the context of a normal TTE. 1. Grolla E, Vestra MD, Zoffoli G, et al. Papillary fibroelastoma, unusual cause of stoke in a young man: a case report; J. Cardiothorac Surg. 2017; 12:33. 2. Yiu AC, Hussain A, Okonkwo UA, O'Shea J. A Case of Cardiac Papillary fibroelastoma – An Increasingly Described Cardiac Tumour with Fatal Consequences; HJH&SW 2021; 80:207-211. 3. Mkalaluh S, Szczechowicz M, Torabi S, Dib B, et al. Surgery for Cardiac Papillary Fibroelastoma: A 12-Year Single Insititution Experience; Med Sci Monit Basic Res 2017; 23: 258-263 4. Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi J. Cardiac papillary fibroelastoma: A comprehensive analysis of 725 cases. Am.Heart J. 2023; 146, No 3: 404-410 5. Semerano A, Saliou G, Sanvito F, et al. Fishing an anemone in the brain: embolised cardiac fibroelastoma revealed after stroke thrombectomy. Cardiovascular Flashlight, Eurheartj 2021; 4094. 6. Liebeskind DS, Buljubasic N, Saver JL. Cardioembolic Stroke Due to Papillary Fibroelastoma. J. Stroke Cerebrovasc. Dis. 2001; 10, No.2: 94-95 7. Maschicharan M, El-Dean Z, Zlocha V, Khoo J. Fibroelastoma in an unusual location: a rare cause of multiple cerebrovascular events. Echo Research and Practice 2019; 6, No. 3: 13-17