By Michael H. Crawford, MD, Editor
SOURCES: Tamin SS, et al. Prognostic and bioepidemiologic implications of papillary fibroelastomas. J Am Coll Cardiol 2015;65:2420-2429.
Fleischmann KE, Schiller NB. Papillary fibroelastoma: Move over myxoma. J Am Coll Cardiol 2015;65:2430-2432.
Papillary Fibroelastomas (PFE) are a common benign cardiac tumor thought to have thromboembolic potential. Investigators from the Mayo Clinic reported their experience with these tumors from 1995 to 2010. Patients who had inflammatory or infectious diseases were excluded. The 511 patients identified were divided into two groups: 1) histologically confirmed PFE removed during surgery, whether for this reason or incidental to other cardiac surgery, and 2) echocardiographically detected PFEs that were distinguished from Lambl’s excrescences. Group 1 was composed of 63% women with a mean age of 63 years. Cerebral symptoms or stroke were the presenting symptoms in 32%. Most PFEs were on valves, and the valve was preserved in 98%. Stroke risk in group 2 at 1 year was 6%, and 13% at 5 years. The authors concluded that patients with PFEs on echo who do not undergo surgical removal have an increased risk of stroke.
This large retrospective observational study makes several interesting points about PFEs. Although rare in their echo database (< 0.1%) they are probably as common or more common than myxomas. Also, they seem to be associated with neuroembolic events, but they could not find any characteristic of the PFEs (size, mobility) that influenced the risk of stroke. Thus, they recommend that all should be considered for surgical removal. In their hands, 98% would be removed without damaging the valve, and there was a very low recurrence rate (1.6%) over a 2-year follow-up.
Several things should be considered in making the decision to remove PFEs. They are more common with age, so you are often operating on older adults with other comorbidities. Many of these patients will have other risk factors for stroke. For example, more than a quarter of their patients had atrial fibrillation. It is unknown whether oral anticoagulants or antiplatelet agents will prevent strokes caused by PFEs. In addition, after surgical removal, 18% of the specimens were not PFEs by histology. Echocardiography does not always make an accurate tissue diagnosis. Thus, before deciding on surgery, an evaluation for other causes of valve masses should be made to exclude systemic lupus erythematosus, antiphospholipid syndrome, or infective endocarditis.