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.