Cardiac Papillary fibroelastoma (CPF): a rare underrecognized etiology of cardioembolic stroke.
Mohamed Eldokmak1, Yohannes Mulatu2, Biniyam Mulatu3, Weijun Jin4
1Neurology, Department of Neurology, State University of New York Downstate Health Sciences University, 2SUNY Downstate, 3Department of Dermatology, Addis Ababa University, 4Maimonides Medical Center
Objective:

NA

Background:

Cardiac papillary fibroelastoma (CPF) is the most common tumor of cardiac valves. It mostly affects the aortic valve. In addition to life-threatening cardiovascular complications such as valve obstruction, it is increasingly being recognized as a source of systemic embolization. TIA, ischemic strokes, and MI are the most common presentations. TTE or TEE will show small mobile pedunculated or sessile valvular or cardiac mass. Treatment includes surgical resection for symptomatic patients and systemic anticoagulation for non-surgical candidates.

Design/Methods:

A case report of aortic valve fibroelastoma presenting with Ischemic stroke.

Results:

A 69-year-old woman with hypertension and diabetes presented with confusion and visual field defects and was found to have bilateral cortical and subcortical cerebral and cerebellar embolic appearing stroke on MRI. CT angiography of the head and neck did not show large vessel occlusion or significant stenosis. TTE was negative for embolic source. Transcranial Doppler showed microemboli in the right MCA. Telemetry and 14-days Holter monitoring were without arrhythmias. TEE revealed a1 cm mobile echo density pedunculated aortic valve mass. Negative blood cultures ruled out infective endocarditis. A hypercoagulable stroke workup was non-revealing. Considering the high risk of stroke recurrence cardiac surgery and resection 0.6x0.2x0.1 cm tan-white soft tissue mass was done under cardiopulmonary bypass. histopathology examination showed branching papillary fronds lined with epithelial cells and collagenous core with focal areas of myxoid change consistent with a papillary fibroelastoma.

Conclusions:

Cardioembolic strokes in the setting of a cardiac mass on echocardiography not only need a workup for vegetations or thrombi but rather should also consider rare etiologies such as CPF. This case highlights the growing recognition of cardiac papillary fibroelastoma as a rare but important cause of cardioembolic stroke. Early surgical excision should be performed in such patients due to the high risk of stroke recurrence.

10.1212/WNL.0000000000202967