Cardiac Papillary Fibroelastoma and Cerebrovascular Events: A Systematic Review

Abstract

Background

Cardiac papillary fibroelastomas (CPFs) are benign endocardial tumours with embolic potential. This article is a systematic review to identify the clinical profile, diagnosis, tumour characteristics, and treatment modalities in patients with CPF presenting with stroke or transient ischemic attack (TIA).

Methods

This systematic review was conducted using the PubMed and Embase databases, including case reports and/or series and observational studies (for a search period of up until April 2022). A descriptive summary of case reports and/or series, and a narrative summary of observational studies, were completed.

Results

A total of 161 cases were identified from 133 case reports and 11 case series. The mean age of patients was 54.8 years, and 46.6% were male. TIA and stroke were reported in 32.3% and 67.8%, respectively. The most common stroke territory was multiple brain sites (36.6%). The mean tumour size was 11.8 mm. Most of the tumours were left-sided (98.7%). The mitral valve was the most involved valve (38.9%), with the anterior leaflet being the commonest site (61.3%). A total of 91.4% of tumours were independently mobile; 10.6% of tumours missed by transthoracic echocardiography were identified on transesophageal echocardiography. Antiplatelet and anticoagulation treatment were used in 87.9% and 12.1% of cases, respectively. Simple excision, valve repair, and valve replacement were performed in 66.7%, 16.7%, and 16.7%, respectively. The logistic regression model revealed that age was the only significant predictor; an increase in the log-odds of recurrent cerebrovascular events occurred with increasing age.

Conclusions

CPFs are a differential diagnosis of cryptogenic stroke, especially if the initial workup for stroke is negative. Transesophageal echocardiography serves as a better imaging tool, compared to transthoracic echocardiography, in identifying CPF. Although the consensus for CPF management remains controversial, surgical excision is the primary approach for left-sided CPF presenting as stroke or TIA.