Prognostic and Bioepidemiologic Implications of Papillary Fibroelastomas

Background: Papillary fibroelastomas (PFE) are benign neoplasms with little available outcome data.

Objectives: This study sought to describe the frequency and clinical course of patients with surgically removed PFE and echocardiographically suspected, but unoperated, PFE.

Methods: Mayo Clinic pathology and echocardiography databases (January 1, 1995, to December 31, 2010) were queried, resulting in 511 patients: group 1 (n = 185), including patients with surgically removed, histopathologically confirmed PFE; group 1a (n = 94; 51%) with PFE removed at primary surgery; and group 1b (n = 91; 49%) with PFE removal at time of another cardiac surgery. Group 2 (n = 326) patients had echocardiographic evidence of PFE but no cardiac surgery to remove PFE.

Results: Group 1 had mean age of 63 ± 14 years (116 women [63%]). During the study period, we identified 112 cardiac myxomas in the pathology database and 142 in the echocardiographic database. Mean age in group 2 was 67 ± 14 years (162 women [50%]). PFE occurred most commonly on cardiac valves (n = 400 [78%]). In group 1, transient ischemic attack or stroke was the presenting symptom in 58 patients (32%). With surgical removal of valvular PFE, the valve was preserved in 92 (98%). Recurrence was documented in 3 patients (1.6%). Follow-up stroke risk in groups 1, 1a, and 1b at 1 year was 2%, 0%, and 4%; at 5 years, 8%, 5%, and 11%, respectively. Cerebrovascular accident risk in group 2 at 1 and 5 years was 6% and 13%.

Conclusions: In patients with echocardiographically suspected PFE who do not undergo surgical removal, rates of cerebrovascular accident and mortality are increased.