Abstract 361: Papillary fibroelastoma as a rare cause of recurrent strokes in a young female: when to intervene?
Authors: Sehgal N. , Rana R. , Siegler J. E. 
Published: January 2025

Introduction

Primary cardiac tumors including papillary fibroelastomas are rare, with an incidence as low as 0.1%, but they pose considerable embolic risk. When untreated, cardiac fibroelastomas can present with embolic stroke in about 30% of patients. While larger, mobile fibroelastomas may be thought to carry a greater risk of cerebral embolism, observational data indicate no single echocardiographic feature to be sufficiently predictive of stroke. We present a case of a young female who presented with recurrent strokes in the setting of a small, untreated papillary fibroelastoma.

Case presentation

A 41‐year‐old Black female with past medical history significant for three ischemic strokes (2020, 2021) without residual deficits, presented in June 2024 to our institution with new left‐sided weakness and dysarthria over 24 hours. NIHSS was 7 for left facial droop, dysarthria, and left‐sided weakness. Initial imaging (CT head, CTA head/neck, CT perfusion) demonstrated chronic, known cortical infarcts without large‐vessel occlusion or perfusion deficit. MRI brain without contrast demonstrated multiple small, acute infarcts in the right perirolandic region and FLAIR changes consistent with prior infarcts in the left MCA, right occipital and right cerebellar regions. A transthoracic echocardiogram demonstrated no structural abnormalities, normal left ventricular ejection fraction, and normal left atrial volume index. However, a transesophageal echocardiogram demonstrated a rounded mobile echodensity measuring 0.48cm x 0.13cm attached to the commissure of left and right cusps on the aortic valve, consistent with a papillary fibroelastoma. Transthoracic echocardiogram in 2020 during her first stroke admission demonstrated a similarly sized, mobile echodensity (~0.3 times 0.2cm) of the aortic valve concerning for fibroelastoma. At the time, this fibroelastoma was thought to be too small to be causative of her ischemic stroke, therefore therapeutic anticoagulation or surgical intervention was deferred. She was discharged with aspirin 81mg daily, and was switched to clopidogrel 75mg daily after her second stroke in 2021. Again, surgery was deferred.

Hypercoagulability workup (anticardiolipin, beta‐2 glycoprotein, protein C, protein S, homocysteine, Factor V Leiden, antithrombin 3) was normal. There was no evidence of atrial fibrillation during any of her four inpatient stroke admissions. Given that this was her fourth ischemic stroke without an alternative mechanism of stroke, aggressive management of papillary fibroelastoma was pursued. She was started on therapeutic anticoagulation with apixaban and evaluated by cardiothoracic surgery for surgical removal, which took place in August 2024.

Discussion

Cardiac fibroelastomas are an underrecognized cause of stroke and carry a high risk of morbidity and mortality if untreated, even when the size is small. This case illustrates the importance of early and aggressive intervention of papillary fibroelastomas when associated with otherwise cryptogenic embolic events. The gold‐standard treatment for management of embolic events in the setting of cardiac fibroelastomas is surgical excision, which can leave the valve intact for >95% of patients and carries a low risk of recurrent stroke. In this case, the patient was started on antiplatelet agents however recurrent strokes occurred while adherent to these therapies. In patients unable to undergo surgical excision, initiation of anticoagulation may be considered, but is of uncertain benefit.