Abstract 11716: A Systematic Review on Cardiac Papillary Fibroelastoma Presenting With Cerebrovascular Events

Abstract

Introduction: Cardiac papillary fibroelastomas (CPF) are rare, benign endocardial tumors with embolic complications like cerebrovascular events. The objective of this systematic review is to identify the clinical profile, diagnostic approach, tumor characteristics, and treatment modalities of patients with CPF presenting with stroke or TIA. Methods: We performed a systematic review using PUBMED and EMBASE databases involving all the published case reports and case series of CPF with TIA or stroke as presentation until April 2022. Viewpoints, editorials, and review articles were excluded. Results: A total of 161 cases were identified from 133 case reports and 11 case series. The mean age was 54.8 ± 15.6 years with 53.4% females. The proportion of patients with TIA and stroke was 32.3% and 67.8% respectively. Neuroimaging demonstrated the involvement of multiple sites (36.6%) and middle cerebral artery territory (35.6%) as major sites of stroke. The primary imaging tool for the diagnosis of CPF was echocardiogram. The size of the tumor varied from 2 to 47 mm (mean 11.8 ± 7.1). Left-sided valves were the most common location of the tumor (73.2%). Mitral valve was the most common valve involved (38.9%) with the anterior leaflet being the more common site (61.3%). The aortic valve was involved in 32.5% with non-coronary cusp being the more common site (37.5%). About 10.6% of cases were missed by TTE (mean size of valvular tumors missed by TTE was 5.6 ± 1.9 mm). Most of the tumors (91.4%) showed independent mobility. Surgical excision was performed in 89.5% cases with histopathological confirmation. Medical management prior to surgery included antiplatelets (87.9%) and anticoagulation (12.1%). Simple excision, excision with valve repair and excision with valve replacement were done in 66.7%, 16.7% and 16.7% respectively. Most patients (97.8%) had uneventful post-operative course. Conclusions: CPFs are potential differential diagnosis for cryptogenic stroke. TEE serves as a better diagnostic modality for cardiac workup of cryptogenic stroke if the initial TTE is negative. While the consensus for CPF management remains controversial, the results of our study support surgical management as the primary approach for left-sided tumors presenting as stroke/TIA.