Cardiac papillary fibroelastoma: So small and yet so dangerous

Cardiac papillary fibroelastoma (PFE) is the third most frequent tumor of the heart after atrial myxoma and lipoma, and is the most frequent tumor of the cardiac valves. Before the advent of echocardiography, these tumors were found incidentally at autopsy and called ‘‘small harmless bodies’’. The first reported pre-mortem case was an incidental finding during surgical ventricular septal defect repair in 1979. However, while histologically benign, PFE is a dangerous condition due to the potential for cerebral and coronary embolization. While in around two thirds of cases diagnosis is made fortuitously by echocardiogram, one third are diagnosed following an embolic event that triggers further exploration. PFE are single in 90% of cases and more than 95% are located in the left heart. Once diagnosed, surgical resection is recommended to avoid embolic complications; this constitutes definitive treatment as recurrence is very rare.